The ageing ovary and uterus: new biological insights.
Nelson S M,Telfer E E,Anderson R A
Human reproduction update
BACKGROUND Advanced maternal age is associated with reduced fertility and adverse pregnancy outcomes. This review details recent developments in our understanding of the biology and mechanisms underlying reproductive ageing in women and the implications for fertility and pregnancy. METHODS Sociological online libraries (IBSS, SocINDEX), PubMed and Google Scholar were searched for relevant demographic, epidemiological, clinical and biological studies, using key words and hierarchical MeSH terms. From this, we identified and focused on key topics where it was judged that there had been clinically relevant advances in the understanding of ovarian and uterine ageing with implications for improved diagnostics and novel interventions. RESULTS Mapping of the ovarian reserve, follicular dynamics and associated biomarkers, across the reproductive lifespan has recently been performed. This now allows an assessment of the effects of environmental, lifestyle and prenatal exposures on follicular dynamics and the identification of their impact during periods of germ cell vulnerability and may also facilitate early identification of individuals with shorter reproductive lifespans. If women choose to time their family based on their ovarian reserve this would redefine the meaning of family planning. Despite recent reports of the potential existence of stem cells which may be used to restore the primordial follicle and thereby the oocyte pool, therapeutic interventions in female reproductive ageing at present remain limited. Maternal ageing has detrimental effects on decidual and placental development, which may be related to repeated exposure to sex steroids and underlie the association of ageing with adverse perinatal outcomes. CONCLUSIONS Ageing has incontrovertible detrimental effects on the ovary and the uterus. Our enhanced understanding of ovarian ageing will facilitate early identification of individuals at greatest risk, and novel therapeutic interventions. Changes in both ovary and uterus are in addition to age-related co-morbidities, which together have synergistic effects on reducing the probability of a successful pregnancy outcome.
Primary ovarian mucinous carcinoid tumor: A case report and review of literature.
Hsu Wen-Wei,Mao Tsui-Lien,Chen Chi-Hau
Taiwanese journal of obstetrics & gynecology
OBJECTIVE:Only a few cases of primary ovarian mucinous carcinoid tumor have been documented in the literature till date. We present a case of primary ovarian mucinous carcinoid tumor, atypical type, and review the reported cases. CASE REPORT:A 33-year-old woman with a left ovarian tumor was diagnosed with primary ovarian mucinous carcinoid tumor, atypical type. She underwent left salpingo-oophorectomy and ipsilateral lymph node dissection. After 5 years, the tumor recurred on the right side, with large para-aortic lymphadenopathy that caused hydronephrosis. Complete surgical staging was performed, followed by nine cycles of weekly paclitaxel and gemcitabine. The tumor progressed after discontinuing the chemotherapy, and the patient died of disease 26 months after recurrence. CONCLUSION:Our patient demonstrated a more aggressive clinical course compared to that reported in previous literature. Based on this experience, complete surgical staging is highly recommended after the patient accomplished her fertility plan. Ovarian carcinoid tumors are relatively chemoresistant compared with epithelial ovarian cancers. The regimen of weekly paclitaxel and gemcitabine stabilized the disease but did not reach remission of the tumor. Further studies are required to determine the appropriate chemotherapy regimen.
Diagnosis of the most frequent benign ovarian cysts: is ultrasonography accurate and reproducible?
Guerriero Stefano,Alcazar Juan Luis,Pascual Maria Angela,Ajossa Silvia,Gerada Marta,Bargellini Roberta,Virgilio Bruna,Melis Gian Benedetto
Journal of women's health (2002)
OBJECTIVE:To evaluate the reproducibility and the accuracy of B-mode ultrasonographic features of three different kinds of benign ovarian cysts: ovarian endometrioma, mature teratoma, and serous cyst. METHODS:Digitally stored B-mode sonographic images of 98 women submitted to surgery for the presence of an adnexal mass were evaluated by five different examiners with different degrees of experience. The histological type of each mass was predicted on the basis of the B-mode typical benign findings, as in the case of endometrioma (groundglass endocystic pattern), cystic teratoma (echogenic pattern with or without acoustic shadow), and serous cyst (anechoic cyst without endocystic vegetations). To assess the reproducibility of the B-mode findings, intraobserver and interobserver agreements were calculated using the kappa index. RESULTS:The intraobserver agreement was good or very good for all examiners and for all patterns (kappa = 0.71-1) except for the dermoid cyst, which showed moderate agreement (kappa = 0.42) for the highly experienced operator. The interobserver agreement was good for all experts for endometrioma (kappa = 0.66-0.78) and for serous cyst (kappa = 0.82-1), whereas it was moderate or good for cystic teratoma (kappa = 0.51-0.72). Interobserver agreement between experts and highly experienced operators was fair (kappa = 0.33-0.36) for teratoma and good or very good for endometrioma (kappa = 0.70-0.83) and serous cyst (kappa = 0.76-0.82). For different kinds of cysts, the accuracy was comparable among different operators. CONCLUSIONS:Typical features of benign masses using grayscale transvaginal ultrasonography are reproducible even in moderately experienced examiners, although more experience was associated with better interobserver agreement. The diagnostic performance of different operators with different degrees of experience is similar.
Primary retroperitoneal dysgerminoma presenting as an adrenal tumor: a case report and literature review.
Yoo Su Hyun,Kim Kyu-Rae,Hong Suk Jun,Cho Kyung-Ja
Primary extragonadal germ cell tumors are rare and mostly occur in young men with predominance of nonseminomatous histology. We report an undescribed case of primary retroperitoneal dysgerminoma presenting as an adrenal tumor in a 17-year-old girl. Surgery was performed on a 10 × 9.5 cm sized adrenal gland tumor and the resected tumor showed unequivocal histological features of dysgerminoma. The diagnosis was confirmed by the tumor's germ cell immunophenotype. Postoperative ultrasonography, CT and PET over a 6-month period revealed no evidence of ovarian lesion. The patient is stable, but with a suspicious residual tumor after adjuvant chemotherapy.
Ovarian carcinoma associated with pregnancy: a clinicopathologic analysis of 23 cases and review of the literature.
