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    [Primary cancer of the female urethra: clinical case]. Bertoli A Archivio italiano di chirurgia
    [Primary carcinoma of the female urethra]. Rodrigues-Netto Júnior N,Giron A M,de Góes G M,de Campos-Freire J G Revista paulista de medicina
    Primary urethral malignancy: review of 22 cases. Allen R,Nelson R P Southern medical journal Twenty-two cases of primary urethral malignancy have been diagnosed at the Medical University Hospital from 1955 to 1976. These lesions are uncommon and prognosis is poor except when disease is diagnosed in the early stages and treated aggressively. Anterior lesions are more often diagnosed at an early stage but may be mistaken for benign processes, delaying appropriate therapy. Our experience with urethral malignancies is reviewed and includes an association of carcinoma with urethral caruncles and diverticula. The first reported association of a urethral diverticulum with carcinoma in the male is presented and two additional cases of adenocarcinoma in female urethral diverticula are reported. Excision of urethral caruncles and diverticula is encouraged. 10.1097/00007611-197805000-00020
    [Primary malignant tumors of the female urethra]. MONTELLA G Archivio italiano di urologia
    Primary malignancy of the female urethra. BAURYS W,ZIOBROWSKI F G Guthrie Clinic bulletin
    [Malignant primary tumors of the female urethra; report of a case]. GONZALEZ ABREU F,KAUFMAN A Boletin de la Liga Contra el Cancer. Liga Contra el Cancer (Cuba)
    [Primary adenocarcinoma of the female urethra: report of a case]. Yoshida O,Shimizu Y,Komatsu Y Hinyokika kiyo. Acta urologica Japonica
    [Primary carcinoma of the urethra in a woman. Apropos of 1 case]. Megias Garrigos J,Schiefenbusch Munne E,Romero Pérez P,Martínez Soriano J,Pérez Llorca L,Mira Llinares A Actas urologicas espanolas
    Primary infiltrating adenocarcinoma of urethral diverticulum in a female. Schileru G,Bussey J,Carter E F Acta urologica Belgica
    Primary carcinoma of the female urethra. Elkon D,Kim J A,Huddleston A L,Constable W C Southern medical journal A retrospective review of women with carcinoma of the urethra is reported. Twelve patients treated between 1947 and 1978 have been characterized as to presenting features, therapy, and prognosis. There average age of patients at diagnosis was 68 years; the most frequent presenting symptom was bleeding (92% of patients); average duration of symptoms before diagnosis was five months. Localized tumors of the distal urethra have been effectively controlled by using interstitial implantation of radioactive sources, five of five patients having no evidence of disease one to ten years after treatment. Our study agrees with reports in the literature on the results of interstitial implantation of radioactive sources. Patients with tumor involving the entire urethra have a poor prognosis, and four of five patients died within the first year after therapy. 10.1097/00007611-198011000-00005
    Primary carcinoma of the female urethra. Benson R C,Tunca J C,Buchler D A,Uehling D T Gynecologic oncology
    [Primary carcinoma of the female urethra]. Takahashi H,Hirano A,Nakano M,Tanaka K,Shinagawa T Hinyokika kiyo. Acta urologica Japonica An 80-year-old female patient with urethral carcinoma is reported. A red, easily bleeding mass at the urethral meatus was diagnosed as transitional cell carcinoma by biopsy. Complete resection of the urethra and tubeless cystostomy were performed. Postoperative M-VAC therapy a combination of methotrexate, vinblastine, adriamycin and cisplatin was employed.
    [Primary cancer of the female urethra]. AIVAZIAN A V Voprosy onkologii
    [Result of therapy for primary carcinoma of the female urethra (author's transl)]. Takeda T,Kawai T Nihon Hinyokika Gakkai zasshi. The japanese journal of urology
    Primary adenocarcinoma of the female urethra. Renders G Acta urologica Belgica
    Carcinoma of the female urethra: reassessment of modes of therapy. Hopkins S C,Vider M,Nag S K,Tai D L,Soloway M S The Journal of urology Of 15 women with primary urethral carcinoma 2 had tumors confined to the urethra and were managed successfully by an operation. Of the 9 patients with tumor extending to the surrounding structures 6 (67 per cent) died of complications related to inadequate control of the primary tumor. The last 4 patients had stage D1 disease or greater at initial diagnosis and died of distant metastases. Our current approach for patients with locally advanced disease is combined brachytherapy and operation in an effort to eradicate the primary tumor, since morbidity and mortality result from failure to control the local tumor. 10.1016/s0022-5347(17)52482-9
    Management of primary urethral cancer. Gheiler E L,Tefilli M V,Tiguert R,de Oliveira J G,Pontes J E,Wood D P Urology OBJECTIVES:To determine the best therapeutic approach for treatment of patients with urethral cancer according to tumor location and clinical-pathologic stage. METHODS:A retrospective review of 21 consecutive patients diagnosed with primary urethral carcinoma was performed. Clinical-pathologic staging, treatment modality, and outcome were analyzed. RESULTS:The overall survival rate was 62%. In patients with clinical Stage Ta-2N0M0 tumors, 8 of 9 patients (89%) are free of disease compared to 5 of 12 patients (42%) with Stage T3-4N0-2M0 tumors (P = 0.03). Best treatment outcome for patients with Stage T3 disease or higher was obtained when multimodality therapy (neoadjuvant chemotherapy and radiation therapy with or without surgery) was administered, with a disease-free survival rate of 60%. CONCLUSIONS:Clinical-pathologic stage was a strong predictor of disease-free survival rate. For patients with Ta-2N0M0 tumors, multimodality therapy may not be required. Conversely, best treatment outcomes in patients with T3-4N0-2M0 tumors are obtained by administering a multimodal therapy combining chemotherapy and radiation therapy with surgical resection. 10.1016/s0090-4295(98)00199-x
    Pathology and natural history of urethral tumors in females: review of 65 cases. Roberts T W,Melicow M M Urology Cancer of the female urethra, although uncommon, is important because unless recognized early and treated adequately, death will ensue, preceded by much morbidity. The authors present a series of 62 patients with 70 primary tumors, and 3 patients with secondary tumors. A comprehensive classification of neoplasms of the female urethra is also included. 10.1016/0090-4295(77)90111-x
    Primary carcinoma of the female urethra. Turner A G,Hendry W F British journal of urology Experience in the diagnosis and management of 39 female patients with primary carcinoma of the urethra is described. The condition presents to both gynaecological and urological units and has a relatively good prognosis provided that it is diagnosed quickly. There must be, however, a clinical awareness of the condition in those patients presenting with non-specific urological symptoms. Radiotherapy is the treatment of choice using interstitial implants for the superficial tumours and external beam for the deeper tumours. Surgery alone is possible in only a small number of cases, it being reserved mainly for recurrent or resistant disease. 10.1111/j.1464-410x.1980.tb03113.x
    [Primary adenocarcinoma of the female urethra: report of a case]. Inoue S,Oe H,Murata S Hinyokika kiyo. Acta urologica Japonica
    Primary urethral neoplasms: review of 30 cases. Bolduan J P,Farah R N The Journal of urology The recent literature shows our findings on primary urethral neoplasms to be consistent with others, although we did not show an increased incidence of these neoplasms in female over male subjects. Similarly, we recommend an operation with or without irradiation, depending on the stage and location of the lesion. The over-all prognosis of urethral neoplasms remains poor. However, the distal urethral lesions in male and female subjects are easier to approach surgically and seem to be diagnosed earlier in the progression of this disease than the more proximally advanced tumors. These early staged and distal neoplasms greatly improve the changes of long survival. The delay in diagnosis gives this neoplasm its poor prognosis. In men the prognosis probably could be improved by more aggressive evaluation of stricture disease, especially when the need for dilation becomes frequent. In women we believe that the caruncle should be biopsied if it shows signs of progression or remains symptomatic (pain, bleeding and so forth). If a 1 to 2-month course of antimicrobials does not resolve this lesion we recommend biopsy. A relationship between tumor and a diverticulum has been noted in the literature, as in 1 of our cases of adenocarcinoma in a female patient. Whether this relationship could be explained on the basis of recurrent infection and stasis remains theoretical. In conclusion, early diagnosis, accurate staging and aggressive treatments are the means for cure of this disease. 10.1016/s0022-5347(17)54965-4
    Primary malignant neoplasm of the female urethra. Ampil F L Obstetrics and gynecology This is a retrospective review of 11 cases of primary malignant neoplasm of the female urethra seen at the Louisiana State University Medical Center in Shreveport from 1951 to 1984. The disease was relatively more frequent in the 60- to 79-year age-group. Squamous cell carcinoma was the most common among the different observed histopathology. A modified clinical staging system is introduced. At diagnosis, eight of 11 subjects (73%) had locally extensive disease. The majority of the patients were treated with radiotherapy alone or in combination with surgery. The overall severe complication rate was low. The poor results (20% local control and survival) obtained in this small experience suggest that perhaps a study using promising adjuvant chemotherapeutic agents is warranted. A literature review summary of reported radiotherapy results is included.
    Primary carcinoma of female urethra: review of 20 cases. Kamat M R,Kulkarni J N,Dhumale R G Journal of surgical oncology Twenty patients of primary urethral carcinoma in females are presented. Age group affected was between 40 and 60 years. Vague symptoms in early stage delayed the diagnosis in most patients. Surgery is the treatment of choice for meatal or anterior urethral lesions while combined Radiotherapy and surgery has been suggested as treatment for entire urethral lesions. In the present series 33% three years and 21% five years survivals were noted.
