Unexpected rare uterine carcinosarcoma with neuroendocrine differentiation: Reflections on clinical diagnosis and treatment of a case report.
Medicine
RATIONALE:Uterine carcinosarcoma (UCS) is a rare and highly invasive malignant tumor.It exhibits an ectopic growth pattern of the uterus,and its histological features are biphasic differentiation of malignant epithelial components (cancer) and malignant mesenchymal components (sarcoma). The pathological pattern of high-component neuroendocrine differentiation is extremely rare. Due to the inherent heterogeneity of tumors, it increases the difficulty of accurate identification and diagnosis. The author introduces a rare case of primary endometrial carcinosarcoma (heterologous) with small cell neuroendocrine carcinoma (SCNEC) components. There is limited literature on this rare pathological differentiation pattern and a lack of guidelines for the best treatment methods, which prompts reflection on the diagnosis, optimal treatment strategies, and how preoperative diagnosis can affect patient prognosis for endometrial carcinosarcoma with neuroendocrine differentiation. PATIENT CONCERNS:The patient is an elderly woman who presents with abnormal vaginal bleeding after menopause. Transvaginal ultrasound examination shows that the uterus is slightly enlarged, and there is a lack of homogeneous echogenicity in the uterine cavity. Subsequently, a hysteroscopic curettage was performed, and a space-occupying lesion was observed on the anterior wall of the uterine cavity. DIAGNOSES:Preoperative endometrial biopsy revealed SCNEC of the endometrium. The patient underwent radical hysterectomy, and the postoperative pathological results showed that UCS (heterologous) was accompanied by SCNEC components (about 80%). INTERVENTION:The patient received radical hysterectomy, followed by adjuvant chemotherapy. OUTCOME:After 7 months of follow-up, no tumor recurrence or metastasis was found at the time of writing this article. LESSONS:The histological type of UCS (heterologous) with cell neuroendocrine carcinoma components is rare and highly invasive, with a high misdiagnosis rate in preoperative biopsy. There are currently no effective treatment guidelines for this type of case. The unusual appearance of SCNEC components in this case poses a challenge for both pathologists and surgeon. The rare differentiation pattern of this case exposes the complexity of its management and the necessity of prospective trials to determine the optimal treatment plan.
10.1097/MD.0000000000038800
Characteristics of MR Imaging for Staging and Survival Analysis of Neuroendocrine Carcinoma of the Endometrium: A Multicenter Study in Japan.
Kitajima Kazuhiro,Kihara Takako,Kawanaka Yusuke,Takahama Junko,Ueno Yoshiko,Murakami Takamichi,Yoshida Kotaro,Kato Fumi,Takahata Akiko,Fukukura Yoshihiko,Munechika Jiro,Fujinaga Yasunari,Fukunaga Takeru,Tanabe Masahiro,Kanie Yuichiro,Kido Ayumu,Tamada Tsutomu,Yoshida Rika,Kamishima Yuki,Yamakado Koichiro
Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine
PURPOSE:This study aimed to examine MRI features and staging of neuroendocrine carcinoma (NEC) of the endometrium and evaluate survival. METHODS:Clinical data, pathological, and preoperative pelvic MRI findings in 22 patients with histologically surgery-proven endometrial NEC were retrospectively reviewed. Tumors were pure NEC (n = 10) or mixed histotype (n = 12), with 13 large and nine small cell type. RESULTS:International Federation of Gynecology and Obstetrics (FIGO) staging was I, II, III, and IV in 6, 2, 12, and 2 patients, respectively. In 13 (76.4%) of 17 patients with pathological deep myometrial invasion, MRI showed abnormal diffusely infiltrative high T signal intensity throughout the myometrium with loss of normal uterine architecture. All tumors had restricted diffusion (apparent diffusion coefficient map low signal intensity, diffusion weighted imaging high signal intensity). Accuracy of T staging by MRI for all cases was 81.8%, with reference to pathology staging, while patient-based sensitivity, specificity, and accuracy for detecting metastatic pelvic lymph nodes was 60.0%, 100%, and 77.8%, respectively. Two intrapelvic peritoneal dissemination cases were detected by MRI. During follow-up (mean 30.4, range 3.3-138.4 months), 16 patients (72.7%) experienced recurrence and 12 (54.5%) died of disease. Two-year disease-free and overall survival rates for FIGO I, II, III, and IV were 66.7% and 83.3%, 50% and 100%, 10% and 33.3%, and 0% and 0%, respectively. CONCLUSION:Abnormal diffusely infiltrative high T signal intensity throughout the myometrium with normal uterine architecture loss and obvious restricted diffusion throughout the tumor are suggestive features of endometrial NEC. Pelvic MRI is reliable for intrapelvic staging of affected patients.