Behtash Nadereh,Karimi Zarchi Mojgan,Modares Gilani Mitra,Ghaemmaghami Fatemeh,Mousavi Azamsadat,Ghotbizadeh Fahimeh
BMC pregnancy and childbirth
BACKGROUND:The aim of this study was to analyze and describe cases of ovarian cancer in pregnant women treated at our center and to review the literature concerned, and to discuss the rationale for therapy. METHODS:Twenty-Three patients of ovarian malignancies during pregnancy were treated at Vali- Asr Hospital between 1991 and 2002. Data on treatment and follow-up were evaluated. RESULTS:The incidence of ovarian carcinoma associated with pregnancy in our series was 0.083/1000 deliveries. Eleven (47.8%) were found with ovarian malignant germ cell tumors, five (21.7%) with low malignant potential tumors, four (17.4%) with invasive epithelial tumors, and three (13%) with sex cord stromal tumors. Seventeen (73.9%) of the patients were diagnosed in stage I and had complete remission. Five of the six in advanced stage died. The mean follow-up was 36.3 months. The prognosis was significantly related with stage and histological type (P < 0.05). Sixteen healthy live babies were recorded in this group, and two premature newborn died of respiratory distress syndrome. Chemotherapy was administered to 44% of the patients, in two cases during pregnancy. Overall survival at 5 years was 61%. In most of case conservative surgical treatment could be performed with adequate staging and debulking. CONCLUSION:Early finding of ascitis by ultrasound and persistent large ovarian mass during pregnancy may be related to malignancy and advanced stage. Pregnant women in advanced stage of ovarian cancer seem to have poor prognosis.
A pelvic mass on ultrasonography and high human chorionic gonadotropin level: not always an ectopic pregnancy.
Rozenholc Alexandre,Abdulcadir Jasmine,Pelte Marie-Françoise,Petignat Patrick
BMJ case reports
A 24-year-old patient with 7-week amenorrhoea consulted for vaginal bleeding without abdominal pain. Ultrasonography revealed a 7 × 4 cm solid right pelvic mass. There was no visible intrauterine gestational sac. The serum β-human chorionic gonadotropin (β-hCG) level was 11 998 IU/l. Emergency laparoscopy was performed for a presumptive diagnosis of ectopic pregnancy. At laparoscopy, the right ovary was enlarged with a non-haemorrhagic 7 × 4 cm solid lesion, which was resected. The histological diagnosis was a dysgerminoma with immunohistochemistry showing nests of syncytiotrophoblastic cells, which were the origin of the hCG production. There was no pregnancy, either intrauterine or ectopic. There was no evidence of metastasis from the dysgerminoma on the positron-emission tomography scanner. The patient underwent a second procedure for surgical staging of this ovarian germ-cell tumour. This ovarian dysgerminoma was staged FIGO 1A, and the patient did not receive adjuvant therapy. There was no recurrence at the last 8-month follow-up.
Ovarian collision tumors: imaging findings, pathological characteristics, diagnosis, and differential diagnosis.
Peng Yang,Lin Jinhua,Guan Jian,Chen Lili,Zhang Xiaoling,Li Shurong,Wang Huanjun,Liu Mingjuan,Guo Yan
Abdominal radiology (New York)
Collision tumors are uncommon neoplasms in which elements of differing histologic origins coexist in a single mass. Ovarian collision tumors are a rare subtype of such lesions. The identification of collision tumors by radiologic examinations is essential to ensure that comprehensive biopsies are performed to guide appropriate treatments. According to the clinical and imaging findings of 12 patients and reviews of previous studies, ovarian collision tumors are mixtures of different combinations of epithelial tumors, germ cell tumors, and sex-cord-stromal tumors. The smaller tumors are usually located inside ("nested tumor") or on the wall ("back to back") of the larger tumors. Each type of ovarian collision tumors presents specific CT/MRI features in accordance with their histologic origins and collision patterns. Knowledge of the imaging features of ovarian collision tumors is crucial to aid preoperative diagnostic accuracy.
Clinical and Computed Tomography Features of Female Pelvic Malignant Germ Cell Tumors in Children and Adolescents: A Series of 30 Cases.
Wang Qing,Yu Dexin,Wang Fang
Journal of pediatric and adolescent gynecology
STUDY OBJECTIVE:To investigate the clinical features and computed tomography (CT) findings of malignant pelvic germ cell tumors in female patients and improve the diagnostic level of this disease. DESIGN, SETTING, AND PARTICIPANTS:The clinical and CT data of 30 female patients with malignant pelvic germ cell tumors confirmed using histopathology were retrospectively analyzed, before surgery, at a tertiary hospital. Patient's age, tumor location, clinical symptoms, tumor marker levels, and CT findings were recorded. INTERVENTIONS:None. MAIN OUTCOME MEASURES:Identification of the clinical features and CT findings of malignant pelvic germ cell tumors. RESULTS:The patients ranged in age from 11 months to 22 years old, with a mean age of 12.3 years. The 30 cases (30 tumors) included 10 cases of yolk sac tumor, 8 cases of dysgerminoma, and 5 cases of immature teratoma. The alpha-fetoprotein levels were elevated in 8 cases of yolk sac tumor. Eight cases of dysgerminoma showed lobulation sign. Four cases of immature teratoma contained fat and calcification. The fibrovascular septa were observed on contrast-enhanced CT images in 4 cases of dysgerminoma. The "bright dot sign" was noted on contrast enhanced CT images in 9 cases of yolk sac tumor. Remarkable enhancement of the solid component or septa was detected in 10 cases of yolk sac tumor. Obvious enhancement surrounding vascular bundles in the tumor was observed in 5 cases of dysgerminoma. CONCLUSION:Malignant pelvic germ cell tumors in female patients frequently affect children and adolescents. The CT findings of dysgerminoma, immature teratoma, and yolk sac tumor have certain characteristic features. The combination of CT manifestations, tumor markers, and patient age helps improve the diagnostic level of this disease.
Significance of the Complete Surgical Staging of Stage I Malignant Ovarian Germ Cell Tumors.
Park Jeong-Yeol,Kim Dae-Yeon,Suh Dae-Shik,Kim Jong-Hyeok,Kim Yong-Man,Kim Young-Tak,Nam Joo-Hyun
Annals of surgical oncology
OBJECTIVE:The aim of this study was to evaluate the role of complete surgical staging of a malignant ovarian germ cell tumor (MOGCT) that is apparently confined to the ovaries. METHODS:This retrospective study included 135 patients with stage I MOGCT after surgery. Patients who were categorized with stage I disease without complete staging operation were staged as IX. Statistical analysis was performed using Chi-squared or Fisher's exact tests and a Cox proportional hazards model. RESULTS:Eighty-six patients (62.7 %) underwent a complete staging operation; 43 had stage IA disease, 3 had stage IB disease, and 40 had stage IC disease. Forty-nine patients (36.3 %) were staged as IX. After surgery, 93 patients (68.8 %) received adjuvant chemotherapy. In the observation group, the recurrence rates were 12.5 % (2/16), 14.3 % (1/7), and 47.4 % (9/19) for stage IA-IB, stage IC, and stage IX disease, respectively (stage IA-IB vs. stage IX, p = 0.035; stage IC vs. stage IX, p = 0.190). In the adjuvant chemotherapy group, the recurrence rates were 6.7 % (2/30), 6.1 % (2/33), and 6.7 % (2/30) for stage IA-IC, stage IC, and stage IX disease, respectively (stage IA-IC vs. stage IX, p > 0.999; stage IC vs. stage IX, p > 0.999). In multivariable survival analysis, complete surgical staging was significantly associated with improved disease-free survival in patients with stage I MOGCT (odds ratio 3.2; 95 % confidence interval 1.1-9.9; p = 0.049). CONCLUSIONS:Complete surgical staging is independently associated with prognosis in women with apparently early MOGCT who did not receive adjuvant chemotherapy. To enter surveillance strategy without adjuvant chemotherapy, complete surgical staging is mandatory.
