Synchronous endometrioid carcinoma of the endometrium and small cell neuroendocrine carcinoma of the cervix: a rare combination

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.

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Mixed large and small cell neuroendocrine carcinoma and endometrioid carcinoma of the endometrium with high microsatellite instability: A case report and literature review

SAGE Open Medical Case Reports ◽

10.1177/2050313x21999200 ◽

2021 ◽

Vol 9 ◽

pp. 2050313X2199920

Author(s):

Kotaro Inoue ◽

Kentaro Kai ◽

Shimpei Sato ◽

Haruto Nishida ◽

Koji Hirakawa ◽

...

Keyword(s):

Microsatellite Instability ◽

Neuroendocrine Carcinoma ◽

Immune Checkpoint Blockade ◽

Japanese Woman ◽

Endometrial Biopsy ◽

Small Cell ◽

Endometrioid Carcinoma ◽

Small Cell Neuroendocrine Carcinoma ◽

Mutation Burden ◽

Solid Part

A 65-year-old, gravida 3, para 2 Japanese woman was referred to our hospital for symptomatic thickening of the endometrial lining. Endocervical and endometrial cytology revealed an adenocarcinoma. The endometrial biopsy specimen was mixed, with a glandular part diagnosed as endometrioid carcinoma and a solid part diagnosed as high-grade mixed large and small cell neuroendocrine carcinoma (L/SCNEC). She underwent extra-fascial hysterectomy with bilateral salpingo-oophorectomy, complete pelvic and para-aortic lymphadenectomy, and omentectomy (FIGO IIIB, pT3b pN0 M0). She currently has no deleterious germline mutation, but high tumor mutation burden and high microsatellite instability (MSI) were identified. She underwent six cycles of platinum-based frontline chemotherapy and achieved complete remission. Immune checkpoint blockade therapy is a promising second-line therapy for MSI-high solid tumors. However, the MSI or mismatch repair (MMR) status of endometrial L/SCNEC remains unclear in the literature. Universal screening for MSI/MMR status is needed, particularly for a rare and aggressive disease.

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1119P Genomic alterations of neuroendocrine carcinoma originated from female genital tract

Annals of Oncology ◽

10.1016/j.annonc.2021.08.201 ◽

2021 ◽

Vol 32 ◽

pp. S919

Author(s):

M. Fang ◽

Z. Xu ◽

J. Cao ◽

P. Zhang ◽

H. Zhang ◽

...

Keyword(s):

Neuroendocrine Carcinoma ◽

Genital Tract ◽

Female Genital Tract ◽

Genomic Alterations ◽

Female Genital

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Emerging Therapeutic Concepts and Latest Diagnostic Advancements Regarding Neuroendocrine Tumors of the Gynecologic Tract

Medicina ◽

10.3390/medicina57121338 ◽

2021 ◽

Vol 57 (12) ◽

pp. 1338

Author(s):

Tiberiu-Augustin Georgescu ◽

Roxana Elena Bohiltea ◽

Octavian Munteanu ◽

Florentina Furtunescu ◽

Antonia-Carmen Lisievici ◽

...

Keyword(s):

Neuroendocrine Tumors ◽

Neuroendocrine Carcinoma ◽

Classification System ◽

Who Classification ◽

Small Cell ◽

International Agency ◽

Low Grade ◽

High Grade ◽

Small Cell Neuroendocrine Carcinoma ◽

Female Genital

Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on histologic subtype and site of origin. Following the expert consensus proposal of the International Agency for Research on Cancer (IARC), the approach in the latest World Health Organization (WHO) Classification System of the Female Genital Tumours is to use the same terminology for NENs at all body sites. The main concept of this novel classification framework is to align it to all other body sites and make a clear distinction between well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The previous WHO Classification System of the Female Genital Tumours featured more or less the same principle, but used the terms ‘low-grade neuroendocrine tumor’ and ‘high-grade neuroendocrine carcinoma’. Regardless of the terminology used, each of these two main categories include two distinct morphological subtypes: NETs are represented by typical and atypical carcinoid and NEC are represented by small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). High-grade NECs, especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade NETs usually occur in the ovary. NENs of the vulva, vagina and fallopian tube are exceptionally rare, with scattered case reports in the scientific literature.

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Small cell cancers of the female genital tract: Molecular and clinical aspects

Gynecologic Oncology ◽

10.1016/j.ygyno.2018.02.004 ◽

2018 ◽

Vol 149 (2) ◽

pp. 420-427 ◽

Cited By ~ 15

Author(s):

Jay R. Patibandla ◽

Julia E. Fehniger ◽

Douglas A. Levine ◽

Petar Jelinic

Keyword(s):

Genital Tract ◽

Female Genital Tract ◽

Small Cell ◽

Clinical Aspects ◽

Female Genital

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Phase II study of pembrolizumab efficacy and safety in women with recurrent small cell neuroendocrine carcinoma of the lower genital tract

Gynecologic Oncology ◽

10.1016/j.ygyno.2020.05.682 ◽

2020 ◽

Vol 158 (3) ◽

pp. 570-575 ◽

Cited By ~ 5

Author(s):

Michael Frumovitz ◽

Shannon N. Westin ◽

Gloria Salvo ◽

Abdulrazzak Zarifa ◽

Mingxuan Xu ◽

...