    Primary adenocarcinoma of female urethra associated with diverticula. Tesluk H Urology Three cases of primary adenocarcinoma of the female urethra associated with diverticula are presented. Although the apparent risk of malignant change in urethral diverticula is low, such lesions are usually adenocarcinomas and may arise from urethritis glandularis, mesonephric rests, periurethral glands, or cloacogenic rests. 10.1016/0090-4295(81)90241-7
    Clear cell adenocarcinoma of the urethra: evidence for origin within paraurethral ducts. Spencer J R,Brodin A G,Ignatoff J M The Journal of urology We report a case of clear cell adenocarcinoma arising in a paraurethral duct treated by anterior pelvic exenteration. Immunohistochemical stains for prostate specific acid phosphatase and prostate specific antigen were positive in the primary tumor and regional metastases. Focal positive staining also was noted in normal paraurethral duct epithelium. Our observations suggest that clear cell adenocarcinoma arises from the female paraurethral ducts, rather than embryonic remnants. These ducts appear to be homologous to the prostate and in some cases may be misinterpreted as urethral diverticula. 10.1016/s0022-5347(17)39887-7
    Female urethral carcinoma: an analysis of treatment outcome and a plea for a standardized management strategy. Dalbagni G,Zhang Z F,Lacombe L,Herr H W British journal of urology OBJECTIVE:To evaluate our experience with primary carcinomas of the female urethra, by analysing the impact of tumour variables and treatment on overall, disease-specific, local recurrence- and metastasis-free survival. PATIENTS AND METHODS:Between 1958 and 1994, 72 women (median age 60 years, mean 59, range 21-84) with primary urethral carcinoma were identified. They were followed for a median (range) of 85 (0-384) months. The patients were stratified by stage, nodal status, histology, treatment, type of surgery, site of disease, year of diagnosis and smoking habit. RESULTS:In a univariate analysis, stage, nodal status, type of surgery and site of the disease were important factors for survival and recurrence. In a multivariate analysis, primary stage, nodal status and site of disease were independent predictors of survival. CONCLUSION:Current modalities of treatment are ineffective for local control and survival; new treatment strategies are needed for female urethral cancers. 10.1046/j.1464-410x.1998.00878.x
    Carcinoma in diverticulum of female urethra. Gonzalez M O,Harrison M L,Boileau M A Urology A case of carcinoma originating in a diverticulum of the urethra in a female patient is presented. A review of 143 cases of carcinoma of the female urethra treated from 1948 to 1984 at The University of Texas M. D. Anderson Hospital and Tumor Institute at Houston disclosed 6 additional patients with diverticular carcinoma. Analysis of their clinical features, treatments--various combinations of primary excision, radiotherapy, and chemotherapy--and survival results indicate that survival is primarily a function of grade. Only 40 cases of carcinoma in urethral diverticula are recorded in the world literature. The majority are adenocarcinomas, and the most frequent presenting symptoms are dysuria, frequency, and urgency. Radiotherapy successfully established long-term control of the disease with low morbidity in all of our patients who had low-grade tumors. 10.1016/0090-4295(85)90177-3
    Primary adenocarcinoma of the female urethra. Tiltman A J The Journal of pathology Three cases of primary adenocarcinoma of the female urethra are presented. The tumour in the first case was associated with urethritis glandularis and is thought to arise from previous glandular metaplasia of the urethral mucosa. The tumour in the second case resembled the clear-cell carcinomas of the lower female genital tract and is thought to be of embryonic rest origin. The tumour in the third case is thought to arise from the paraurethral glands. 10.1002/path.1711170207
    [Primary urethral cancer in the female: a diagnostic and therapeutic challenge]. Diederichs W,Haupt G Geburtshilfe und Frauenheilkunde Primary carcinoma of the urethra in women is extremely rare and associated with non-specific symptoms of the lower urinary tract, the vagina and the pelvic floor. On the basis of four case reports, the frequent delay in diagnosis is presented and the therapeutic course is outlined. 10.1055/s-2007-1026199
    Carcinoma of female urethra. Manitoba experience: 1958-1987. Hahn P,Krepart G,Malaker K Urology Fourteen female patients with primary urethral carcinoma were treated at the Manitoba Cancer Foundation in the last twenty-nine years. The relationship of natural history to the stage, location, and therapeutic modality has been reviewed. A higher stage and length of urethral involvement affected prognosis negatively, whereas lower stage had a positive prognostic effect and location of tumor had no prognostic influence. Two patients with Stage C, who failed to received inguinal node radiotherapy, died of disease recurring in the inguinal area. Patients who received inguinal radiation (3 patients Stages B, C, and D1) had no regional recurrence. It is suggested that, for all female urethral carcinoma, bilateral ilioinguinal nodes be included in the radiation field. For radical treatment, iridium 192 insertion in combination with external beam treatment is recommended. 10.1016/0090-4295(91)80202-i
    [Two cases of primary urethral carcinoma in females]. Yamada Y,Honda N,Hayase Y,Senda H,Fukatsu H,Segawa A Hinyokika kiyo. Acta urologica Japonica Two cases of primary female urethral carcinoma are presented and discussed. The patients were 70 years old and 65 years old, and the pathological diagnoses were squamous cell carcinoma and adenocarcinoma, respectively. The patient with squamous cell carcinoma had metastasized inguinal lymph nodes at the first consultation, despite the administration of bleomycin, and the tumor had widely metastasized to skin of the lower extremities. She died of cachexia and disseminated intravascular coagulopathy (DIC). The other patient with adenocarcinoma was treated by postoperative cobalt radiation therapy in a total dose of 6,000 rads. After 18 months, she had recurrence of tumor in urethra, and developed Virchow's node metastasis, she died of acute renal failure. Both patients died within two years.
    Primary carcinomas of the urethra. Amin M B,Young R H Seminars in diagnostic pathology Urethral carcinomas are rare, and information reported in the literature is relatively limited. In this review, the authors present separate discussions on the pathology of carcinomas occurring in men and women with emphasis on the diverse histological subtypes. Because tumor type and prognosis correspond to anatomic location, normal anatomy and histology have been given due consideration. Of particular note in the recent literature is an expanded experience with clear cell carcinoma, a distinctive tumor primarily of the female urethra that has generated considerable interest with respect to its prognosis and relationship to urethral diverticulum. The authors use this review to illustrate several features of the pathology of these neoplasms synthesize the literature, and place into perspective the clinical, pathological, and prognostic features.
    Primary carcinoma of the female urethra. Bracken R B,Johnson D E,Miller L S,Ayala A G,Gomez J J,Rutledge F The Journal of urology The clinical and morphological features in 81 cases of carcinoma of the female urethra were reviewed. The over-all 5 and 10-year survival rate for the entire group was 32 per cent. Survival expectations for patients with squamous carcinoma, transitional cell carcinoma and adenocarcinoma were similar when analyzed according to stage and all cell types appeared to respond equally to irradiation. Prognosis was related directly to the clinical stage of the disease. A plea is made for more accurate assessment of the disease status. The high incidence of local recurrence noted for all forms of single modality therapy (46 to 64 per cent) suggests the need for clinical trials with combination preoperative irradiation followed by definitive surgical procedures. 10.1016/s0022-5347(17)58741-8
    Primary carcinoma of the female urethra. Desai S,Libertino J A,Zinman L The Journal of urology 10.1016/s0022-5347(17)60317-3
    Primary adenocarcinoma of the female urethra with three histologic patterns and partial AFP positivity. Hanai J,Lin M Acta pathologica japonica A rare case of adenocarcinoma of the female urethra with alpha-fetoprotein (AFP) positivity in a 52-year-old woman is reported. The tumor was papillary polypoid, localized in the posterior wall of the mid-urethra and microscopically showed three histologic components. Upon immunostaining and histochemical staining, the tumor was characterized by intestinal-type cells positive for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA), EMA-negative and AFP-positive columnar vacuolated cells and mainly EMA-positive clear cells. On the basis of these features together with the known embryogenesis of the urethra, an endodermal origin of the tumor is suggested, possibly arising from the reserve or stem cells in the urethral mucosa. This case and its immunohistochemical features are quite unique and the histologic combination is meaningful when considering the oncogenesis and histogenesis of urethral tumors.
    [Cancer of the urethra in women: experience of the National Institute of Cancer of Brazil: 1992-1997]. Ornellas A A,Khouri F R,Campos F,Koifman N,Quirino R Progres en urologie : journal de l'Association francaise d'urologie et de la Societe francaise d'urologie OBJECTIVE:Describe the results of surgery, radiation therapy and chemotherapy for treatment of primary malignant neoplasms of female urethra. PATIENTS AND METHODS:Since 1982, 31 patients with urethral cancer were evaluated at our institution (follow-up ranging from 0 to 127 months). Ten patients were treated with external beam irradiation and 1 patient received preoperative therapy after surgery. Two patients refused treatment and 1 received chemotherapy. Three patients presented with disseminated metastatic disease at the first examination received exclusively palliative treatment. RESULTS:Five patients have survived from 3 to 10 years following treatment without recurrence. Of 31 patients 18 developed distant metastases during the first 24 months of follow-up irrespective of treatment employed. CONCLUSION:With exception of primary melanoma, prognosis was not related to histologic features. Patients who underwent surgery and radiotherapy had a better survival rate than did those who received radiotherapy alone. Total urethrectomy with appendico-vesicostomy can be an alternative surgical method for entire urethral ivnasive lesions without cystourethrectomy and preserving urinary continence.
    [Glandular metaplasia and primary adenocarcinoma of the urethra]. Ebel K,Klinger D Der Urologe. Ausg. A This is a case report of a 67-year-old woman who had a mucinous adenocarcinoma of the urethra: she died 3 years after the clinical symptoms started owing to progression of the tumor. The tumor developed from a glandular metaplasia, which has to be regarded as a premalignant lesion. Problems of the diagnosis, including the differential diagnosis, are discussed.
    Primary carcinoma of female urethra. Johnson D E,O'Connell J R Urology The clinical and morphologic features in 29 cases of primary carcinoma of the female urethra were reviewed. Only 2 of 12 patients treated with radiotherapy are known to have failed; one is dead of disease at one year, and the other is alive with local recurrence at two years. We consider these results sufficiently satisfactory to warrant the continued use of radiotherapy in early-stage lesions, as well as in selected patients who have infiltrating carcinoma. Integrated therapy consisting of 5,000 rad/25 fractions/five weeks, followed in six weeks by radical cystourethrectomy, was used in 7 patients. The low morbidity and absence of operative mortality recommend continuation of this aggressive approach for infiltrating tumors in selected patients. 10.1016/0090-4295(83)90120-6
    [Carcinoma of the female urethra]. Nan X Y Zhonghua zhong liu za zhi [Chinese journal of oncology] From 1962 to 1980, 24 patients with primary urethral carcinoma and 1 vulval cancer involving the urethra were treated in our hospital. In this series, the mean age was 52 years. 23 lesions in the distal (anterior) urethra were all diagnosed preoperatively. Pathological classification were as follows: squamous cell carcinoma 16, adenocarcinoma 3, transitional cell carcinoma and undifferentiated carcinoma 4. The chief method of treatment were resection of the tumor and reconstruction of a new urethra, using bladder flap in two forms: abdominal urethra and reconstructed urethra in the original position. Preoperative or postoperative irradiation was given in one third of the patients. 17 patients have shown satisfactory results in the follow-up. The 7 and 10 year survival rates are 36% and 18% among 11 patients who are living and well.