A case report of non-islet cell tumour hypoglycaemia associated with ovarian germ-cell tumour.
Powter Louise,Phillips Suzanne,Husbands Emma
BACKGROUND:Non-islet cell tumour hypoglycaemia is a rare paraneoplastic condition in which tumours secrete a high-molecular-weight precursor of insulin-like growth factor-II causing hypoglycaemia and can be difficult to identify and treat. CASE PRESENTATION:This is the case of a 27-year-old patient from Africa with metastatic ovarian yolk sac tumour who presented with hypoglycaemia and was subsequently diagnosed with non-islet cell tumour hypoglycaemia. CASE MANAGEMENT:Our patient required higher doses of glucocorticosteroids than reported in the literature in combination with recombinant growth hormone therapy in order to control her hypoglycaemia. CASE OUTCOME:This is the first case of non-islet cell tumour hypoglycaemia described in association with a germ-cell tumour. Her management required collaboration between the endocrinology team, the palliative care team, the acute medicine team and physicians in Africa to enable her safe journey home. CONCLUSIONS:This case illustrates the need for awareness among general physicians of rare tumour manifestations and the need for multidisciplinary input for the optimal management of these patients.
Salvage high-dose chemotherapy in female patients with relapsed/refractory germ-cell tumors: a retrospective analysis of the European Group for Blood and Marrow Transplantation (EBMT).
De Giorgi U,Richard S,Badoglio M,Kanfer E,Bourrhis J H,Nicolas-Virelizier E,Vettenranta K,Lioure B,Martin S,Dreger P,Schuler M K,Thomson K,Scarpi E,Rosti G,Selle F,Mangili G,Lanza F,Bregni M,
Annals of oncology : official journal of the European Society for Medical Oncology
Background:High-dose chemotherapy (HDC) with hematopoietic progenitor cell transplantation is a standard option for relapsed/refractory testicular germ-cell tumor (GCT), but only few data have been reported in female patients with GCT. We conducted a retrospective analysis of female patients with GCT treated with HDC and registered with the European Society for Blood and Marrow Transplantation. Patients and methods:Between 1985 and 2013, 60 registered female patients with GCT, median age 27 years (range 15-48), were treated with salvage HDC. Forty patients (67%) had primary ovarian GCT, 8 (13%) mediastinal, 7 (12%) retroperitoneal and 5 (8%) other primary sites/unknown. Twenty-two patients (37%) received HDC as second-line therapy, 29 (48%) as third-line, and 9 (15%) as fourth- to sixth-line. Nine of 60 patients (15%) received HDC as late-intensification with no evidence of metastasis before HDC. The conditioning HDC regimens comprised carboplatin in 51 of 60 cases (85%), and consisted of a single HDC cycle in 31 cases (52%), a multi-cycle HDC regimen in 29 (48%). Results:Nine cases who underwent late intensification HDC were not evaluable for response. Of the other 51 assessable patients, 17 (33%) achieved a complete response (CR), 8 (16%) a marker-negative partial remission (PRm-), 5 (10%) a marker-positive partial remission, 5 (10%) stable disease, and 13 (25%) progressive disease. There were 3 toxic deaths (6%). With an overall median follow-up of 14 months (range 1-219), 7 of 9 (78%) patients with late intensification and 18 of the 25 patients (72%) achieving a CR/PRm- following HDC were free of relapse/progression. In total, 25 of 60 patients (42%) were progression-free following HDC at a median follow-up of 87 months (range 3-219 months). Conclusions:Salvage HDC based on carboplatin represents a therapeutic option for female patients with relapsed/refractory GCT.
Clinical, morphological and immunohistochemical evidence that small-cell carcinoma of the ovary of hypercalcaemic type (SCCOHT) may be a primitive germ-cell neoplasm.
McCluggage W Glenn,Witkowski Leora,Clarke Blaise A,Foulkes William D
AIMS:The histogenesis and cell lineage of small-cell carcinoma of the ovary of hypercalcaemic type (SCCOHT) is unknown. We aim to provide evidence that this may be a primitive germ-cell neoplasm arising from a teratoma. METHODS AND RESULTS:Following the identification of two cases of SCCOHT associated with germ-cell tumours (one dermoid cyst, one immature teratoma with a focus of yolk sac tumour), we undertook a literature review to look for any prior reports of SCCOHT in association with other neoplasms or elements. This revealed two cases associated with immature teratomas, one arising in an ovary where a cystectomy had been undertaken previously for a teratoma and another in association with a mucinous borderline tumour. Mucinous elements have also been reported in SCCOHT, this type of epithelium potentially being of teratomatous derivation. We stained whole tissue sections of nine cases of SCCOHT and a tissue microarray (TMA) containing 34 different SCCOHT with germ-cell markers SALL4, OCT3/4, alpha fetoprotein (AFP) and glypican 3. All except one of the whole tissue sections and approximately half the TMA cases were positive with SALL4, while all cases were OCT3/4-, AFP- and glypican 3-negative, except for focal glypican 3 staining in an occasional case. CONCLUSIONS:Our findings provide additional evidence to that proposed by others that SCCOHT is a primitive germ-cell neoplasm arising from a teratoma.
Malignant Ovarian Germ Cell Tumors in Postmenopausal Patients: The Royal Marsden Experience and Literature Review.
