NEUROENDOCRINE CARCINOMA OF THE UTERUS (original) (raw)
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Annals of Diagnostic Pathology, 2013
Neuroendocrine carcinoma cervix Small cell carcinoma cervix Large cell neuroendocrine carcinoma cervix Immunohistochemistry in cervix carcinomas Cervix cancer Neuroendocrine carcinomas of the cervix are uncommon, characterized by a histomorphological spectrum and, mostly, an aggressive clinical course. There are only few substantial studies on such cases documented from our country, where cervical cancer is the second most common cancer affecting women. Herein, we present a spectrum of 50 cervical neuroendocrine carcinomas, including histopathologic features, terminology, immunohistochemical (IHC) profile, and clinical outcomes, wherever available. Fifty tumors occurred in women, with their age ranging from 23 to 69 years (mean, 48.6 years; median, 46.5 years). Stagewise, among 25 cases, most cases (6, or 24%) presented with stage IB. Average tumor size was 4.7 cm. On histopathologic review, 26 tumors (52%) were classified as small cell carcinoma (SMCA); 14 (28%), as large cell neuroendocrine carcinomas (LCNECs); 4 (8%), as SMCA + LCNECs; and 6, as mixed carcinomas, including 3 tumors (6%) with SMCA and squamous cell carcinoma (SCC), 2 tumors (4%) with LCNEC and adenocarcinoma, and a single tumor (2%) with LCNEC and squamous cell carcinoma. On IHC performed in 41 tumors (82%), 36 tumors (87.8%) were positive for at least a single neuroendocrine marker, and 22 (53.6%) expressed 2 neuroendocrine markers. Synaptophysin was positive in 22 (59.4%) of 37 tumors; chromogranin, in 27 (72.9%) of 37; CD56, in 8 (100%) of 8; and neuron-specific enolase in 7 (87.5%) of 8 tumors. Treatment wise, among 30 patients (60%), 6 (20%) underwent surgery, including Wertheim hysterectomy (5) and simple hysterectomy (1); 8 (26.6%) underwent surgery with adjuvant treatment, and 10 patients (33.3%) were offered chemotherapy and/or radiotherapy. On follow-up (27 patients, or 54%) over 1 to 144 months, 16 patients (59.2%) were alive with disease over median duration of 9 months, and 7 (25.9%) were free of disease over median duration of 26.5 months. There were 5 recorded deaths. Thirteen tumors (48.1%) metastasized, most commonly to liver. In cases with early stage disease and adjuvant treatment, including radiotherapy, LCNEC histology fared well. This study forms the largest documented series on cervical neuroendocrine carcinomas from our country, testifying the current histopathologic classification system. Although SMCAs can be recognized on morphology, LCNECs need to be correctly identified because these can be misdiagnosed in the absence of neuroendocrine markers. Synaptophysin, chromogranin, and CD56 are optimal IHC markers. Small cell carcinomas, pure or mixed, are relatively more aggressive. All these tumors are best treated with multimodal therapy. Early stage disease treated with radical surgery and adjuvant treatment seems to increase survival. Despite aggressive treatment, prognosis is dismal.
An 84-year-old woman suffered from post-menopausal genital bleeding for 3 months. Based on the endometrial cytological findings (suggestive of high grade neuroendocrine carcinoma) showing that there were rosette-like and cord-like structures consisting of small rounded tumor cells with oval nuclei and scanty cytoplasm, radical hysterectomy was performed. Histopathological and immunohistochemical examinations on the operated specimens revealed primary high grade neuroendocrine carcinoma of the endometrium. Despite the extensive treatment against the malignancy, the patient died due to widespread metastases after 5 months after the surgery and autopsied.