    Female urethral adenocarcinoma: evidence for more than one tissue of origin? Dodson M K,Cliby W A,Pettavel P P,Keeney G L,Podratz K C Gynecologic oncology Adenocarcinoma of the female urethra accounts for 10% of all urethral cancers. Controversy continues to exist over the origin of primary urethral adenocarcinomas. The periurethral (Skene's) glands appear to be the homologues of the male prostate as defined by authors evaluating cadaver-derived periurethral glands pathologically and immunohistochemically (prostate-specific antigen (PSA)). It is traditionally assumed that the origin of female urethral adenocarcinoma is the Skene's gland. However, no one has evaluated a series of primary urethral adenocarcinomas in an effort to scrutinize this assumption. We, therefore, evaluated 13 primary adenocarcinomas of the female urethra comparing histologic and immunohistochemical characteristics. Tumors were classified into two major histologic groups: columnar/mucinous (11) and clear cell (2). Excluding one case, the columnar/mucinous tumors resembled either endometrial or colonic adenocarcinoma. The exception was a case bearing a striking resemblance to prostatic adenocarcinoma. Immunohistochemical results revealed positive PSA staining for this tumor alone. The patient's preoperative serum PSA was elevated, but rapidly declined postoperatively. Based on immunohistochemical findings and the presence of distinct histologic subtypes (columnar/mucinous, clear cell), it appears that female urethral adenocarcinoma has more than one tissue of origin with a minority arising from the Skene's glands. 10.1006/gyno.1995.9963
    [Female primary urethral tumors]. Sakashita S,Kashiwagi A,Nakanishi S,Ishii D,Hisajima S,Koyanagi T Hinyokika kiyo. Acta urologica Japonica Our experience on treatment of female urethral tumors is presented. Nineteen female patients with primary urethral tumors were treated at our University Hospital during the 31 years since 1953. Ten of the patients had carcinoma (6 had squamous cell carcinoma, 2 had adenocarcinoma and 2 had transitional cell carcinoma) and the other 9 patients had benign urethral neoplasms. Although the patients with urethral carcinomas did not always undergo standardized treatment, 5 patients were treated with distal urethrectomy, 3 with urethrectomy, 1 with total cystourethrectomy and the other patient with TUR. Three patients were lost to follow up, the other females were alive, 4 months to 7 years after treatment excluding one patient who died of disseminated disease 1 year after TUR. Since primary female urethral malignancies are rare diseases, there is still controversy as to the choice of treatment for the disease because of the poor prognosis even after an operation. Our experience is not enough to conclude on the best choice of treatment, but more extensive operation including puboosteotomy is recommended in the latest literature.
    Primary adenocarcinoma of the female urethra. A review and report of 3 cases. KNOBLICH R American journal of obstetrics and gynecology 10.1016/0002-9378(60)90138-1
    Villous adenoma of the urinary tract: a lesion frequently associated with malignancy. Seibel Jeffrey L,Prasad Saket,Weiss Robert E,Bancila Edita,Epstein Jonathan I Human pathology Villous adenomas arising in the urinary tract are rare. We identified 18 cases of villous adenomas of the bladder, urachus, and prostatic urethra. Patients ranged in age from 53 to 93 years with an average age of 69.6 years and a male preponderance of 67%. In six cases (33%), the lesion was pure villous adenoma. In three cases (17%), there was villous adenoma with in situ adenocarcinoma. In six cases (33%) there was villous adenoma with in situ and infiltrating adenocarcinoma. One case (6%) had villous adenomas with in situ (noninvasive) papillary urothelial carcinoma. One case (6%) had villous adenomas with in situ adenocarcinoma and in situ papillary (noninvasive) and infiltrating urothelial carcinoma. The remaining case (6%) had villous adenoma with in situ and infiltrating adenocarcinoma and in situ (noninvasive) papillary and infiltrating urothelial carcinoma. Clinical outcome was available in eight of the cases, with a mean follow-up of 4.6 years. No evidence of recurrence was found in two patients with pure villous adenoma or in two patients with villous adenoma and only in situ adenocarcinoma, all of whom were treated by nonradical excision. However, two of three cases with infiltrating cancer developed distant metastases despite radical surgery; the remaining patient was disease-free 11 years after transurethral resection. The case with villous adenoma and in situ urothelial carcinoma progressed to sarcomatoid urothelial carcinoma following partial cystectomy. Eight of 10 villous adenomas cases studied expressed the epitope for mAbDas1, found on colonic epithelium and primary adenocarcinomas of the bladder and urachus but not on normal or neoplastic urothelium. This study expands the spectrum of histologic features accompanying villous adenomas of the urinary tract. Coexisting infiltrating adenocarcinoma is often present, necessitating thorough sampling of any lesion diagnosed by biopsy as villous adenoma. Pure villous adenoma and those well-sampled lesions also containing in situ adenocarcinoma portend a favorable prognosis, even without radical treatment. Coexisting in situ or infiltrating carcinoma suggests a more aggressive course. Histologically, immunohistochemically, and prognostically, these lesions appear analogous to their counterparts in the intestine. 10.1053/hupa.2002.31293
    [Primary adenocarcinoma of the female urethra: report of two cases]. Horiuchi S,Kaneoya F,Negishi T,Yoshida K Hinyokika kiyo. Acta urologica Japonica Two cases of primary adenocarcinoma of the female urethra are reported. Case 1 was a 75-year-old female presenting with the complaint of genital bleeding. Physical examination revealed a pea-sized tumor in the urethral meatus. Tumor resection was performed under the preoperative diagnosis of caruncles, but, histopathological examination revealed adenocarcinoma. She was readmitted because of recurrence of tumor 7 months after the first operation. En bloc resection including the bladder, urethra and uterus was done followed by ileal conduit for urinary diversion. Case 2 was an 85-year-old female presenting with the complaint of macroscopic hematuria. Physical examination revealed a thumb's-head-sized tumor in the urethral meatus and also the left enlarged inguinal lymph nodes. Tumor resection and biopsy of the left inguinal lymph nodes were performed. Histopathological examination revealed adenocarcinoma. Postoperative irradiation to the left inguinal lymph nodes was given to a total dose of 4,000 rads. Ninety-two cases of primary adenocarcinoma of the female urethra in the Japanese literature including our cases are reviewed.
    PRIMARY ADENOCARCINOMA OF THE FEMALE URETHRA: A REVIEW AND REPORT OF TWO CASES. SCHNITZER B The Journal of urology 10.1016/s0022-5347(17)63906-5
    Primary adenocarcinoma arising from a paraurethral cyst in a female patient. Nagano Masafumi,Hasui Yoshihiro,Ide Hisamitsu,Itoi Tatsunori,Takehara Toshiyuki,Osada Yukio Urologia internationalis A very rare case of primary adenocarcinoma arising from a paraurethral cyst in a 63-year-old woman is reported. Initially she was diagnosed as having a simple paraurethral cyst because of absent communication with the urethra. The resected paraurethral cyst was histologically associated with adenocarcinoma. We also performed chemotherapy composed of methotrexate, vinblastine, Adriamycin and cisplatin because of lymph node metastasis. Our treatment, however, was not effective and the patient died of systemic metastases. 10.1159/000063937
    [Villous adenoma of the urinary tract: a clinicopathological study]. Yin Wu,Mo Xiang-lan,Wen Zong-hua,Zhou Xiang-zhen,Zhou Min-yan,Wei Hai-ming Zhonghua bing li xue za zhi = Chinese journal of pathology OBJECTIVE:To explore the clinicopathological features, immunophenotype, differential diagnosis, pathogenesis and prognosis of villous adenoma with poorly differentiated adenocarcinoma of the urinary tract. METHODS:Clinical and pathologic findings of 3 cases of villous adenoma with poorly differentiated adenocarcinoma of the urinary tract were analyzed by gross examination, microscopic investigation and immunohistochemical staining. The related literatures were reviewed. RESULTS:All of the three cases were middle-aged or elderly patients. Three cases all presented with hematuria and mucusuria. Endoscopic examination identified that case 1 had a polyp with broad attachment in the dome of bladder, case 2 had a solid mass in the ureter, and case 3 had a exophytic fungating tumor in the renal pelvis. Microscopically, case 1 revealed a papillary lesion with finger-like processes lined by pseudostratified columnar epithelium with abundant goblet cells. The cells demonstrated moderate degree dysplasia. In case 2 and case 3, both villous adenomas and poorly differentiated adenocarcinoma were observed, the adenoma cells arranged in a cribriform pattern, and the tumor cells showed severe atypia, mitotic activity, and transition with invasive poorly differentiated adenocarcinoma. Immunohistochemically, the tumor cells in three cases were positive for CK20, CEA,EMA and MUC-1; none of them expressed cdx-2 and PSA; In case 2 and 3, the same immunophenotype of villous adenomas and their associated adenocarcinomas was observed, but the number of the positive cells of p53 and Ki-67 staining were significantly increased in the area of adenocarcinomas than in that of the villous adenomas. CONCLUSIONS:Villous adenoma of the urinary tract is rare. It can occur in the urinary bladder, urachus, renal pelvis, ureter and urethra. These lesions may have malignant potential and frequently coexist with other malignant tumors. So, villous adenoma of the urinary tract should be removed completely and sampled thoroughly to avoid missing a more aggressive component. 10.3760/cma.j.issn.0529-5807.2013.07.002
    Carcinoembryonic antigen positive adenocarcinoma of a female urethral diverticulum: case report and review of the literature. Kato H,Ogihara S,Kobayashi Y,Toguri A G,Igawa Y,Nishizawa O International journal of urology : official journal of the Japanese Urological Association Paraurethral glands of the female urethra, which are assumed to be embryologically homologous to the male prostate gland, are possible origins for diverticular cancer of the urethra. A case of primary adenocarcinoma arising in a female urethral diverticulum is presented. Pathology revealed a columnar/mucinous type adenocarcinoma which stained positively for carcinoembryonic antigen (CEA) and negatively for PSA. Normal paraurethral ducts located near the urethra and normal urethral epithelium also stained positively for CEA. These findings suggest that the adenocarcinoma in our case originated from the paraurethral duct near the urethral lumen.
    Primary signet ring cell carcinoma of female urethra. Suzuki K,Morita T,Tokue A International journal of urology : official journal of the Japanese Urological Association A 69-year-old woman presented with difficulty in urination and bloody fluid discharge from the urethra. Based upon a diagnosis of primary urethral carcinoma, she underwent total cysto-urethrectomy and hysterectomy with pelvic lymph node dissection and urinary diversion of ileal conduit. Microscopically, the tumor was composed of mucinous adenocarcinoma and signet ring cell carcinoma. There was no recurrence 17 months after the surgery.