Boussios Stergios,Attygalle Ayoma,Hazell Steve,Moschetta Michele,McLachlan Jennifer,Okines Alicia,Banerjee Susana
BACKGROUND:Ovarian germ cell tumors (OGCT) account for 2-5% of ovarian malignancies, with an annual incidence of 1:100,000, and typically occur in young women and adolescents. The yolk sac tumor (YST) is the second most common subtype of OGCTs and has an aggressive phenotype. Their rarity in postmenopausal women has the potential to cause initial diagnostic uncertainty and lead to delayed or sub-optimal treatment. In this article, we report two cases. The first case is a 67-year-old woman with a pure YST and the second refers to a 59-year-old patient with YST with neuroendocrine differentiation. We also review and summarise the current literature. DISCUSSION:YSTs in older women, either in association with ovarian epithelial tumors or without an identifiable epithelial precursor, are a challenging clinical situation. The disease is aggressive and the outcome remains poor. Thirty-seven cases, including the two reported in this article, have been described in the literature to date. 12/ 37 described patients with malignant OGCTs died within 8 months of diagnosis. CONCLUSION:Ovarian cancer with a YST component in postmenopausal women has an aggressive behaviour and adjuvant platinum-based chemotherapy should be considered.
The role of staging and adjuvant chemotherapy in stage I malignant ovarian germ cell tumors (MOGTs): the MITO-9 study.
Mangili G,Sigismondi C,Lorusso D,Cormio G,Candiani M,Scarfone G,Mascilini F,Gadducci A,Mosconi A M,Scollo P,Cassani C,Pignata S,Ferrandina G
Annals of oncology : official journal of the European Society for Medical Oncology
Background:Surgery followed by platinum-based chemotherapy is the standard of care for MOGCTs, except for stage IA dysgerminoma and stage IA grade 1 immature teratoma where surveillance only is recommended. The role of adjuvant chemotherapy and surgical staging is debated. Patients and methods:Data from 144 patients with stage I MOGTs were collected among MITO centers (Multicenter Italian Trials in Ovarian Cancer) and analyzed. Results:Fifty-five (38.2%) patients were affected by dysgerminomas, 49 (34%) by immature teratomas, 26 (18.1%) by yolk sac tumors and 14 (9.7%) by mixed tumors. Seventy-three (50.7%) patients receive surgery plus chemotherapy, while 71 (49.3%) patients underwent surgery alone. The latter group included 32 dysgerminomas (14 IA-13 Ix, 3 IB, and 2 IC), 34 immature teratomas (20 1A-13 IA grade 1, 6 Ix, 1 IB, and 7 IC), 4 mixed tumors and 1 yolk sac tumor. Forty-four patients did not received chemotherapy, even if it would have been indicated by recommended approach. 94 (65.3%) patients received peritoneal surgical staging. Twenty-three (15.9%) developed a recurrence. Incomplete surgical staging was associated with recurrence (P < 0.05; OR 2.37) at Cox regression analysis. Seven patients died. Four patients were affected by yolk sac tumors, two by mixed tumors and one by immature teratoma. Five patients died for disease, one for acute leukemia and one for suicide. Prognostic parameter analyses showed that yolk sac component is a predictor for survival (P < 0.05). Five-years OS rates were 96.8% and 88.7% in the surgically staged and the incomplete staged group, respectively, while 93.8% and 94.1% in the standard treatment and in the surveillance group, respectively. Conclusions:This study shows that surveillance seems not to affect survival; chemotherapy should be reserved for relapse resulting in high cure rate. Incomplete peritoneal surgical staging is associated with recurrence. Yolk sac histology worsens the prognosis.
Paediatric extracranial germ-cell tumours.
Shaikh Furqan,Murray Matthew J,Amatruda James F,Coleman Nicholas,Nicholson James C,Hale Juliet P,Pashankar Farzana,Stoneham Sara J,Poynter Jenny N,Olson Thomas A,Billmire Deborah F,Stark Daniel,Rodriguez-Galindo Carlos,Frazier A Lindsay
The Lancet. Oncology
Management of paediatric extracranial germ-cell tumours carries a unique set of challenges. Germ-cell tumours are a heterogeneous group of neoplasms that present across a wide age range and vary in site, histology, and clinical behaviour. Patients with germ-cell tumours are managed by a diverse array of specialists. Thus, staging, risk stratification, and treatment approaches for germ-cell tumours have evolved disparately along several trajectories. Paediatric germ-cell tumours differ from the adolescent and adult disease in many ways, leading to complexities in applying age-appropriate, evidence-based care. Suboptimal outcomes remain for several groups of patients, including adolescents, and patients with extragonadal tumours, high tumour markers at diagnosis, or platinum-resistant disease. Survivors have significant long-term toxicities. The challenge moving forward will be to translate new insights from molecular studies and collaborative clinical data into improved patient outcomes. Future trials will be characterised by improved risk-stratification systems, biomarkers for response and toxic effects, rational reduction of therapy for low-risk patients and novel approaches for poor-risk patients, and improved international collaboration across paediatric and adult cooperative research groups.
Neo-adjuvant chemotherapy in the treatment of advanced malignant germ cell tumors of ovary.
Talukdar Shobhana,Kumar Sunesh,Bhatla Neerja,Mathur S,Thulkar S,Kumar Lalit
OBJECTIVE:In order to preserve fertility, we attempted neo-adjuvant chemotherapy (NACT) in patients of malignant ovarian germ cell tumor (MOGCT) with advance and bulky disease. PATIENTS AND METHODS:Between January 1988 and December 2009, 23 patients received NACT. Patient's median age was 19 years, ranging from 14 to 28 years. FIGO stages III - 20 and IV - 3. Histology subtypes were: dysgerminoma, n = 14, mixed GCT, n=6 and 3 had endodermal sinus tumor. Patients were planned for four cycles of BEP (bleomycin, etoposide and cisplatin) chemotherapy followed by fertility sparing surgery (unilateral salpingo-oophorectomy+omentectomy ± lymphadenectomy). RESULTS:Following NACT - 21 patients responded; complete (CR) - 16 and partial response (PR) - 5. One patient progressed and another was lost to follow-up after 2 cycles. 18 of 21 responders underwent surgery; 13/18 had pathological CR, 5/18 had residual disease and achieved CR following 2 more cycles of BEP. 3 patients refused for surgery; 2 relapsed at 9 and 12 months, and achieved second CR following salvage chemotherapy and surgery, third patient continues to be disease-free. Currently, 21 of 23 patients are alive and disease-free at a median follow-up of 74 months. 18/21 patients have resumed menstruation and 10 eligible patients have delivered 13 full term healthy babies. These results are comparable to patients with advanced disease (n = 43) treated with standard approach (initial surgery and adjuvant chemotherapy) during the same period. CONCLUSION:NACT followed by fertility sparing surgery could be a reasonable option for patients of advanced MOGCT, not suitable for optimal cyto-reduction.
The genetic landscape of 87 ovarian germ cell tumors.