10.2463/mrms.mp.2020-0056
Endometrial metastasis of a primitive neuroendocrine ovarian carcinoma: management and treatment of a case.
Tartaglia E,Di Serio C,Rotondi M,Di Serio M,Scaffa C,Tolino A
European journal of gynaecological oncology
BACKGROUND:Neuroendocrine tumours are a heterogeneous group of separate clinico-pathological entities which have a common characteristic, i.e., expression of potential endocrine differentiation. In the ovary, the term "neuroendocrine" relates mainly to widely known carcinoids, but it may also be applied to rare neuroendocrine carcinomas as non-small cell type and small cell carcinomas of pulmonary type. In the literature only 11 cases of primary ovarian non-small cell neurendocrine carcinomas have been described and ten of these were associated with a surface epithelial ovarian tumour. Small cell neuroendocrine carcinoma of the ovary is a rare malignant tumour of the ovary. Advanced small cell carcinoma of the ovary is a very aggressive tumour with an overall poor prognosis and unfavourable outcome. CASE REPORT:The case reported is unique in the literature because the authors describe a rare case of endometrial metastasis of a primary ovarian non-small cell neurendocrine carcinoma without any surface epithelial ovarian tumour association. The tumour invaded up to less than half of the myometrium. The first symptoms were related to endometrial metastasis as metrorrhagia and pelvic pain while the asymptomatic presence of primary ovarian carcinoma was not acknowledged with physical examination, routine biochemistry, tumour markers, blood count and traditional transvaginal greyscale ultrasound. CONCLUSION:Magnetic resonance and three-dimensional (3D) ultrasonography with power Doppler are a great help in the diagnosis of ovarian localisation but only immunohistochemistry on histological material can provide a correct diagnosis. Immunohistochemistry expression of Ki67 is a useful marker of malignancy. Due to the rarity of this neoplasm, a general consensus for optimal treatment has yet to emerge. The reported biological aggressiveness of these tumours prompts combined treatment with radical surgery and adjuvant polychemotherapy.
Tongue base metastasis from neuroendocrine endometrial small cell carcinoma.
Marioni Gino,Savastano Marina,Mattiello Laura,Koussis Haralabos,Carpenè Silvia,Marino Filippo,Staffieri Alberto
American journal of otolaryngology
The endometrial small cell carcinoma (ESCC) is a rare and aggressive malignancy with an estimated prevalence of less than 1% of endometrial carcinomas. Endometrial small cell carcinoma is frequently diagnosed at an advanced stage and has a poor prognosis. Although the most common presenting sign of ESCC is definitely a peri- or postmenopausal vaginal bleeding, we report for the first time the occurrence of tongue base bleeding as first manifestation of disseminated neuroendocrine ESCC. From a clinical viewpoint, tongue metastasis from primary endometrial carcinoma is a manifestation of widespread disease. Albeit, our patient underwent complete hysterectomy and postoperative chemotherapy, she died of disseminated disease 3 months after ESCC diagnosis.
10.1016/j.amjoto.2006.09.011
Large-cell Neuroendocrine Carcinoma of the Endometrium in Myomatous Uterus.