Neuroendocrine carcinoma of the endometrium: A very rare gynecologic malignancy
Journal of Gynecology Obstetrics and Human Reproduction, 2020
To investigate the clinicopathologic characteristics, prognostic factors, outcome, and treatment of the neuroendocrine carcinoma (NEC) of the endometrium. Materials and methods: We retrospectively reviewed the clinicopathologic and survival data of 10 patients who underwent surgery for NEC. The patients were collected between 1999 and 2017 from four referral centers in Turkey. Results: The median age of patients was 67 years (range: 34-75 years). The NEC of endometrium consist of 9 cases with small cell carcinoma (SC) NEC (two with mixed histotypes), and one with a large cell (LC) NEC. According to FIGO 2009 criteria, 70 % (7/10) of patients had advanced stage (III and IV) disease. All patients except one underwent surgical staging, eight patients received platinum-based chemotherapy (CTX) and of 6 those were additionally treated with radiotherapy (RT). Four patients died of disease ranging from 2 to 10 months and six were alive 12-72 months with no evidence of disease. In addition, 4 SC NEC cases raised in polypoid features had no evidence of disease from 24 to 72 months. Discussion: NEC of the endometrium is a rare disease with poor prognosis, which frequently diagnosed in advanced stages. The main treatment modality was the administration of platinum-based CTX as an adjuvant to surgery or surgery and RT. Our result suggests that the polypoid feature of the tumor might be one of the best predictors for the prognosis of SC NEC.
Journal of Chemotherapy, 2006
Background. Gynecological neuroendocrine neoplasms (NENs) are extremely rare, accounting for 1.2-2.4% of the NENs. The aim of this study was to test cervical NENs for novel markers of potential utility for differential diagnosis and target therapy. Methods. All cases of our center (n = 16) were retrieved and tested by immunohistochemistry (IHC) for 12 markers including markers of neuroendocrine differentiation (chromogranin A, synaptophysin, CD56), transcription factors (CDX2 and TTF1), proteins p40, p63, p16INK4a, and p53, somatostatin receptors subtypes (SST2-SST5) and the proliferation marker Ki67 (MIB1). Results. All cases were poorly differentiated neuroendocrine carcinomas (NECs), 10 small cell types (small cell-neuroendocrine carcinomas, SCNECs) and 6 large cell types (large cell-neuroendocrine carcinomas, LCNECs); in 3 cases a predominant associated adenocarcinoma component was observed. Neuroendocrine cancer cells expressed at least 2 of the 3 tested neuroendocrine markers; p16 was intensely expressed in 14 (87.5%) cases; SST5 in 11 (56.25%, score 2-3, in 9 cases); SST2 in 8 (50%, score 2-3 in 8), CDX2 in 8 (50%), TTF1 in 5 (31.25%), and p53 in 1 case (0.06%). P63 and p40 expressions were negative, with the exception of one case that showed moderate expression for p63. Conclusions. P40 is a more useful marker for the differential diagnosis compared to squamous cell carcinoma. Neither CDX2 nor TTF1 expression may help the differential diagnosis versus potential cervical metastasis. P16 expression may suggest a cervical origin of NEC; however, it must be always integrated by clinical and instrumental data. The expression of SST2 and SST5 could support a role for SSAs (Somatostatin Analogues) in the diagnosis and therapy of patients with cervical NECs.
2019
Large cell neuroendocrine carcinoma (LCNEC) of the endometrium is a rare and highly malignant neoplasm with no characteristic findings in terms of clinical manifestations, diagnostic imaging, or pathology, and thus, the definitive preoperative diagnosis of LCNEC is difficult. A 61-year-old postmenopausal woman presented with low abdominal pain and a rapidly growing uterine mass without postmenopausal bleeding. Magnetic resonance imaging of the pelvis revealed an enlarged uterus with a 7.5 cm mass. Intraoperative frozen examination revealed a malignant tumor, and accordingly, cytoreductive surgery was performed. Microscopically, the tumor showed extensive necrosis, hemorrhage, and an organoid nesting pattern of large cells. Immunohistochemistry revealed tumor cells were diffusely positive for the neuroendocrine markers CD56 and synaptophysin. Thus the tumor was diagnosed as LCNEC of endometrium. Postoperatively, the disease pursued a progressive course and relapsed even after repeate...
Biomedical Research-tokyo, 2017
Neuroendocrine Tumours (NEC), are neoplasms that arise from cells of the endocrine (hormonal) and nervous systems. Many are benign, while some are malignant. They most commonly occur in the lung, intestine, where they are often called carcinoid tumours, but they are also found in the pancreas, and the rest of the body. The female genital counterpart is aggressive and uncommon, with propensity to involve the cervix and ovary, the endometrium variant is seldom occurring and tends to be of a conventional small cell type (up to 60 cases). Only twelve cases of large cell type NEC. In our centre, we diagnose a case of Large-Cell Neuroendocrine Carcinoma (LCNEC) of the, endometrium in an 82 y old female. It was report it as mixed type tumour papillary serous and large neuroendocrine, with FIGO nuclear grade III, the endocrine origin of the tumour was confirmed by immunohistochemically endocrine marker CD56 and NSE. Patient medical condition was unfit for surgery and she chose palliative treatment.