Van Nieuwenhuysen Els,Busschaert Pieter,Neven Patrick,Han Sileny N,Moerman Philippe,Liontos Michalis,Papaspirou Maria,Kupryjanczyk Jolanta,Hogdall Claus,Hogdall Estrid,Oaknin Ana,Garcia Angel,Mahner Sven,Trillsch Fabian,Cibula David,Heitz Florian,Concin Nicole,Speiser Paul,Salvesen Helga,Sehouli Jalid,Lambrechts Diether,Vergote Ignace
BACKGROUND:Ovarian germ cell tumors (OGCT) are rare gynecological neoplasms, mostly affecting children and young women. The underlying molecular genetic background of these tumors is poorly characterized. METHODS:We analyzed somatic copy number aberration (CNA) profiles in 87 OGCT tumors and performed whole exome sequencing (WES) on 24 OGCT tumor and matched germline samples to further elucidate their molecular genetic landscape. RESULTS:The overall mutation rate was very low in OGCT compared to other human cancers, with an average of 0.05 mutations per Mb, consistent with their embryological origin. We identified recurrent mutations in KIT and KRAS, while CNA profiling revealed frequent focal amplifications affecting PIK3CA and AKT1 in yolk sac tumors, recurrent focal deletions affecting chromosomal regions 1p36.32, 2q11.1, 4q28.1, 5p15.33, 5q11.1 and 6q27, as well as gains in chromosome 12p that were present in all tumors, except for pure immature teratomas. CONCLUSION:We here present the first whole exome sequencing data and to our knowledge the largest CNA study in OGCT. We confirmed that earlier reported KIT mutations were frequent in dysgerminomas and mixed forms with a dysgerminoma component, whereas chromosome 12p gains were present in all histological subtypes except pure immature teratomas. We detected recurrent KRAS mutations, recurrent focal deletions and an enrichment in the PI3K/AKT/PTEN pathway in yolk sac tumors. Several of these aberrations involve targetable pathways, offering novel treatment modalities for OGCT.
Polyembryoma of the testis: a report of two cases dominant within mixed germ cell tumors and review of gonadal polyembryomas.
Stall Jennifer N,Young Robert H
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
Two testicular mixed germ cell tumors, from men of 21 and 41 years, in which polyembryoma predominated are described. A literature review uncovered an additional five testicular and nine ovarian cases. One tumor occurred in a 60-year-old man, but all others occurred within the typical age range of gonadal germ cell tumors. One male presented with gynecomastia and one female with sexual precocity, but all otherwise had standard clinical manifestations. These tumors are typically large with non-specific gross features, but a few have a prominent hemorrhagic appearance. No tumor is known to have been entirely composed of embryoid bodies, the unit upon which the diagnosis of polyembryoma is based. The most common additional germ cell tumor component is teratoma, present in the great majority of cases, with an approximately equal smaller number of tumors being associated with embryonal carcinoma and yolk-sac tumor, manifest as overgrowths of these elements, derived from the parent epithelium within the embryoid body. Rarely there is choriocarcinoma, and syncytiotrophoblast and hepatoid cells are occasionally present. The microscopic features of the tumors vary according to the arrangement of embryoid bodies with other elements, the prominence of associated typically myxoid to edematous stroma, and the degree to which embryoid bodies are perfectly or imperfectly formed. Although its presence in a gonadal mixed germ cell tumor is probably not associated with any special behavior, its unique features should result in polyembryoma being recorded, particularly when present in significant amount. Furthermore, awareness of its features may facilitate recognition, particularly when seen at metastatic sites or extra-gonadal sites of primary germ cell neoplasia. Whether polyembroma should be considered a distinctive pattern of mixed germ cell neoplasia or a particular variant of high-grade immature teratoma is considered, herein, and arguments can be made in favor of each viewpoint.
Imaging features of growing teratoma syndrome following a malignant ovarian germ cell tumor.
Han Na Yeon,Sung Deuk Jae,Park Beom Jin,Kim Min Ju,Cho Sung Bum,Kim Kyeong Ah,Song Jae Yun
Journal of computer assisted tomography
OBJECTIVE:To access imaging findings of growing teratoma syndrome (GTS), which is a rare complication of malignant ovarian germ cell tumor (GCT) after chemotherapy. METHODS:Five patients met the criteria for GTS. Computed tomography and magnetic resonance images were retrospectively reviewed by 2 radiologists in consensus for margin, attenuation, and the presence of gross fat or calcification of GTS lesions, which were compared with primary GCTs regarding tumor composition. RESULTS:Growing teratoma syndrome lesions were characterized as follows: poorly circumscribed, diffuse peritoneal masses in 2 patients; well-circumscribed, localized peritoneal masses in 1 patient, and ovarian masses in 2 patients. Features more noticeable in GTS lesions were more prominent fatty components in 4 patients and purely cystic lesion in 1 patient. CONCLUSIONS:Growing teratoma syndrome can be manifested as intraperitoneal masses with an increased fatty or cystic component. Radiologists should consider GTS when there are such masses on follow-up imaging studies in patients with malignant ovarian GCT.
Fertility management for malignant ovarian germ cell tumors patients.
Di Tucci Chiara,Casorelli Assunta,Morrocchi Elisa,Palaia Innocenza,Muzii Ludovico,Panici Pierluigi Benedetti
Critical reviews in oncology/hematology
Malignant Germ Cell Tumors have primarily affecting adolescents and young adults. In advanced disease, greater than 70% of patients can be cured with standard chemotherapy regimens and fertility-sparing surgery appears to be safe with excellent survival after long-term follow-up. Due to their rarity, follow up and fertility management is largely based on trials of epithelial ovarian cancer or on few small studies. We report a review of the literature studies about the assessment, the monitoring and the treatment of fertility for Malignant Germ Cell Tumors as pratical guidelines for management of fertility in these patients.
Analysis of outcomes and prognostic factors after fertility-sparing surgery in malignant ovarian germ cell tumors.
Park Jeong-Yeol,Kim Dae-Yeon,Suh Dae-Shik,Kim Jong-Hyeok,Kim Yong-Man,Kim Young-Tak,Nam Joo-Hyun
OBJECTIVE:To evaluate the oncologic and reproductive outcomes and to analyze prognostic factors after fertility-sparing surgery in patients with early and advanced malignant ovarian germ cell tumors (MOGCTs). METHODS:This study included 171 patients who underwent fertility-sparing surgery. Data were gathered from patients' medical records. Survival analysis was performed using the log-rank test and Cox's proportional hazards model. Reproductive outcomes were analyzed. RESULTS:Twenty-five patients (14.6%) had recurrent disease, and five patients (2.9%) died of disease during the median follow-up time of 86months (range, 9-294months). The 5-year disease-free survival (DFS) was 86%, and the 5-year overall survival (OS) was 97%. The 5-year DFS was 84% for stage I and 89% for stage II-IV. The 5-year OS was 99% for stage I and 91% for stage II-IV. In multivariate analysis, yolk sac tumor, incomplete staging surgery, and residual tumor were independent risk factors for reduced DFS, and yolk sac tumor and residual tumor were independent risk factors for reduced OS. Reproductive and obstetric outcomes were evaluable in 124 patients, and 106 patients (85.5%) had regular menstruation, 12 patients (9.7%) had irregular menstruation, and six patients (4.8%) had premature menopause. Twenty patients tried to conceive, 15 patients (75%) succeeded in achieving 21 pregnancies, and 13 of the patients (65%) gave birth to 20 healthy babies. CONCLUSION:Fertility-sparing surgery has excellent survival outcomes in young women with MOGCTs, even in advanced stages. Reproductive and obstetric outcomes were promising. Yolk sac tumor, incomplete surgical staging, and residual tumor were independent prognostic factors.