    Female urethral adenocarcinoma: immunohistochemical evidence of more than 1 tissue of origin. Murphy D P,Pantuck A J,Amenta P S,Das K M,Cummings K B,Keeney G L,Weiss R E The Journal of urology PURPOSE:Urethral adenocarcinoma is a rare malignancy whose origin remains controversial. The monoclonal antibody mAbDas1 (formerly 7E12H12) was developed against a unique colonic epithelial epitope and is reactive in areas of intestinal metaplasia. Recently the antibody was shown to react in cystitis glandularis as well as adenocarcinoma of the bladder, suggesting that cystitis glandularis may be the precursor of bladder adenocarcinoma. We examined urethral adenocarcinomas and benign urethral specimens using mAbDas1 to determine whether it could provide insight into their histogenesis. MATERIALS AND METHODS:Archival tissue from 12 cases of primary female urethral adenocarcinoma and urethral specimens of inflamed urethral mucosa, urethritis glandularis and transitional cell carcinoma was studied. Immunohistochemical analysis of formalin fixed, paraffin embedded archival tissue was done using the monoclonal antibody mAbDas1. Tumors were also evaluated with a prostate specific antigen (PSA) polyclonal antibody as previous studies have noted PSA reactivity in these tumors. RESULTS:Of the 12 cases 9 were columnar/mucinous adenocarcinoma, 2 clear cell adenocarcinoma and 1 a cribriform pattern resembling adenocarcinoma of the prostate. All columnar/mucinous adenocarcinomas reacted positively (6 strongly and 3 focally) with the mAbDas1 antibody but did not react with the PSA antibody. The tumor with a cribriform pattern reacted strongly with PSA but did not react with mAbDas1. The 2 clear cell adenocarcinomas did not react with either antibody. The benign urethral specimens demonstrated strong reactivity to the mAbDas1 antibody in areas of urethritis glandularis but normal and inflamed urethral mucosa and transitional cell carcinoma did not react. CONCLUSIONS:Primary adenocarcinoma of the female urethra arises from more than 1 tissue of origin. Columnar/mucinous adenocarcinomas of the female urethra and urethritis glandularis demonstrate consistent reactivity with the mAbDas1 antibody, suggesting that these tumors arise from glandular metaplasia analogous to the potential histogenesis previously demonstrated in the bladder. PSA reactivity occurred in 1 tumor with a cribriform pattern and likely represents origin from Skene's glands. Clear cell adenocarcinomas did not react with either antibody, suggesting a third possible pathway in the development of this rare subset of adenocarcinomas. 10.1016/s0022-5347(05)68833-7
    [A case of primary adenocarcinoma of the female urethra]. Seki H,Ukimura S,Mizutani Y,Kawauchi A,Nakao M,Miki T Hinyokika kiyo. Acta urologica Japonica A 53-year-old female visited our hospital with a complaint of acute urinary retention. Transvaginal ultrasonography, computed tomography and magnetic resonance imaging showed extreme thickening of the urethral wall. Histopathological examination of the transvaginal needle biopsy suggested well-differentiated adenocarcinoma. She underwent total cysto-urethrectomy with partial vaginal wall resection and ileal conduit urinary diversion. Pathological diagnosis of the tumor was columnar adenocarcinoma invading into the vaginal wall and periurethral connective tissue. Fifty-eight cases of primary adenocarcinoma of the female urethra in the Japanease literature are briefly reviewed.
    Surgical treatment for local control of female urethral carcinoma. Dimarco David S,Dimarco Connie S,Zincke Horst,Webb Maurice J,Bass Sarah E,Slezak Jeffrey M,Lightner Deborah J Urologic oncology We reviewed 53 patients (mean age 63 years) who underwent partial urethrectomy (n = 26) or radical extirpation (n = 27) for primary female urethral cancer from 1948 through 1999. Clinical stage, histology, high pathologic stage (3 or 4) and grade, tumor location, nodal status, surgery type, adjuvant therapy, and treatment decade were candidate outcome predictors. The predominant carcinomas were squamous cell (n = 21), transitional cell (TCC) (n = 15), and adenocarcinoma (n = 14). For adjuvant therapy, 20 patients had radiation (8 preoperatively), 2 had radiation + chemotherapy, and 1 had chemotherapy alone. During mean follow-up of 12.8 years, 27 patients had recurrence; 15 local only, 2 distant only and 10 local + distant. Of patients undergoing partial urethrectomy for pT1-3 tumors, 6/27 (22%) had urethral recurrence. Overall, there were no bladder recurrences. Recurrence-free survival +/- standard error (SE) at 10 years was 45 + 8%. Those who recurred had a cancer mortality rate of 71% at 5 years postrecurrence. The estimated 10-year cancer-specific survival (CSS) and crude survival (CS) rates were 60 +/- 8% and 42 +/- 7%, respectively. Pathologic stage was predictive for local recurrence (P = 0.02) and CSS (P = 0.01). Positive nodes on pathology were related to local and distant recurrence and CSS (P = 0.01). Upon review, partial urethrectomy resulted in a high urethral recurrence rate (22%) with no bladder recurrences. These patients may be better served with radical urethrectomy and creation of continent catheterizable stoma. 10.1016/S1078-1439(03)00174-1
    [Organ and function preservation in urethral cancer]. Tritschler S,Lellig K,Roosen A,Horng A,Stief C Der Urologe. Ausg. A Primary urethral carcinomas are rare tumors that can occur both in men and women. Histological patterns of these tumors are mixed, urothelial tumors occur as well as squamous cell tumors or adenocarcinomas.There are different clinical factors that define clinical prognosis, and the 1- and 5-year cancer-free survival is 75% and 54%. Therapy of locally limited disease is surgical resection, and organ-preserving treatment is possible if negative frozen sections prove complete surgical resection. However, in men a perineal urethrostomy might be necessary, and in women there is a high risk of urinary incontinence if more than 2 cm of the distal urethra is resected.In case of locally advanced tumors or tumors of the proximal urethra, a radical urethrectomy with supravesical urinrary diversion is necessary. In some cases neoadjuvant (radio-)chemotherapy may be an option. 10.1007/s00120-014-3555-8
    Pathologic Complete Response After Chemoradiation of a Massive Primary Urethral Carcinoma. Singh Tanmay,Li Qing Kay,Song Daniel Y Advances in radiation oncology 10.1016/j.adro.2019.02.005
    The treatment of primary urethral carcinoma--the dilemmas of a rare condition: experience with partial urethrectomy and adjuvant chemotherapy. Hakenberg O W,Franke H J,Froehner M,Wirth M P Onkologie BACKGROUND:Primary urethral carcinoma is a very rare condition, and no large-scale experience with such cases has been published. Treatment will therefore have to follow rules established for the treatment of similar conditions. PATIENTS:Six cases of primary urethral carcinoma (5 male, 1 female) who had been treated at our institution between 1995 and 1999 were retrospectively analyzed. In 3 male cases, a primary urothelial carcinoma of the distal urethra was treated by distal urethrectomy only. In 3 other cases with locally advanced tumors and/or lymph node metastases surgical treatment was followed by adjuvant cisplatinum-containing chemotherapy. RESULTS:In the 3 cases with distal urethral carcinoma, partial urethrectomy with preservation of the penis resulted in cure, with a follow-up of 12-71 months. In the cases with advanced disease, adjuvant chemotherapy after surgery has resulted in complete remissions in all 3 cases, with a follow-up of 4-47 months at present. CONCLUSIONS:In localized, noninvasive carcinoma of the distal male urethra, partial urethrectomy seems adequate and the avoidance of penile amputation justified. In advanced cases, after local excision and lymphadenectomy adjuvant chemotherapy which by necessity must follow the guidelines established for the treatment of other urothelial or squamous cell malignancies seems to be beneficial. 10.1159/000050282
    Non-surgical treatment of primary female urethral cancer. Libby Bruce,Chao David,Schneider Bernard F Rare tumors Primary carcinomas of the female urethra are extremely rare, with an annual incidence of less than ten in one million. Currently, there is no consensus regarding management of this malignancy. However, there have been several case reports demonstrating the efficacy of chemoradiation in the treatment of female urethral cancer. In this report we present two cases of female primary urethral adenocarcinoma that were treated by concomitant chemotherapy and external beam radiotherapy, followed by interstitial brachytherapy. 10.4081/rt.2010.e55
    Cytologic findings of clear cell adenocarcinoma of the urethra: a case report. Han Jee-Young,Kim Kyu-Ho,Kim Lucia,Choi Suk-Jin,Park In-Suh,Kim Joon-Mee,Chu Young-Chae,Yoon Sang-Min Korean journal of pathology Clear cell adenocarcinoma of the urethra is a rare disease entity with an uncertain histogenesis. Here, we present a case of primary clear cell adenocarcinoma of the female urethra with its cytological findings. A 54-year-old woman presented with a painless gross hematuria lasting 3 months. On vaginal sonography, there was a sausage-like, elongated mass in the urethra, measuring 3.8×4.3 cm. The voided urine cytology revealed small clusters of rounded or papillary cells. The necrotic debris and inflammatory cells were present within some clusters of tumor cells. These tumor cells were enlarged and had abundant clear or granular cytoplasm with cytoplasmic vacuoles. The nucleus was granular and contained vesicular chromatin with prominent nucleoli. The hobnail cells and hyaline globules were also present as in a histologic section. The histologic findings were compatible with clear cell adenocarcinoma. The tumor showed distinctive cytological features. Cytologically, however, it is necessary to make a differential diagnosis from other adenocarcinoma or high-grade urothelial carcinoma. 10.4132/KoreanJPathol.2012.46.2.210
    [Two cases of primary female urethral cancer]. Koizumi Takahiro,Bando Shigehiro,Kanda Kazuya,Inai Tooru Nihon Hinyokika Gakkai zasshi. The japanese journal of urology Herein, we report two cases of female urethral cancer. Case 1 presented with acute urinary retention and case 2 presented with a painful perineal mass. Magnetic resonance imaging (MRI) revealed a urethral tumor in both cases. Histopathological examination of transperineal biopsy specimens from both patients suggested clear cell adenocarcinoma in case 1 and squamous cell carcinoma in case 2. Both cases underwent total urethrectomy with partial resection of the vaginal wall and cystostomy urinary diversion. With reference to case 1, obturator lymph node metastases were observed during surgery, and treatment comprised combined radiotherapy to 60 Gy and chemotherapy with 5-fluorouracil and cisplatin following surgery. However, metastases appeared in the lung 6 months after initial treatment and she died 20 months after surgery. For case 2, tumor marker failure was observed 5 months after surgery. The same combined treatment was performed and a complete response was obtained. At 19 months after surgery, the patient showed no evidence of recurrence.
