Large cell neuroendocrine carcinoma of the endometrium: An extremely uncommon diagnosis, but worth the efforts

Deodhar, Kedar; Kerkar, Rajendra; Suryawanshi, Pallavi; Menon, Hari; Menon, Santosh

doi:10.4103/0973-1482.82942

Cited by 31 publications

(25 citation statements)

References 6 publications

(14 reference statements)

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“…Most studies published on endometrial NECa have demonstrated keratin positivity in tumor cells, but keratin negative cases have been reported 3, 4, 9, 10, 24, 26 and are encountered in routine practice. In our series, 17 of 18 cases were positive with both a sensitive pankeratin cocktail and with keratin 18, most commonly in a patchy or diffuse distribution.…”

Section: Discussionmentioning

confidence: 99%

Neuroendocrine Carcinoma of the Endometrium

Pocrnich

Ramalingam

Euscher

et al. 2016

American Journal of Surgical Pathology

108

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Neuroendocrine carcinoma (NECa) of the endometrium is an uncommon tumor. In this study, we present the clinicopathologic features of 25 such cases. The patients ranged in age from 37 to 87 years (median, 57), and most commonly presented with vaginal bleeding. The tumors were either pure NECa (10) or mixed with other histotypes (15), most commonly endometrioid carcinoma. The NECas were large cell type (15), small cell type (4), or a mixture of both (6). NECa was under-recognized in 89% of referral/consultation cases. All tumors were positive for ≥ 1 neuroendocrine marker (chromogranin, synaptophysin, CD56). Additional immunohistochemical (IHC) studies were obtained in 18 cases, with positive results as follows: keratin cocktail (17), diffuse p16 (6), PAX-8 (6), CD117 (6), and TTF-1 (1). Mismatch repair protein expression by IHC was abnormal in 8 of 18 cases (6 MLH1/PMS2 loss; 1 MSH2/MSH6 loss; 1 MSH6 loss). According to FIGO staging, cases were distributed as follows: I (6), II (2), III (10), and IV (7). All patients underwent surgical treatment, and 20 patients received adjuvant therapy. Twelve patients died of disease (mean survival 12.3 months). Eleven patients were alive 5 to 134 months following diagnosis, including 7 who achieved a 5-year survival (3 stage I; 4 stage III). In summary, most of our endometrial NECas were large cell type, mixed with other histotypes, and under-recognized. These tumors tend to be PAX-8 negative and may be associated with microsatellite instability. The recognition of NECa may have an impact on the treatment of the patients affected by this disease. Although NECa usually has an aggressive behavior, 28% of our patients survived at least 5 years.

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Section: Discussionmentioning

confidence: 99%

Neuroendocrine Carcinoma of the Endometrium

Pocrnich

Ramalingam

Euscher

et al. 2016

American Journal of Surgical Pathology

108

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“…As for the chemotherapy, patients are generally received six cycles of cisplatin (60 mg/m 2 , day 1) and etoposide (60 mg/m 2 , day 1, 8, and 15). 2,7,10,14 In Case #1, because the patient had numerous metastases throughout her body, it was impossible for her to undergo surgery. Administration of chemotherapy was considered, but due to worsening of the patient's performance status, any active treatment was impossible.…”

Section: Discussionmentioning

confidence: 99%

Large Cell Neuroendocrine Carcinoma Originating from the Uterine Endometrium: A Report on Magnetic Resonance Features of 2 Cases with very Rare and Aggressive Tumor

Makihara¹,

Maeda²,

Nishimura³

et al. 2012

Rare Tumors

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Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and uncommon tumors, which usually involve the uterine cervix and ovary, and are seen very rarely in the endometrium. Only less than 10 cases of large cell NEC (LCNEC) of the endometrium have been reported in the literature and their radiological findings are not well described. We report here two cases of pathologically proven LCNEC of the uterine endometrium. In both cases, the uterine body was enlarged and the tumor occupied part of the uterine cavity. Endometrial mass exhibited heterogeneous high intensity on T2-weighted magnetic resonance (MR) images, and diffusion-weighted MR images revealed high intensity throughout the tumor, consistent with malignancy. LCNEC is a highly malignant neoplasm without particular findings in terms of diagnostic imaging and pathology, so its preoperative definitive diagnosis is very difficult. However, when laboratory test, pathologic diagnosis and MR imaging suggest a poorly differentiated uterine malignancy, positron emission tomography-computed tomography scan should be performed as a general assessment to help with diagnosis.

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“…LCNEC arising from the endometrium are rarer than those arising from the cervix and ovaries [4][5][6][7][8][9][10][11][12][13][14]. Based on published reports in English, the neoplasms are either "pure" LCNEC or admixed with other histologic components [5][6][7][8][9][10][11][12][13][14][15][16]. Among other histologic components of endometrial LCNEC, endometrioid carcinoma is the most common histologic type, followed by serous carcinoma.…”

Section: Discussionmentioning

confidence: 99%

Endometrial neuroendocrine carcinoma frequently associated with mismatch repair deficiency

Zhou

Chen

et al. 2020

Preprint

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Background: Endometrial neuroendocrine carcinoma (NEC) is a uncommon histologic subtype of endometrial cancer. It is currently unclear if the endometrial NEC has any particular relationship with mismatch repair (MMR) protein expression patterns. Here we report 3 endometrial NEC cases showing loss of MMR expression.Case presentation: The cancers had two components with one poorly differentiated or NEC component, accounting for 60–90% of the neoplasms and the other of well differentiated glandular component. The NEC cells mainly showed solid sheets and organoid patterns, whereas insular, trabecular and rosette/pseudorosette patterns were also found. The NEC component was composed of cells with large, polygonal, vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. In all cases, the NEC component was diffusely positive for p16 and at least 2 of the 3 neuroendocrine markers (CGA, SYN, CD56) reactive in > 10% of the cancer cells. Loss of MMR protein expression was found in all three cases with loss of MLH1 in cases 1 and 2 and loss of MSH2 and MSH 6 in case 3. No PMS2 loss was found. In addition, aberrant TP53 and SMARCB1 (INI1) expression was found in case 3 only. All patients underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy, and two of them received postoperative chemotherapy and/or radiation therapy. The patients remained alive with no disease for 51, 17 and 6 months, respectively.Conclusion: Endometrial NEC is frequently associated with MMR deficiency, some of which may have a better prognosis than we expect.

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Large cell neuroendocrine carcinoma of the endometrium: An extremely uncommon diagnosis, but worth the efforts

Cited by 31 publications

References 6 publications

Neuroendocrine Carcinoma of the Endometrium

Neuroendocrine Carcinoma of the Endometrium

Large Cell Neuroendocrine Carcinoma Originating from the Uterine Endometrium: A Report on Magnetic Resonance Features of 2 Cases with very Rare and Aggressive Tumor

Endometrial neuroendocrine carcinoma frequently associated with mismatch repair deficiency

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