Detection of Relapse by Tumor Markers Versus Imaging in Children and Adolescents With Nongerminomatous Malignant Germ Cell Tumors: A Report From the Children's Oncology Group.
Fonseca Adriana,Xia Caihong,Lorenzo Armando J,Krailo Mark,Olson Thomas A,Pashankar Farzana,Malogolowkin Marcio H,Amatruda James F,Billmire Deborah F,Rodriguez-Galindo Carlos,Frazier A Lindsay,Shaikh Furqan
Journal of clinical oncology : official journal of the American Society of Clinical Oncology
PURPOSE:To investigate relapse detection methods among children and adolescents with nongerminomatous malignant germ cell tumors (MGCTs) and to determine whether tumor markers alone might be sufficient for surveillance. METHODS:We retrospectively reviewed all patients enrolled in a phase III, single-arm trial for low-risk and intermediate-risk MGCTs. The method used to detect relapse was assessed based on case report forms, tumor markers, imaging, and pathology reports. Relapses were classified into one of two categories on the basis of whether they were (1) detectable by tumor marker elevation or (2) not detectable by tumor markers. RESULTS:A total of 302 patients were enrolled, and 284 patients had complete data for review. Seven patients had normal tumor markers at initial diagnosis, and none experienced a relapse. At a median follow-up of 5.3 years, 48 patients (16.9%) had experienced a relapse. After central review, 47 of 48 relapses (98%) were detected by tumor marker elevation. Of the 47 patients, 16 (33.3%) had abnormal tumor markers with normal/unknown imaging, 31 patients (64.6%) had abnormal tumor markers with abnormal imaging, and one patient (2.1%) had abnormal imaging with unknown marker levels at relapse. CONCLUSION:Tumor marker elevation is a highly sensitive method of relapse surveillance, at least among children and adolescents with tumor marker elevation at initial diagnosis. Eliminating exposure to imaging with ionizing radiation may enhance the safety of relapse surveillance in patients treated for MGCT.
Germ Cell Tumors of the Female Genital Tract.
Euscher Elizabeth D
Surgical pathology clinics
Ovarian germ cell tumors are a histologically diverse group of neoplasms with a common origin in the primitive germ cell. The vast majority are represented by mature cystic teratoma. In the minority are malignant germ cell tumors including immature teratoma, dysgerminoma, yolk sac tumor, embryonal cell carcinoma, and choriocarcinoma. This article reviews the histologic and immunohistochemical features of the most common ovarian germ cell tumors. The differential diagnoses for each are discussed.
Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features.
Shaaban Akram M,Rezvani Maryam,Elsayes Khaled M,Baskin Henry,Mourad Amr,Foster Bryan R,Jarboe Elke A,Menias Christine O
Radiographics : a review publication of the Radiological Society of North America, Inc
Ovarian malignant germ cell tumors (OMGCTs) are heterogeneous tumors that are derived from the primitive germ cells of the embryonic gonad. OMGCTs are rare, accounting for about 2.6% of all ovarian malignancies, and typically manifest in adolescence, usually with abdominal pain, a palpable mass, and elevated serum tumor marker levels, which may serve as an adjunct in the initial diagnosis, monitoring during therapy, and posttreatment surveillance. Dysgerminoma, the most common malignant germ cell tumor, usually manifests as a solid mass. Immature teratomas manifest as a solid mass with scattered foci of fat and calcifications. Yolk sac tumors usually manifest as a mixed solid and cystic mass. Capsular rupture or the bright dot sign, a result of increased vascularity and the formation of small vascular aneurysms, may be present. Embryonal carcinomas and polyembryomas rarely manifest in a pure form and are more commonly part of a mixed germ cell tumor. Some OMGCTs have characteristic features that allow a diagnosis to be confidently made, whereas others have nonspecific features, which make them difficult to diagnose. However, imaging features, the patient's age at presentation, and tumor markers may help establish a reasonable differential diagnosis. Malignant ovarian germ cell tumors spread in the same manner as epithelial ovarian neoplasms but are more likely to involve regional lymph nodes. Preoperative imaging may depict local extension, peritoneal disease, and distant metastases. Suspicious areas may be sampled during surgery. Because OMGCTs are almost always unilateral and are chemosensitive, fertility-sparing surgery is the standard of care.
Fertility and gonadal function after adjuvant therapy in women diagnosed with a malignant ovarian germ cell tumor (MOGCT) during the "cisplatin era".
Solheim O,Tropé C G,Rokkones E,Kærn J,Paulsen T,Salvesen H B,Hagen B,Vereide A B,Fosså S D
PURPOSE:By self-report and serum levels of anti-Mullerian hormone (AMH) this study aims to assess post-treatment fertility after modern treatment of women with malignant ovarian germ cell tumors (MOGCT). PATIENTS AND METHODS:In 2013 a questionnaire-based survey was performed in 61 MOGCT patients diagnosed at age <40years from 1980-2009. Forty-nine of them also attended the out-patient clinic. The event of first post-treatment pregnancy ("fertility") was documented as cumulative estimates for all 61 patients and within each of 4 treatment groups: Group 1: Surgery only (n=10); Group 2: ≤3cycles of cisplatin-based chemotherapy (CBCT) (n=20); Group 3: >3cycles of CBCT (n=15) and Group 4: other adjuvant treatment (n=16). AMH was determined in 22 women <40years at survey. Statistics were based on Kaplan Meier procedure, log-rank test and a significance level p<0.05. RESULTS:At least one post-treatment pregnancy was reported by 34 of 39 MOGCT survivors who attempted motherhood after treatment. The 15-year cumulative post-treatment fertility estimate was 28% (95% CI: 26-30) for all 61 survivors and was significantly higher in patients treated with 3 or fewer cycles of CBCT (53% [95% CI: 50-55]) than those treated with more than 3cycles (20% [95% CI: 17-22]) (P=0.03). Of 22 AMH levels, two were <3pmol/l, with one women being pregnant at survey. CONCLUSION:After fertility-sparing surgery and modern cisplatin-based chemotherapy, fertility is preserved in most MOGCT survivors though dependent on the number of cycles. AMH's role as a biomarker of gonadal function seems promising but requires further research.