    Primary proximal urethral adenocarcinoma: Case report and brief review. Siosaki Marcos Duarte,Machado Roberto Dias,Souza Ana Tarsila Fonseca,Magnabosco Wesley Justino,Santos Alexandre Cesar,Gonçalves Fernando Zapparoli,Pereira Fernando Coutinho,de Araújo Silva Carlos Flávio,Faria Eliney Ferreira Canadian Urological Association journal = Journal de l'Association des urologues du Canada Primary urethral cancer in females is rare. It has a poor prognosis. The published data on this topic are limited, composed mostly of small case series. This paper presents a case of an advanced adenocarcinoma of the urethra, intestinal type, treated with anterior exenteration. 10.5489/cuaj.399
    [Adenocarcinoma of the female urethra: report of two cases and bibliography review]. Feltes Ochoa Javier Amalio,Blanco Carballo Ovidio,Sánchez Angel Tejido,Villacampa Aubá Felipe,Conde Gallego Esther Archivos espanoles de urologia OBJECTIVES:Primary adenocarcinoma of the female urethra is a rare malignancy. We report two cases and a review of the latest articles focused on classification and treatment of this kind of neoplasm. METHODS:We present two females diagnosed of urethral adenocarcinoma, describing clinic and pathological features, diagnosis and treatment. CONCLUSIONS:Female urethral adenocarcinoma is an uncommon neoplasm with a heterogeneous histogenesis. The distal urethral carcinoma is more amenable to treatment, and the prognosis is better than that of proximal or entire urethral carcinoma, which is often associated with extensive local invasion and metastasis. 10.4321/s0004-06142005000700015
    Primary clear-cell adenocarcinoma of the proximal female urethra: case report and review of the literature. Miller Joe,Karnes R Jeffrey Clinical genitourinary cancer Primary carcinoma of the female urethra is a rare entity. Adenocarcinomas account for only 10% of cases. Clear cell adenocarcinoma (CCA) of the urethra is more common among women than men.1 We report a case of primary CCA of the urethra and review the literature concerning the histogenesis, management, and outcome of this uncommon tumor. 10.3816/CGC.2008.n.022
    Survival Outcomes and Predictive Factors for Female Urethral Cancer: Long-term Experience with Korean Patients. Kang Minyong,Jeong Chang Wook,Kwak Cheol,Kim Hyeon Hoe,Ku Ja Hyeon Journal of Korean medical science The aim of this study was to evaluate female urethral cancer (UCa) patients treated and followed-up during a time period spanning more than 20 yr at single institution in Korea. We reviewed medical records of 21 consecutive patients diagnosed with female UCa at our institution between 1991 and 2012. After exclusion of two patients due to undefined histology, we examined clinicopathological variables, as well as survival outcomes of 19 patients with female UCa. A Cox proportional hazards ratio model was used to identify significant predictors of prognosis according to variables. The median age at diagnosis was 59 yr, and the median follow-up duration was 87.0 months. The most common initial symptoms were voiding symptoms and blood spotting. The median tumor size was 3.4 cm, and 55% of patients had lesions involving the entire urethra. The most common histologic type was adenocarcinoma, and the second most common type was urothelial carcinoma. Fourteen patients underwent surgery, and 7 of these patients received adjuvant radiation or systemic chemotherapy. Eleven patients experienced tumor recurrence after primary therapy. Patients with high stage disease, advanced T stage (≥T3), and positive lymph nodes had worse survival outcomes compared to their counterparts. Particularly, lymph node positivity and advanced T stage were significant predictive factors for all survival outcomes. Tumor location was the only significant predictor for recurrence-free survival. Although our study included a small number of patients, it conveys valuable information about this rare female urologic malignancy in a Korean population. 10.3346/jkms.2015.30.8.1143
    Adenocarcinoma of the urethra with mucinous features. Satyanarayan Arthi,Redd Lucas,Dyer Anthony,Wright Andrew,Walker Jonathan Reviews in urology Primary adenocarcinoma of the female urethra is a rare malignancy. Previous studies hypothesize multiple origins, including periurethral glands or intestinal metaplasia. We report a case of a 60-year-old white woman with adenocarcinoma of the urethra who initially presented with obstructive voiding complaints secondary to a urethral mass. Wide local excision revealed invasive adenocarcinoma of the urethra with mucinous features. There was intestinal metaplasia adjacent to the tumor, as well as separate identification of intestinal metaplasia along the urethra. Ultimately, the patient underwent radical cystectomy with ileal conduit urinary diversion with no evidence of recurrence, indicating the role of early identification and surgical intervention for such cases.
    Locally advanced female urethral adenocarcinoma of enteric origin: the role of adjuvant chemoradiation and brief review. Chen Ling-Ping,Lin Shyh-Jer,Fu Ting-Ying,Yu Ming-Sun The Kaohsiung journal of medical sciences Primary female urethral adenocarcinoma (FUA) is rare and has a poor prognosis. The common manifestations include urethrorrhagia, urinary frequency, dysuria, urethral obstructions, focal tenderness, and urinary tract infection. These symptoms are neither diagnostic nor pathognomonic; therefore, a delay in diagnosis and even a misdiagnosis is hardly uncommon. The histogenesis of FUAs may have derived from urethritis glandularis, Mullerian ducts, Skene's glands, or mixed origins. Tumors of different embryologic origins displayed heterogeneous pathological morphology and immunohistochemistical phenotypes. Because of its rarity and the lack of large-scale studies, there is no current consensus on the optimal treatment of urethral adenocarcinomas. Here, we report two cases of locally advanced FUA of enteric origin. They manifested as slightest warning symptoms of urinary tract infection and stress urinary incontinence, respectively. One patient died of disease progression 2 months after curative operation. The other patient underwent surgery followed by adjuvant irinotecan-containing chemoradiation, and the effect was at least modest. Hence, we recommend adjuvant chemoradiation in locally advanced FUA. Individualizing cancer care of chemoregimens in accordance with the tumor origins may probably be beneficial in FUAs. 10.1016/j.kjms.2010.12.009
    [Primary adenocarcinoma of the female urethra treated by multimodal therapy]. Tanaka Hajime,Masuda Hitoshi,Komai Yoshinobu,Yokoyama Minato,Iwai Aki,Numao Noboru,Sakai Yasuyuki,Saito Kazutaka,Fujii Yasuhisa,Kobayashi Tsuyoshi,Kawakami Satoru,Kihara Kazunori Hinyokika kiyo. Acta urologica Japonica A 64-year old female presented with urinary retention. Physical examination revealed a firm mass on the anterior vaginal wall. Magnetic resonance imaging showed a tumor surrounding the urethra, which invaded to the vesical triangle and the anterior vaginal wall. Serum levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were elevated, but squamous cell carcinoma antigen and prostate specific antigen were within normal limits. Pathological examinations of the transurethral and transvaginal needle biopsy specimen suggested mucinous adenocarcinoma. First, the patient received local chemoradiotherapy and systemic chemotherapy using a fluoropyrimidine drug TS-1 and cisplatin. The tumor markers declined to within normal limits after this preoperative therapy. Then she underwent total cysto-urethrectomy with anterior vaginal wall resection, pelvic lymphadenectomy, and urinary diversion with ureterocutaneous fistula. Histopathological examination of the surgical specimen showed mutinous adenocarcinoma invading to the vesical triangle and the anterior vaginal wall. No metastasis was found in the lymph nodes. The final diagnosis was urethral adenocarcinoma, pT4N0, Stage IV. Five months after surgery, local recurrence and distant metastases appeared, and she died 14 months after surgery.
    Clear cell adenocarcinoma of female urethra: A case report. Hartman Kasondra,Li Jinghong,Garg Tullika Urology case reports Primary malignancies of the female urethra are rare, accounting for less than 1% of genitourinary malignancies. Clear cell adenocarcinoma of the urethra (CCAU) occurs more infrequently, accounting for 0.003% of malignancies of the female urogenital tract. Definitive clinical diagnosis of CCAU is difficult and must be differentiated from tumors of the vagina. Currently, there is limited understanding of the causes of CCAU and there is no established standard of care for treatment. Immunohistochemistry and pathologic analysis can be used to identify the origin of the tumor. 10.1016/j.eucr.2018.04.008
    Clear Cell Adenocarcinoma of Female Urethra. Rane Sharada Raju,Ghodke Ashwini Nivrutti,Vishwasrao Sharvari Journal of clinical and diagnostic research : JCDR Primary malignancies of female urethra are infrequent, constituting a fraction of less than 1% of genitourinary malignancies. Primary clear-cell adenocarcinoma of the urethra, is even rarer, that histomorphologically resembles clear-cell carcinoma of the female genital tract, occurs predominantly in women and is associated with a relatively poor prognosis. The histogenesis of this rare urethral neoplasm has not been completely determined. Various hypotheses concerning the origin have been postulated, including (1) diverticular origin (2) mullerian origin (3), glandular differentiation of urothelium or urothelial carcinoma. Here, we report a case of 67-year-old female with obstructive urinary symptoms and pain in abdomen, diagnosed with adenocarcinoma of urethra. Immunohistochemistry (IHC) workup of the tumour was done to find the origin of the tumour. 10.7860/JCDR/2017/25261.10127