Neuroendocrine carcinoma of cervix and review literature
Neuroendocrine carcinoma of the cervix (NECC) is a rare variant of cervical cancer with poor prognosis and high mortality. Recurrence is seen with multi-organ metastasis including liver. Case presentation: A 65 year old female presented with vaginal bleeding for the past one year. Cervical cancer screening and biopsy demonstrated poorly differentiated squamous carcinoma. Immunohistochemistry showed positive expression of chromogranin, synaptophysin, pancytokeratin, TTP1, and CEA and negative expression of p40 and estrogen receptors. An adenocarcinoma with neuroendocrine tumor was suggested. Hysterectomy with bilateral salpingo-oophrectomy was performed. This was followed by carboplatin and etoposide therapy to have clinical remission for a year. Then recurrence was observed to start same drugs again resulting in to partial improvement. It was followed by radiotherapy. The patient succumbed to death approximately after three months. Conclusion: A metastatic lesion in liver may be a case of Neuroendocrine tumor of cervix, a rare condition that can be easily missed on histopathological examination. More studies are required to establish a standard therapeutic protocol.
The Significance of Neuroendocrine Expression in Undifferentiated Carcinoma of the Endometrium
International Journal of Gynecological Pathology Official Journal of the International Society of Gynecological Pathologists, 2009
Carcinomas with neuroendocrine (NE) differentiation have been associated with poor outcome in different organs. The purpose of this study was to evaluate the presence and significance of NE expression in a series of 46 undifferentiated endometrial carcinomas diagnosed between 1988 and 2005. NE expression was studied by immunohistochemistry including synaptophysin, chromogranin, and/or CD56. The patients' age ranged from 30 to 84 years (mean 55). Staging information was available for 45 cases and according to the International Federation of Gynecology and Obstetrics system they were distributed as follows: stage I (9 cases), stage II (2 cases), stage III (9 cases), and stage IV (25 cases). NE expression was present in 19/46 (41%) cases; however, it was diffuse in only 9% of the tumors. The median survival for patients without NE expression was 7 months (95% confidence interval 4-10 mo) and for patients with NE expression was 12 months (95% confidence interval 6-27 mo). The survival curves do not differ significantly (P ΒΌ 0.49). NE expression is common in undifferentiated carcinoma of the endometrium, as it was found in 41% of our cases. In most cases, NE expression is only focally present (r10% of the cells). There is no difference in overall survival in patients with or without NE expression.
Large-cell neuroendocrine carcinoma of the uterine cervix--a clinicopathological study of five cases
PubMed, 2001
Objective: The present study describes 5 cases of large-cell neuroendocrine carcinoma (LCNEC) of the uterine cervix, evaluating their clinical features and pathological profiles. Methods: Clinical data were obtained from the patients' clinical files at the combined gynaecological-oncology unit of Johannesburg Hospital and the University of the Witwatersrand Medical School, Johannesburg, South Africa. A histopathological diagnosis was obtained after biopsy material from all 5 patients was examined microscopically and subjected to immunohistochemical staining with MNF116 (pankeratin) synaptophysin and chromagranin A, all of which are neuroendocrine markers. Two patients received pelvic radiotherapy only. None of the 5 patients in this series received chemotherapy or underwent surgery. Results: All 5 patients were adult females, with an average age of 57.3 years. The majority were multiparous, with the most common presenting complaint being vaginal bleeding. Three of the 5 patients presented with advanced-stage cervical carcinoma, with evidence of metastases in 2 of them. Treatment responses and long-term survival in our series proved to be disappointing as 3 of the 5 patients died in less than 6 months. On histopathological examination, all 5 tumours showed features of a high-grade poorly differentiated malignant neoplasm with ulceration and extensive tumour necrosis including trabecular and organoid growth patterns. All 5 neoplasms also showed strong immunoreactivity for MNF116, while their endocrine nature was confirmed by staining for synaptophysin in all cases. None of the tumours showed positive straining for chromagranin A. Conclusions: LCNECs are rare tumours and distinct from other neoplasms of the uterine cervix. The results of this study reaffirm the biologically aggressive nature of this uncommon tumour and its very unfavourable prognosis.