Perspectives on testicular sex cord-stromal tumors and those composed of both germ cells and sex cord-stromal derivatives with a comparison to corresponding ovarian neoplasms.
Roth Lawrence M,Lyu Bingjian,Cheng Liang
Sex cord-stromal tumors (SCSTs) are the second most frequent category of testicular neoplasms, accounting for approximately 2% to 5% of cases. Both genetic and epigenetic factors account for the differences in frequency and histologic composition between testicular and ovarian SCSTs. For example, large cell calcifying Sertoli cell tumor and intratubular large cell hyalinizing Sertoli cell neoplasia occur in the testis but have not been described in the ovary. In this article, we discuss recently described diagnostic entities as well as inconsistencies in nomenclature used in the recent World Health Organization classifications of SCSTs in the testis and ovary. We also thoroughly review the topic of neoplasms composed of both germ cells and sex cord derivatives with an emphasis on controversial aspects. These include "dissecting gonadoblastoma" and testicular mixed germ cell-sex cord stromal tumor (MGC-SCST). The former is a recently described variant of gonadoblastoma that sometimes is an immediate precursor of germinoma in the dysgenetic gonads of patients with a disorder of sex development. Although the relationship of dissecting gonadoblastoma to the previously described undifferentiated gonadal tissue is complex and not entirely resolved, we believe that it is preferable to continue to use the term undifferentiated gonadal tissue for those cases that are not neoplastic and are considered to be the precursor of classical gonadoblastoma. Although the existence of testicular MGC-SCST has been challenged, the most recent evidence supports its existence; however, testicular MGC-SCST differs significantly from ovarian examples due to both genetic and epigenetic factors.
Metachronous Synovial Sarcoma After Treatment of Mixed Germ Cell Tumor in a Child with Complete Gonadal Dysgenesis.
Karahan Feryal,Citak Elvan Caglar,Yaman Emel,Alakaya Mehmet,Sağcan Fatih,Yılmaz Eda Bengi,Kuş Funda,Gürses İclal,Balcı Yüksel
Journal of clinical research in pediatric endocrinology
Patients with complete XY gonadal dysgenesis (GD) show a high predisposition to germ cell tumors (GCT). Patients with coexistence of GCT and GD have been reported previously. Here we present a 15-year-old girl with mixed GCT and GD who also developed an intra-abdominal synovial sarcoma one year after the treatment. This is the first report, to our knowledge, of synovial sarcoma associated with XY GD.
Malignant Mixed Germ Cell Tumor Overgrowing a Gonadoblastoma in a Female With a 46, XX Karyotype: A Case Report.
Arafah Maria A,Raddaoui Leen E
International journal of surgical pathology
Gonadoblastoma is an uncommon ovarian tumor arising primarily in females with gonadal dysgenesis and a 46, XY karyotype. Germ cell tumors arising within and/or overgrowing a gonadoblastoma have been reported. We report a rare case of a malignant mixed germ cell tumor (yolk sac tumor and choriocarcinoma) arising in a gonadoblastoma of the left ovary in a 19-year-old female with a 46, XX karyotype. The patient's initial α-fetoprotein level was 20 000 KIU/L. The patient underwent a laparoscopic unilateral salpingo-oophorectomy with omentectomy and peritoneal washing followed by adjuvant chemotherapy.
Clinicopathological pattern and outcome of pediatric malignant ovarian germ cell tumors: South Egypt Cancer Institute experience.
Ali Amany,Sayed Heba,Salem Mohamed,Hamdy Mohamed,Farok Amro
Journal of pediatric surgery
BACKGROUND:Malignant ovarian germ cell tumors (MOGCTs) are rare and represent 1-1.5% of all cancers in children and adolescents. The aim of this study is to analyze the clinicopathological pattern at presentation and management and outcome of MOGCTs in children and adolescents. PATIENTS AND METHODS:Retrospective study included all girls diagnosed with MOGCTs between January 2005 and January 2015 in Pediatric and Surgical Oncology Departments at South Egypt Cancer Institute, Assiut University. Data were collected from patients' records including initial presentation, diagnosis (tumor markers and imaging), surgical staging and pathologic types. Management (surgical and chemotherapy details) and outcomes were also analyzed. RESULTS:Forty girls aged between 4 to 17years (mean age of 9.5years) with diagnosis of MOGCTs during study period were included. The most common presenting symptoms and signs were abdominal swelling, abdominal pain, and pelvic mass. Precocious puberty was noted in two patients. Surgical interventions in most patients were unilateral salpingo-oophorectomy (n=20). Early stages I and II were reported in 15 and 12 patients respectively, while 10 patients had stage-III disease and 3 patients had stage IV. Yolk sac tumors were reported in 27.5% of patients. All patients were treated with platinum based chemotherapy. The 7-year overall survival was higher for patients with early stages (I and II) compared with advanced stages (III and IV) (100% versus 30.8% respectively. CONCLUSIONS:Early presentation with appropriate management using fertility sparing surgery and platinum-based chemotherapy provides excellent survival with fertility preservation in children and adolescents. Based on the lower survival of patients with advanced disease, efforts should focus on increasing the awareness in the community of the importance of early diagnosis of ovarian tumors. LEVEL OF EVIDENCE:II (retrospective study).
Malignant mixed ovarian germ cell tumor with embryonal component.
Moniaga Natalie Catharine,Randall Leslie M
Journal of pediatric and adolescent gynecology
BACKGROUND:Malignant germ cell tumors of the ovary are relatively rare, making up less than 10% of all ovarian cancers. However, although they represent only a small fraction of ovarian cancers overall, they frequently affect adolescent women of reproductive age, making fertility sparing treatment of paramount importance. Malignant germ cell tumors are subdivided into dysgerminoma and nondygerminomatous tumors. The most common types of nondysgerminomatous tumors are yolk sac and immature teratoma. Mixed germ cell tumors with embryonal carcinoma, nongestational choriocarcinoma, and polyembryoma are less common. Embryonal carcinomas, though rare, are one of the most malignant cancers arising in the ovary. CASE:A 19-year-old female with abdominal pain and massive ascites was found to have a malignant mixed ovarian germ cell tumor with a large embryonal component which was treated via surgical resection and chemotherapy. CONCLUSIONS:Malignant germ cell tumors frequently affect adolescent women of reproductive age. Management of these tumors requires consideration of fertility sparing surgical techniques and chemotherapy management. Using these techniques, the vast majority of patients will maintain their ovarian function and the ability to bear children after their recovery.