    [Combined Chemotherapy with Radiation was Tolerable and Effective Treatment in Female Octogenarian Patients with Urethral Cancer -Two Case Reports]. Tachibana Takashi,Matsumoto Kazumasa,Nagi Shoji,Hagiwara Masahiro,Kobayashi Kentaro,Tsumura Hideyasu,Yoshida Kazunari,Iwamura Masatsugu Hinyokika kiyo. Acta urologica Japonica We report two octogenarian patients with primary urethral cancer treated with chemotherapy and external beam radiation therapy. An 85-year-old female presented with perineal bleeding. Magnetic resonance imaging (MRI) showed a locally advanced tumor in the urethra. Biopsy was performed and pathologic findings demonstrated squamous cell carcinoma. After receiving one cycle of a half dose of gemcitabine and nedaplatin, the patient received external beam radiation therapy with gemcitabine and nedaplatin treatment followed by two more cycles of chemotherapy. Complete response was achieved. An 87-year-old female presented with vaginal bleeding. MRIrevealed locally advanced urethral tumor with bilateral inguinal lymph node metastases. Scratch and urine cytology of tumor demonstrated squamous cell carcinoma. After the same treatment as in case 1, primary cancer and lymph node metastases were significantly decreased. There have been no signs of recurrence or progression after treatment, and no severe adverse events in either patient during 53 and 26 months'follow up, respectively. 10.14989/ActaUrolJap_62_7_367
    Management of Proximal Primary Urethral Cancer: Should Multidisciplinary Therapy Be the Gold Standard? Zinman Leonard N,Vanni Alex J The Urologic clinics of North America Primary urethral cancer (PUC) is a rare, but devastating genitourinary tumor that affects men and women. Although most PUC are localized, proximal PUC frequently presents with locally advanced disease, with 30% to 40% having lymph node metastasis. Single modality surgical or radiation therapy has dismal results. Multimodal therapy with cisplatin-based chemotherapy and consolidation surgery has greatly improved the local recurrence and overall survival rates for this aggressive disease. In locally advanced squamous cell carcinoma of the urethra, radiotherapy combined with radiosensitizing chemotherapy is an option for genital preservation. Prospective, multi-institutional studies are required to further define the optimal multidisciplinary treatment strategy for this destructive disease. 10.1016/j.ucl.2016.06.011
    Retrospective analysis of survival outcomes and the role of cisplatin-based chemotherapy in patients with urethral carcinomas referred to medical oncologists. Dayyani Farshid,Pettaway Curtis A,Kamat Ashish M,Munsell Mark F,Sircar Kanishka,Pagliaro Lance C Urologic oncology OBJECTIVES:Primary carcinomas of the urethra (PCU) are rare and often advanced when diagnosed. Treatment standards are lacking. We studied treatment response and survival in a cohort of patients with PCU, with emphasis on modern platinum-containing chemotherapy regimens plus surgery for advanced disease. MATERIALS AND METHODS:This was a retrospective chart review of consecutive patients with PCU seen by medical oncologists at our institution over a recent 5-year period. Outcome was measured as best response to chemotherapy. Kaplan-Meier estimates were generated for survival and Cox proportional hazard was used for prognostic factors for survival. RESULTS:The 44 patients (64% women) included had a median age at diagnosis of 66.5 years. The most prevalent histologic subtypes of PCU were squamous cell carcinoma and adenocarcinoma. At diagnosis, 43% already had lymph node-positive [lymph node (LN)+] disease, and 16% had distant metastases. The entire cohort's overall survival (OS) was 31.7 months. The response rate to platinum-containing neoadjuvant chemotherapy was 72%. Twenty-one patients with locally advanced or LN+ PCU underwent chemotherapy plus surgery. Their median OS from chemotherapy initiation was 25.6 months. Four of 9 patients (44%) with LN+ PCU at diagnosis were alive at our review, with a minimum follow-up of more than 3 years. CONCLUSIONS:Modern platinum-containing regimens appear to be effective in advanced PCU. Preoperative chemotherapy is associated with prolonged disease-free survival in a subgroup of LN+ cases. 10.1016/j.urolonc.2012.01.011
    Survival Outcomes Associated With Female Primary Urethral Carcinoma: Review of a Single Institutional Experience. Peyton Charles C,Azizi Mounsif,Chipollini Juan,Ercole Cesar,Fishman Mayer,Gilbert Scott M,Juwono Timothy,Lockhart Jorge,Poch Michael,Pow-Sang Julio M,Spiess Philippe E,Wiegand Lucas,Sexton Wade J Clinical genitourinary cancer BACKGROUND:Primary urethral carcinoma (PUC) is rare, and standard treatment recommendations are lacking. We examined the variation in treatments and survival outcomes of female PUC at a single, tertiary referral cancer center. METHODS:Records of women with PUC referred to our multidisciplinary genitourinary oncology service between 2003 and 2017 were reviewed. Clinical, demographic, pathologic, primary and salvage therapy details, and overall (OS) and recurrence-free survival (RFS) were recorded. Survival outcomes were analyzed for the entire cohort, and cases of locally-advanced (≥ T2 tumor), non-metastatic PUC were evaluated according to treatment intensity. Multimodal treatment (cystourethrectomy + concomitant therapy) was compared with non-multimodal therapy. Contingency analyses and Kaplan-Meier estimates were performed. RESULTS:Thirty-nine women with PUC were identified. In total, median OS was 36 months (95% confidence interval, 10.6-61.4 months). Twenty-four had T3 to T4 disease, 12 were node-positive, and 3 had distant metastases. Histology included 22 adenocarcinomas, 11 urothelial, 5 squamous, and 1 neuroendocrine. Patients with locally advanced, non-metastatic disease (n = 25) had significantly reduced OS (36 vs. 99 months; P = .016) and RFS (46 months vs. unmet; P = .011) compared with patients with locally confined tumors. Approximately one-half of locally advanced cases were managed with multimodal therapy (4 with neoadjuvant therapy + cystourethrectomy, 8 with cystourethrectomy + adjuvant therapy, and 1 with chemoradiation + consolidative cystourethrectomy). Multimodal therapy had nonsignificant longer OS (36 vs. 16 months) and RFS (58 vs. 16 months), P > .05. CONCLUSIONS:Locally advanced female PUC has relatively poor survival outcomes. Although we observed a nonsignificant interval improvement in survival with multimodality therapy, the treatment paradigm is inconsistent. Because it is a rare disease, collaborative multi-institutional studies are needed. 10.1016/j.clgc.2018.05.012
    [Primary carcinoma of the female urethra: report of 4 cases]. Yamada Yuta,Takahashi Atsuko,Kanemura Mikio,Minowada Shigeru,Homma Yukio Nihon Hinyokika Gakkai zasshi. The japanese journal of urology We experienced 4 cases of primary carcinoma of the female urethra during 1998 to 2011. All of the cases were diagnosed primary urethral cancer according to tumor biopsy, cystoscopy, and computed tomography (CT) or magnetic resonance imaging (MRI). Patients were between 66 to 92 years of age at the time of presentation. Presenting symptoms included gross hematuria in 1 case, urinary retention in another case, and vulvar bleeding in 2 cases. Pathology showed urothelial carcinoma in 2 cases, adenocarcinoma in 1 case, and squamous cell carcinoma in another case. There were 2 patients with stage D, 1 patient with C, and another with B. Three patients were treated with total cystectomy and ileal conduit. One patient was treated with radiation. Two patients died from urethral cancer, 1 patient is free from disease for 12 months, and another patient became lost during follow-up.
    Management of primary adenocarcinoma of the female urethra: Report of two cases and review of the literature. Wang Xinjun,Bai Peiming,Su Hanzhong,Luo Guangcheng,Zhong Zhaohui,Zhao Xiaokun Oncology letters Primary adenocarcinoma of the female urethra is rare and only a few retrospective cases have been published. The origin of urethral adenocarcinomas remains unclear. Certain authors have suggested that urethral adenocarcinomas in females originate at the periurethral Skene's glands. We report one case of urethral adenocarcinoma of the proximal urethra in a 44-year-old female who presented with painless urethral bleeding. Abdominal and pelvic CT scan with contrast and chest radiology were unremarkable. Biopsy of the mass revealed adenocarcinoma of the urethra. The patient was treated with partial urethrectomy and was free of disease for more than 5 years. We also present another rare case of mucinous adenocarcinoma in a 52-year-old female who complained of an enlarged urethral mass. Pelvic MRI revealed a tumor surrounding the whole urethra and bilateral inguinal lymph nodes. A puncture biopsy later revealed that the tumor was mucinous adenocarcinoma. Anterior pelvic exenteration with pelvic and bilateral lymph node dissection was performed and chemotherapy was administered. The patient was followed up for 12 months and did not experience local recurrence or distant metastasis. In conclusion, for the diagnosis of urethral cancer, a biopsy is necessary for any suspicious urethral lesions. MRI is recommended for tumor staging. Small, superficial, distal urethral tumors may be treated with excision of the distal urethra. For advanced female urethral cancer, a combination of chemotherapy, radiation therapy and surgery is recommended for optimal local and distant disease control. Regular follow-up is required in these patients. 10.3892/ol.2012.886
    [A case of primary urethral adenocarcinoma accompanied by vaginal wall infiltration in which the CA19-9 level was very high]. Maekawa Shigekatsu,Hishima Tsunekazu,Yamada Yukio,Ichikawa Hiroki,Natsui Shinsuke,Shinohara Mitsuru Hinyokika kiyo. Acta urologica Japonica A 55-year-old woman had urinary frequency and a constant urge to urinate. Computed tomography confirmed a urethral tumor, and transurethral biopsy confirmed adenocarcinoma. She visited our hospital to undergo treatment, and we performed an anterior pelvic excenteration. On histology, the tumor had spread to the bladder, urethra, and vagina. However, the majority of the tumor was located in the bladder and urethra, a duct with intestinal metaplasia was present around the urethra, and carcinoma in situ was seen in the urethral mucosa. Based on the above findings, the patient was diagnosed as having primary urethral adenocarcinoma. No tumor cells were seen in the resection stump. Six months after surgery, the patient developed bone metastasis, followed by peritoneal and pleural dissemination, as well as multiple lung metastases. The patient died nine months after surgery. In the present patient, the carbohydrate antigen (CA) 19-9 level changed with the clinical course, and it was a useful marker. Urethral tumor is relatively rare. A urethral tumor accompanied by vaginal wall infiltration is likely to be mistaken for a primary vaginal tumor. It was very difficult to identify the primary organ in our case. To the best of our knowledge, the present patient is the sixth reported case of primary urethral carcinoma accompanied by vaginal wall infiltration in Japan. The six reported cases are compared and analyzed.