Shopov Spasimir T,Anavi Benyamin L,Krastev Dobrin K
Folia medica
Large-cell neuroendocrine carcinoma of the endometrium is an insufficiently researched aggressive tumor with a short survival regard-less of the treatment type.We present here the 38th consecutive case of literature reported cases of large cell neuroendocrine carcinoma of the endometrium (posi-tive for synaptophysin, CD56, chromogranin A, p53, and Vimentin) found in the myomatous uterus of a 76-year-old woman.We also describe in the study the morphological algorithm for differentiation of malignant blastomas with small (under 10 %) neuro-endocrinal component.Accumulated clinical and morphological evidence raises the question whether the large-cell and the small-cell neuroendocrine carcino-mas are just different variations according to their histology and topical occurrence (uterine body/cervix) or they are just independent forms with their respective clinical signs, morphology, treatment, and prognosis.
10.3897/folmed.62.e49815
Primary small-cell carcinoma of the endometrium: clinicopathological study of a case and review of the literature.
Varras M,Akrivis Ch,Demou A,Hadjopoulos G,Stefanaki S,Antoniou N
European journal of gynaecological oncology
BACKGROUND:Small-cell carcinomas are almost always primary in the lungs and are highly malignant. These tumors may also occur in the female genital tract. However, primary small-cell carcinoma of the endometrium is extremely rare with very few cases reported in the English literature. This tumor may exhibit evidence of neuroendocrine differentiation and has a high propensity for systemic spread and poor prognosis. CASE:A 55-year-old postmenopausal woman with primary small-cell carcinoma of the endometrium, FIGO stage Ib, underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and sampling node biopsies of the parametrial spaces, followed by adjuvant combined chemotherapy. CONCLUSION:A case of small-cell carcinoma of the endometrium, is reported and its clinical, histological and immunohistochemical features are discussed.
Synchronous endometrioid carcinoma of the endometrium and small cell neuroendocrine carcinoma of the cervix: a rare combination.
Nakra Tripti,Biswas Rituparna,Pandey Rambha,Yadav Rajni
BMJ case reports
Synchronous multiple primaries of female genital tract are uncommon, with the most frequently encountered combination being of endometrium and ovary. Concurrent primary tumours of endometrium and cervix are rare. We report a case of coexistent endometrioid carcinoma of the endometrium and small cell neuroendocrine carcinoma of the cervix in 48-year-old woman who presented with menometrorrhagia and was detected to have metastases to distant sites on imaging. She underwent multimodality treatment which resulted in a significant reduction in the tumour bulk.
10.1136/bcr-2018-227155
New imaging modalities to distinguish rare uterine mesenchymal cancers from benign uterine lesions.
Current opinion in oncology
PURPOSE OF REVIEW:Uterine sarcomas are rare and are often challenging to differentiate on imaging from benign mimics, such as leiomyoma. As functional MRI techniques have improved and new adjuncts, such as machine learning and texture analysis, are now being investigated, it is helpful to be aware of the current literature on imaging features that may sometimes allow for preoperative distinction. RECENT FINDINGS:MRI, with both conventional and functional imaging, is the modality of choice for evaluating uterine mesenchymal tumors, especially in differentiating uterine leiomyosarcoma from leiomyoma through validated diagnostic algorithms. MRI is sometimes helpful in differentiating high-grade stromal sarcoma from low-grade stromal sarcoma or differentiating endometrial stromal sarcoma from endometrial carcinoma. However, imaging remains nonspecific for evaluating rarer neoplasms, such as uterine tumor resembling ovarian sex cord tumor or perivascular epithelioid cell tumor, primarily because of the small number and power of relevant studies. SUMMARY:Through advances in MRI techniques and novel investigational imaging adjuncts, such as machine learning and texture analysis, imaging differentiation of malignant from benign uterine mesenchymal tumors has improved and could help reduce morbidity relating to misdiagnosis or diagnostic delays.
10.1097/CCO.0000000000000758
A case of primary small cell neuroendocrine carcinoma of the uterus.
Radiology case reports
Neuroendocrine carcinoma of the uterine endometrium is extremely rare and found in <1% of all primary endometrial carcinomas. We report a case of neuroendocrine carcinoma of the endometrium detected in a 65-year-old woman and focus our attention on the main imaging features. The low apparent diffusion coefficient value and high maximum standardized uptake value for neuroendocrine cancer serve to distinguish this cancer from endometrial cancer.
10.1016/j.radcr.2022.07.065