Minimally Invasive Staging of Apparent Stage I Malignant Ovarian Germ Cell Tumors: Prevalence and Outcomes.
Nasioudis Dimitrios,Minis Evelyn,Chapman-Davis Eloise,Frey Melissa K,Caputo Thomas A,Witkin Steven S,Holcomb Kevin
Journal of minimally invasive gynecology
STUDY OBJECTIVE:Evaluate the prevalence, trends, and outcomes of minimally invasive surgical (MIS) staging of malignant ovarian germ cell tumors (MOGCTs) apparently confined to the ovary. DESIGN:Retrospective cohort study (Canadian Task Force classification II-2). SETTING:Participating hospitals in the National Cancer Data Base. PATIENTS:Women diagnosed between 2010 and 2014 with a MOGCT apparently confined to the ovary with information on the planned surgical approach. INTERVENTIONS:Staging with MIS or laparotomy. MEASUREMENT AND MAIN RESULTS:A total of 918 patients were identified. MIS was planned for 294 patients (32%): a laparoscopic approach for 237 patients and a robotic-assisted approach for 57 patients. Rate of conversion to laparotomy was 11% (46 cases), 1.7% and 15.6% in the robotic and laparoscopy groups, respectively (p = .003). No difference in the use of MIS was noted based on year of diagnosis (p = .38). By multivariate analysis white race, higher level of education, and smaller tumor size were associated with the receipt of MIS. Patients in the MIS group were less likely to undergo lymph node dissection (39.6% vs 51.3%, p = .001) and omentectomy (18.7% vs 28.5%, p = .002). Hospital stay after surgery was shorter for patients who had MIS (median, 2 vs 3 days; p <.001). Unplanned 30-day readmission rate was also lower in the MIS group (1.4% vs 3.9%, p = .043). No difference in overall survival was noted between the 2 groups (p = .81). CONCLUSION:MIS for apparent early-stage MOGCTs was less comprehensive but associated with a decreased hospital stay and unplanned readmission rate.
Mixed ovarian germ cell tumor composed of immature teratoma, yolk sac tumor and embryonal carcinoma.
Wang Ying,Zhou Feng,Qian Zhida,Qing Jiale,Zhao Mengdam,Huang Lili
Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
We report the case of a 19-year-old woman experiencing lower abdominal distension and pain. Laboratory tests indicated elevated serum levels of Alpha-Fetoprotein (AFP) and human Chorionic Gonadotropin (hCG). A large mass was detected in the abdomen by physical examination and by transvaginal ultrasonography. Exploratory laparotomy was performed, and a smooth-surfaced, spherical, solid tumor was found on the left ovary, measuring 11.5 x 9.9 x 6.9 cm. Histological evaluation revealed that the tumor consisted of a combination of immature teratoma, Yolk Sac Tumor, and embryonal carcinoma; this is a very rare combination in mixed germ cell tumors.
Laparoscopic Treatment of Mixed Malignant Ovarian Germ Cell Tumor in a 16-Year-Old Female Adolescent.
Friedman Caroline,Fenster Tamatha
Journal of pediatric and adolescent gynecology
BACKGROUND:Malignant ovarian germ cell tumors are rare entities, although they account for a large proportion of ovarian masses in young women. These tumors have traditionally been removed via laparotomy, because of their large size and solid nature. The use of laparoscopy for treatment of adnexal masses in adolescents has been heavily debated and poorly studied to date. CASE:A 16-year-old female patient presented with abdominal pain and an 11-cm adnexal mass on ultrasound. An emergent laparoscopic salpingo-oophorectomy was performed without complication. Pathology revealed a mixed malignant ovarian germ cell tumor. SUMMARY AND CONCLUSION:Laparoscopic fertility-sparing surgery offers many benefits over laparotomy, and should be considered in cases of young women with large adnexal masses, even if potential for malignancy exists.
Recurrent ovarian mixed germ cell tumor with unusual malignant transformation: a case report.
Lee Yi-Le,Lai Chiung-Ru,Yen Ming-Shyen
Journal of ovarian research
BACKGROUND:The value of this report is the identification of late recurrence with an extremely unusual combination of malignant transformation. In particular, the retroconversion of immature to mature teratoma as well as a somatic-type malignant transformation were both observed postchemotherapeutically in our case. CASE PRESENTATION:We report the case of a 20-year-old girl who completed fertility-sparing surgery and chemotherapy under the diagnosis of ovarian mixed germ cell tumor (immature teratoma and yolk sac tumor) and experienced subsequent recurrence 4 years after a second debulking surgery with a somatic type malignant transformation (teratoma with melanoma and leiomyosarcoma). Multiple metastases developed after a third debulking surgery, and the patient survived for 18 additional months. CONCLUSIONS:Recurrent disease after repeated cytoreduction and chemotherapy hints a poor outcome despite a generally excellent long-term survival rate among ovarian germ cell malignancies. It is important for clinicians to distinguish those at risk of poorer outcomes and establish individualized postoperative surveillance. Fertility-compromising surgery may be considered in selected patients.
Synchronous adrenocortical carcinoma and ovarian malignant mixed germ cell tumor: A case report and literature review.
He Ying,Xu Lian,Li Qingli,Feng Min,Wang Wei
RATIONALE:Adrenocortical carcinoma (ACC) is an endocrine malignancy with poor prognosis, which commonly arises in a sporadic manner, but may also become a part of a familial syndrome. ACC rarely arises simultaneously with other malignant tumors. PATIENT CONCERNS:We report a case of a 29-year-old woman with ACC synchronously followed by an ovarian malignant mixed germ cell tumor. We describe the clinical, histopathological, and immunohistochemical findings and review the English literatures. So far, as we know, the patient presented here is the first case with synchronous malignant tumors of the adrenal gland and ovary. DIAGNOSES:She was diagnosed with ovarian malignant mixed germ cell tumor with admixture of dysgerminoma and yolk sac tumor after ACC. INTERVENTIONS:The left adrenal tumor was resected laparoscopically on April 28, 2017. A total laparoscopic hysterectomy with unilateral (right) adnexectomy was performed on November 11, 2017. OUTCOMES:Up to now, illness condition has not progressed. Patient is free of disease at 3 months of follow-up. LESSONS:This is the first report in English literature about coexistence of ACC with ovarian malignant mixed germ cell tumor and the sixteenth case that presents a synchronous tumor associated with a sporadic ACC. This case reminds us that a comprehensive examination of patients with ACC is necessary to identify a possible synchronous tumor.