    Management of advanced primary urethral carcinomas. Dayyani Farshid,Hoffman Karen,Eifel Patricia,Guo Charles,Vikram Raghu,Pagliaro Lance C,Pettaway Curtis BJU international Primary urethral carcinoma (PUC) is a rare malignancy accounting for <1% of genitourinary cancers, with a predilection for men and African-Americans. The sites and histology of urethral carcinoma vary by gender and anatomical location. Squamous cell carcinoma is most common among both genders but adenocarcinomas are noted in 15-35% of cases among women. Obstructive or irritative symptoms and haematuria are common modes of presentation. Clinical evaluation includes cystourethroscopy with biopsy and examination under anaesthesia. Magnetic resonance imaging provides a highly effective method to image the primary tumour while defıning the potential involvement of surrounding structures. Most tumours are localised, with regional metastases to nodal sites seen in up to 30% of cases in both genders, while distant metastases at presentation are rare (0-6%), but occur in up to 40% of cases with recurrent disease. Among men, the two most important prognostic factors are disease location and stage. Low-stage tumours (T1-2) and tumours involving the fossa navicularis or the penile urethra have a better prognosis than higher stage tumours (>T2 or N+) and lesions involving the bulbomembranous urethra. In women, in addition to stage and location, the size of the tumour has also prognostic implications. While surgery and radiation therapy (RT) are of benefit in early stage disease, advanced stage PUC requires multimodal treatment strategies to optimise local control and survival. These include induction chemotherapy followed by surgery or RT and concurrent chemoradiation with or without surgery. The latter strategy has been used successfully to treat other human papillomavirus-related cancers of the vagina, cervix and anus and may be of value in achieving organ preservation. Given the rarity of PUC, prospective multi-institutional studies are needed to better define the optimal treatment approach for this disease entity. 10.1111/bju.12630
    Current Disease Management of Primary Urethral Carcinoma. Janisch Florian,Abufaraj Mohammad,Fajkovic Harun,Kimura Shoji,Iwata Takehiro,Nyirady Peter,Rink Michael,Shariat Shahrokh F European urology focus CONTEXT:Primary urethral cancer (PUC) is a rare cancer entity. Owing to the low incidence of this malignancy, the main body of literature consists mainly of case reports, making evidence-based management recommendations difficult. OBJECTIVE:To review reported disease management strategies of PUC and their impact on oncological outcomes. EVIDENCE ACQUISITION:A systematic research was performed according to the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement using Medline, Scopus, and Web of Science, to find studies of the past 10yr including ≥20 patients, and investigating treatment strategies and their impact on outcomes of the three most frequent histologies: urothelial carcinoma, adenocarcinoma, and squamous cell carcinoma. EVIDENCE SYNTHESIS:In localized PUC, penis-sparing surgery can be performed in males, while in females, complete urethrectomy with surrounding tissue is advised to minimize recurrence due to positive margins. Radiotherapy (RT) has worse survival and recurrence rates, as well as more adverse effects, than surgery, limiting its use in genital-preserving therapy. Locally advanced PUC should be treated with multimodal therapy, as monotherapies result in inferior recurrence and survival rates. Extent of surgery is still undecided, favoring radical cyst(oprostat)ectomy with total urethrectomy (RCU). Lymph node involvement is a predictor of survival, highlighting the role of lymph node dissection for disease control and staging. RT can improve survival in combination with surgery and/or chemotherapy (CHT). Neoadjuvant platinum-based CHT can improve overall and recurrence-free survival. At recurrence, salvage therapy with surgery and/or CHT can improve survival. Superficial urothelial carcinoma of the prostatic urethra can be treated with transurethral resection. Stromal invasion often features concomitant bladder cancer with a poor prognosis and requires RCU with or without systemic preoperative CHT. CONCLUSIONS:PUC is a rare malignancy with an often poor natural course, requiring a stage- and gender-specific risk-based treatment strategy. The role of systematic perioperative CHT and the extent of surgery are becoming more important. PATIENT SUMMARY:In this review, we looked at the treatment options for primary urethral cancer. We found that while an organ-confined disease can be managed with local resection, growth beyond the organ border makes a combination of different treatment modalities, such as surgery and systematic chemotherapy, necessary to improve outcomes. 10.1016/j.euf.2019.07.001
    Primary urethral carcinoma in females: an epidemiologic study on demographical factors, histological types, tumour stage and survival. Derksen Joris W,Visser Otto,de la Rivière Guy Brutel,Meuleman Eric J,Heldeweg Eddi A,Lagerveld Brunolf W World journal of urology PURPOSE:To obtain insight into demographical factors, histology and survival rates of females diagnosed with primary urethral cancer and to determine favourable treatment. METHODS:Data from 91 females with primary urethral carcinoma, age varying from 15 to 85 years, registered between 1989 and 2008 at the National Cancer Registry of the Netherlands were used for this study. Demographical factors, incidence rate, morphology and tumour stage according to TNM classification were analysed. Kaplan-Meier survival curves were constructed and stratified by stage, histological type and treatment modality. RESULTS:The overall crude annual incidence was 0.7 per million women with a peak incidence in the age group of 80-84 years. Analysis of the morphology showed urothelial cell carcinoma (UCC) in 45 %, squamous cell carcinoma (SCC) in 19 %, adenocarcinoma (AC) in 29 %, and unknown or undifferentiated carcinoma accounted for 6 %. Almost half of patients (46 %) had advanced disease at time of diagnosis and was mainly treated with surgery and/or radiotherapy. The 5-year survival rates of stage 0-II, stage III and stage IV were 67, 53 and 17 %, respectively. The 5-year survival rates of SCC, UCC and AC were 64, 61 and 31 %, respectively. CONCLUSIONS:Female primary urethral carcinoma is a rare condition, and the majority of patients were aged above 65 years. Almost half of patients have advanced disease upon diagnosis. TNM stage and histological type of disease are the most determining factors for survival. Extended surgery with or without radiotherapy seems to be the most favourable treatment. Awareness and early diagnosis are important to improve survival. 10.1007/s00345-012-0882-5
    Docetaxel, cisplatin and 5-fluorouracil chemotherapy with concurrent radiation for unresectable advanced urethral carcinoma. Itoh Jun,Mitsuzuka Koji,Kimura Shingo,Tanaka Takaki,Yamamuro Taku,Yamashita Shinichi,Yamada Shigeyuki,Saito Hideo,Kaiho Yasuhiro,Arai Yoichi International journal of urology : official journal of the Japanese Urological Association The present case report describes two cases of unresectable advanced primary urethral carcinoma. Case 1 was a 61-year-old man with squamous cell carcinoma of the urethra that directly invaded into the perineal skin. Case 2 was a 64-year-old woman with adenocarcinoma, and solitary lung and lymph node metastases. Both patients received docetaxel/cisplatin/5-fluorouracil chemotherapy with concurrent radiation. The docetaxel/cisplatin/5-fluorouracil chemotherapy regimen consisted of docetaxel (75 mg/m(2) ) on day 2, cisplatin (100 mg/m(2) ) on day 2 and 5-fluorouracil (1000 mg/m(2) ) on days 1 to 5 and was repeated every 4 weeks. Complete response was achieved after two cycles of chemotherapy combined with radiation in case 1. In case 2, complete response was achieved locally after six cycles of chemotherapy combined with radiation, but the solitary lung lesion remained viable. We experienced two cases with advanced urethral carcinoma for whom docetaxel/cisplatin/5-fluorouracil chemotherapy chemotherapy with radiation was effective. 10.1111/iju.12333
    Primary urethral clear-cell adenocarcinoma: comprehensive analysis by surgical pathology, cytopathology, and next-generation sequencing. Mehra Rohit,Vats Pankaj,Kalyana-Sundaram Shanker,Udager Aaron M,Roh Michael,Alva Ajjai,Pan Jincheng,Lonigro Robert J,Siddiqui Javed,Weizer Alon,Lee Cheryl,Cao Xuhong,Wu Yi-Mi,Robinson Dan R,Dhanasekaran Saravana M,Chinnaiyan Arul M The American journal of pathology Primary clear-cell adenocarcinoma of the urethra, a rare tumor that histomorphologically resembles clear-cell carcinoma of the female genital tract, occurs predominantly in women and is associated with a relatively poor prognosis. The histogenesis of this rare urethral neoplasm has not been completely resolved, but it is thought to arise from either müllerian rests or metaplastic urothelium. Herein, we present comprehensive surgical pathological and cytopathological findings from a patient with primary urethral clear-cell adenocarcinoma and describe next-generation sequencing results for this patient's unique tumor-the first such reported characterization of molecular aberrations in urethral clear-cell adenocarcinoma at the transcriptomic and genomic levels. Transcriptome analysis revealed novel gene fusion candidates, including ANKRD28-FNDC3B. Whole-exome analysis demonstrated focal copy number loss at the SMAD4 and ARID2 loci and 38 somatic mutations, including a truncating mutation in ATM and a novel nonsynonymous mutation in ALK. 10.1016/j.ajpath.2013.11.023
    Clear cell adenocarcinoma of the urethra with inguinal lymph node metastases: A rare case report and review of literature. Mallya Varuna,Mallya Ashwin,Gayathri J Journal of cancer research and therapeutics Urethral cancer is a rare malignancy with urothelial subtype being the most common followed by adenocarcinoma. In women, the usual presentation is urinary retention and hematuria. Clear cell variety of adenocarcinoma is a rarer entity which usually has a better prognosis than other variants. Lymph node metastasis is seen in about 30% of urethral cancers. Histopathological differential diagnosis includes Skene gland carcinoma and nephrogenic adenoma. Herein, we present a 58-year-old female patient who presented for evaluation of lymph node mass in the inguinal region. The detailed evaluation revealed clear cell adenocarcinoma of the urethra as the primary with lymph node metastasis. 10.4103/0973-1482.226734
    Primary enteric-type mucinous adenocarcinoma of the urethra in a patient with ulcerative colitis. Dimitroulis Dimitrios,Patsouras Dimitrios,Katsargyris Athanasios,Charalampoudis Petros,Anastasiou Ioannis,Kouraklis Gregory International surgery Primary carcinoma of the male urethra accounts for less than 1% of malignancies in men. Mucinous adenocarcinoma of the urethra is extremely rare, and its biologic behavior is poorly understood. We present herein a rare case of mucinous urethral adenocarcinoma in a male patient with longstanding ulcerative colitis and multiple sclerosis. The patient presented with a voluminous pelvic mass; core biopsy of the lesion demonstrated a mucus-producing adenocarcinoma. Given the patient's history of subtotal colectomy, preoperative diagnosis was oriented towards a rectal stump adenocarcinoma. The patient underwent a pelvic exenteration: surprisingly, histology marked the prostatic urethra as the primary lesion site. 10.9738/INTSURG-D-13-00073.1
    Multimodal management of primary adenocarcinoma of the female urethra: About four cases. Deberne M,Timsit M-O,Verkarre V,Eiss D,Kreps S,Dupont S,Housset M Cancer radiotherapie : journal de la Societe francaise de radiotherapie oncologique PURPOSE:To retrospectively analyse female patients treated for urethral adenocarcinoma, modalities of treatment and long-term outcomes. PATIENTS:Four cases of primary female urethral adenocarcinoma were treated in the departments of urology and radiation-oncology at Georges-Pompidou and Necker hospitals (France) over a 7-year period. RESULTS:All of them underwent surgery, with three presenting stage pT3-pT4 and one a positive cytology on inguinal node. Three patients received adjuvant cisplatin-based chemoradiotherapy up to 60Gy, and one preoperative chemoradiotherapy at 45Gy. Two recurrences were observed: one local relapse occurred at 9months from the diagnosis and was treated by anterior pelvic exenteration followed by chemoradiotherapy, with no recurrence. One tumour relapsed both at the local level and on distant metastatic sites at 9months from the diagnosis, and died 21months after this progression. Median survival and progression-free survival are respectively 4.2years and 13months. Three patients are alive at 7, 4.5 and 3years from diagnosis. CONCLUSION:Female urethral adenocarcinoma is a very rare entity and often present in locally advanced stages. Initial extensive surgery with pelvic exenteration should be considered, followed by chemoradiotherapy according to the surgical margins and lymph nodes involvement. 10.1016/j.canrad.2015.11.005
    Primary mucinous adenocarcinoma of the female urethra: a contemporary clinicopathologic analysis. Harari Saul E,Cheng Liang,Osunkoya Adeboye O Human pathology Primary mucinous adenocarcinoma of the female urethra is very rare and may lead to both diagnostic and therapeutic challenges. Although primary mucinous adenocarcinoma of the prostate and prostatic urethra has been well characterized in men, this is the largest clinicopathologic study to date of primary mucinous adenocarcinoma of the female urethra. A search was made through the files of 2 major academic institutions for cases of confirmed primary mucinous adenocarcinoma arising from the female urethra. Tumors arising from adjacent organs were excluded both clinically and pathologically in all cases. Five cases were identified. The mean patient age was 67 years (range, 54-74 years). All patients presented with a polypoid/papillary mass arising from the urethra. Pathologic stages were as follows: pT4 3 (60%) of 5 cases; pT3 1 (20%) of 5 cases, and pT2 1 (20%) of 5 cases. Immunohistochemical stains for GATA3, p63, CK7, CK20, CDX2, ER, PAX8, and β-catenin were performed on all cases. Immunohistochemical stains were positive in the tumor cells for CDX2 in 4/5 (80%) cases; focally positive for CK20 in 4/5 (80%) cases; focally positive for CK7 in 4/5 cases (80%); and negative for p63, GATA3, ER, PAX8 and β-catenin in all cases. In the 4 patients with available follow-up data, mean follow-up was 25 months (range, 4-54 months). It is critical for pathologists to be aware of this entity in light of potential diagnostic pitfalls and therapeutic implications. 10.1016/j.humpath.2015.09.014
    Female urethra adenocarcinoma: report of two cases and bibliographic review. García-Barreras Silvia,Fiter-Gómez Luis,Tellez-Martínez Fornés Miguel Archivos espanoles de urologia OBJECTIVE:Primary cancer of female urethra is rare and represents about 0.02% of all neoplasias found in women and the majority of them are squamous cell carcinomas. Diagnosis is difficult due to the fact that the disease has usually reached advanced stage. We report our experience with two patients with urethral adenocarcinoma. METHODS:We describe clinical-pathological findings and treatment carried out in two patients with urethral adenocarcinoma. We review the literature focusing on the origin of these tumors and available treatment options. RESULTS:After diagnosis, radical surgery was performed in both patients. Despite this, one patient died of local progression. The other patient is still alive and free of recurrence. CONCLUSIONS:Adenocarcinoma of the female urethra is a rare tumor of difficult diagnosis. Surgery is the only curative treatment. Chemotherapy (CT ) and radiotherapy (RT ) must be used in patients in whom surgery is not possible, although there is no consensus on the best therapeutic approach. Prognosis tends to be poor due to delay in diagnosis.
    EAU guidelines on primary urethral carcinoma. Gakis Georgios,Witjes J Alfred,Compérat Eva,Cowan Nigel C,De Santis Maria,Lebret Thierry,Ribal Maria J,Sherif Amir M, European urology CONTEXT:The European Association of Urology (EAU) Guidelines Group on Muscle-Invasive and Metastatic Bladder Cancer prepared these guidelines to deliver current evidence-based information on the diagnosis and treatment of patients with primary urethral carcinoma (UC). OBJECTIVE:To review the current literature on the diagnosis and treatment of patients with primary UC and assess its level of scientific evidence. EVIDENCE ACQUISITION:A systematic literature search was performed to identify studies reporting urethral malignancies. Medline was searched using the controlled vocabulary of the Medical Subject Headings database, along with a free-text protocol. EVIDENCE SYNTHESIS:Primary UC is considered a rare cancer, accounting for <1% of all malignancies. Risk factors for survival include age, tumour stage and grade, nodal stage, presence of distant metastasis, histologic type, tumour size, tumour location, and modality of treatment. Pelvic magnetic resonance imaging is the preferred method to assess the local extent of urethral tumour; computed tomography of the thorax and abdomen should be used to assess distant metastasis. In localised anterior UC, urethra-sparing surgery is an alternative to primary urethrectomy in both sexes, provided negative surgical margins can be achieved. Patients with locally advanced UC should be discussed by a multidisciplinary team of urologists, radiation oncologists, and oncologists. Patients with noninvasive UC or carcinoma in situ of the prostatic urethra and prostatic ducts can be treated with a urethra-sparing approach with transurethral resection and bacillus Calmette-Guérin (BCG). Cystoprostatectomy with extended pelvic lymphadenectomy should be reserved for patients not responding to BCG or as a primary treatment option in patients with extensive ductal or stromal involvement. CONCLUSIONS:The 2013 guidelines document on primary UC is the first publication on this topic by the EAU. It aims to increase awareness in the urologic community and provide scientific transparency to improve outcomes of this rare urogenital malignancy. 10.1016/j.eururo.2013.03.044
    Re: Impact of Perioperative Chemotherapy on Survival in Patients with Advanced Primary Urethral Cancer: Results of the International Collaboration on Primary Urethral Carcinoma. Chang Sam S The Journal of urology 10.1016/j.juro.2015.10.017
    Treatment and Outcomes of Primary Urethra Cancer. Eng Tony Y,Chen Tiffany W,Patel Abhilasha J,Vincent Jill N,Ha Chul S American journal of clinical oncology BACKGROUND:Urethral cancer is a rare malignancy, representing <1% of all malignancies. Optimal management, due to its rarity, presents as a treatment dilemma for physicians. There is a lack of consensus regarding treatment as large randomized trials cannot be performed; thus, optimal management decisions rely on study of retrospective cases. This is a review of our institutional experience with urethral cancer treated with various treatment modalities. METHODS:A retrospective chart review was performed on 31 patients treated for primary cancer of the urethra from 1958 to 2008. The patients were stratified by sex, histologic type, stage, date of diagnosis, type of treatment, and last follow-up. Early stage cases were designated as Tis-T2N0M0 and advanced cases were designated as T3-4, N+ or M+. Analysis was performed based on clinical stage, treatment modalities and outcomes. RESULTS:Fourteen early stage cases and 17 advanced stage cases of urethral cancer were analyzed. The majority of early stage cases occurred in men (M:F=8:6) and the majority of advanced stage cases occurred in women (M:F=5:12). The most common histology was squamous cell carcinoma for both early and advanced stage cases. Surgery was the preferred modality of treatment for early stage cases (surgery used in 13 cases vs. chemo/radiotherapy used in 1 case) while for advanced cases, radiation ±chemotherapy was commonly used. Overall survival for this series was 45% at mean follow-up of 7 years. Eight of the 14 cases of early stage cancer remained disease free at last follow-up. Comparatively, only 5 of 17 with advanced cancers had no apparent disease at last follow-up. All but one of those patients were treated with combined modality therapy. CONCLUSIONS:Patients with early stage urethral cancers do well with single modality therapy, whereas patients who present with advanced cancers may benefit from combined modality therapy. More extensive study is required to recommend a particular treatment protocol. However, in this rare malignancy, institutional experiences provide the best evidence currently due to the lack of multi-institutional trials. 10.1097/COC.0000000000000391
    Impact of perioperative chemotherapy on survival in patients with advanced primary urethral cancer: results of the international collaboration on primary urethral carcinoma. Gakis G,Morgan T M,Daneshmand S,Keegan K A,Todenhöfer T,Mischinger J,Schubert T,Zaid H B,Hrbacek J,Ali-El-Dein B,Clayman R H,Galland S,Olugbade K,Rink M,Fritsche H-M,Burger M,Chang S S,Babjuk M,Thalmann G N,Stenzl A,Efstathiou J A Annals of oncology : official journal of the European Society for Medical Oncology BACKGROUND:To investigate the impact of perioperative chemo(radio)therapy in advanced primary urethral carcinoma (PUC). PATIENTS AND METHODS:A series of 124 patients (86 men, 38 women) were diagnosed with and underwent surgery for PUC in 10 referral centers between 1993 and 2012. Kaplan-Meier analysis with log-rank testing was used to investigate the impact of perioperative chemo(radio)therapy on overall survival (OS). The median follow-up was 21 months (mean: 32 months; interquartile range: 5-48). RESULTS:Neoadjuvant chemotherapy (NAC), neoadjuvant chemoradiotherapy (N-CRT) plus adjuvant chemotherapy (ACH), and ACH was delivered in 12 (31%), 6 (15%) and 21 (54%) of these patients, respectively. Receipt of NAC/N-CRT was associated with clinically node-positive disease (cN+; P = 0.033) and lower utilization of cystectomy at surgery (P = 0.015). The objective response rate to NAC and N-CRT was 25% and 33%, respectively. The 3-year OS for patients with objective response to neoadjuvant treatment (complete/partial response) was 100% and 58.3% for those with stable or progressive disease (P = 0.30). Of the 26 patients staged ≥cT3 and/or cN+ disease, 16 (62%) received perioperative chemo(radio)therapy and 10 upfront surgery without perioperative chemotherapy (38%). The 3-year OS for this locally advanced subset of patients (≥cT3 and/or cN+) who received NAC (N = 5), N-CRT (N = 3), surgery-only (N = 10) and surgery plus ACH (N = 8) was 100%, 100%, 50% and 20%, respectively (P = 0.016). Among these 26 patients, receipt of neoadjuvant treatment was significantly associated with improved 3-year relapse-free survival (RFS) (P = 0.022) and OS (P = 0.022). Proximal tumor location correlated with inferior 3-year RFS and OS (P = 0.056/0.005). CONCLUSION:In this series, patients who received NAC/N-CRT for cT3 and/or cN+ PUC appeared to demonstrate improved survival compared with those who underwent upfront surgery with or without ACH. 10.1093/annonc/mdv230
    A rare case of primary female urethral adenocarcinoma: Columnar type with colonic adenocarcinoma features. Gangadhar Prithal,Pai Muktha R,Sandhya I Indian journal of pathology & microbiology Female urethral carcinoma is extremely rare and accounts for 0.02% of all women's cancers and <1% of cancers in the female genitourinary tract. Adenocarcinoma accounts for only 10% of urethral carcinomas in females. Due to their location, presentation is usually late and tumors are often missed on physical examination. As in this case, nonspecific symptoms in the early stages may delay the diagnosis in most patients. Herein, we present an extremely rare case of the columnar type of primary female urethral adenocarcinoma exhibiting colonic adenocarcinoma features which to the authors' best knowledge has not been reported to date. The present study emphasizes the importance of a careful clinical examination and also highlights the role of imaging studies, and biopsy in making an accurate preoperative diagnosis of this rare disease. The disease may have devastating sequelae due to local and metastatic involvement if not recognized and treated earlier. 10.4103/IJPM.IJPM_745_16
    Carcinoma of the urethra. Zhang Miao,Adeniran Adebowale J,Vikram Raghunandan,Tamboli Pheroze,Pettaway Curtis,Bondaruk Jolanta,Liu Jinsong,Baggerly Keith,Czerniak Bogdan Human pathology Primary carcinomas of the urethra are rare and poorly understood lesions; hence, their clinical and pathologic spectrum is not completely defined. We analyzed a series of 130 primary urethral tumors and classified 106 of them as primary urethral carcinomas. The age at diagnosis of patients with primary urethral carcinomas ranged from 42 to 97 years (mean, 69.4 years; median, 70 years). There were 73 male and 33 female patients with a ratio of 2.2:1. In male patients, the tumors most frequently developed in the bulbous-membranous segment of the urethra. In female patients, the entire length of the urethra was typically involved. Microscopically, they were poorly differentiated carcinomas with hybrid squamous and urothelial features and developed from precursor intraepithelial conditions such as dysplasia and carcinoma in situ, which were frequently present in the adjacent urethral mucosa. High-risk human papilloma virus infection could be documented in 31.6% of these tumors. Follow-up information was available for 95 patients. Twenty-three patients died of the disease with a mean and median survival of 39 and 21 months, respectively. Urethral carcinomas are aggressive tumors with a high propensity for regional and distant metastases with mean and median survival of 39 and 21 months, respectively. Our observations have important implications for the management of patients with primary carcinoma of the urethra by defining them as a unique entity linked to human papilloma virus infection. 10.1016/j.humpath.2017.08.006