Keyword(s):

Phase Ii ◽

Neuroendocrine Carcinoma ◽

Genital Tract ◽

Phase Ii Study ◽

Small Cell ◽

Efficacy And Safety ◽

Small Cell Neuroendocrine Carcinoma ◽

Lower Genital Tract

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Small cell carcinoma of the female genital tract: A single institution experience.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.e15540 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. e15540-e15540

Author(s):

Faraj El-Gehani ◽

Julia Sun ◽

Muna Kamal ◽

Danielle Pertschy ◽

Kurian Joseph ◽

...

Keyword(s):

Genital Tract ◽

Female Genital Tract ◽

Retrospective Chart Review ◽

Distant Recurrence ◽

Cranial Irradiation ◽

Concurrent Chemotherapy ◽

Small Cell ◽

Entire Cohort ◽

Limited Stage ◽

Female Genital

e15540 Background: Small cell carcinomas (SCC) of the female genital tract (FGT) are rare and there is no standard treatment strategy for managing these patients (pts). The objectives of this study are to report the clinical experience and management of pts with SCC of the FGT, treated at the Cross Cancer Institute, Edmonton, AB, Canada from 1982 to 2009. Methods: A retrospective chart review of all pts diagnosed with SCC of the FGT between 1982 and 2009 was undertaken. Data was collected on demographics, clinical and pathological characteristics, and patient outcomes. Results: Twenty-seven pts were identified with primary sites as follows: cervix 16 (59%), endometrium 7 (26%), ovary 3 (11%) and vagina 1 (4%). Median age at diagnosis was 53 years. Seventy-eight percent (21/27) had pure SCC; the rest had mixed histology. Seventy percent (19/27) were positive for at least one neuroendocrine marker. Overall, thirty percent (8/27) had limited stage (LS) disease; Seventy percent (19/27) had extensive disease (ES). Treatment of limited stage pts was 3/8 (38%) concurrent chemotherapy with radiation, 3/8 (38%) surgery, 1/8 (12%) chemotherapy alone and 1/8 (12%) radiation alone. For extensive stage pts, 6/19 (31%) treated with surgery with or without adjuvant chemotherapy, 4/19 (21%) concurrent chemotherapy with radiation, 2/19 (11%) palliative chemotherapy and 7/19 (37%) supportive care only. For relapsing pts brain was the site of first distant recurrence in three pts. Median survival for the entire cohort was 7.4 months (25.9 months for limited stage disease and 4.8 months for extensive; p < .0001). Conclusions: SCC of the FGT tract is aggressive with an overall poor prognosis. There is no standard of care for these pts and they are often treated according to local protocols based on data extrapolated from SCC lung cancer. Incidence of brain metastasis as the site of first relapse is relatively lower in FGT SCC, suggesting unclear benefit to prophylactic cranial irradiation.

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Neuroendocrine Carcinoma of Female Genital Tract: Series of Nine Cases

Indian Journal of Gynecologic Oncology ◽

10.1007/s40944-020-00480-x ◽

2021 ◽

Vol 19 (1) ◽

Author(s):

Vishakha C. Bidkar ◽

Geeta Acharya ◽

Kiran Abhijit Kulkarni ◽

G. Sumangala ◽

T. S. Premalatha ◽

...

Keyword(s):

Neuroendocrine Carcinoma ◽

Genital Tract ◽

Female Genital Tract ◽

Female Genital

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Effective multimodality treatment for advanced epidermoid carcinoma of the female genital tract.

Journal of Clinical Oncology ◽

10.1200/jco.1985.3.7.917 ◽

1985 ◽

Vol 3 (7) ◽

pp. 917-924 ◽

Cited By ~ 7

Author(s):

J Kalra ◽

E Cortes ◽

S Chen ◽

B Krumholz ◽

J J Rovinsky ◽

...

Keyword(s):

Squamous Cell Carcinoma ◽

Genital Tract ◽

Female Genital Tract ◽

Multimodality Treatment ◽

Tumor Shrinkage ◽

Simultaneous Administration ◽

Recurrent Squamous Cell Carcinoma ◽

Complete Tumor ◽

Female Genital

Fifteen patients with advanced or recurrent squamous-cell carcinoma of the cervix, vulva, vagina, and urethra were treated with simultaneous combination chemotherapy (5-fluorouracil infusion and mitomycin C) and radiotherapy (3,000 rad for a period of three weeks). Three to four weeks after completion of radiotherapy, 13 of 15 patients achieved partial or complete tumor shrinkage. Nine of 15 patients are alive, eight of whom (at a median follow-up time of 24 months) have no evidence of disease. The longest survival time was 45 + months. There was minimal toxicity associated with this therapy. The results of this pilot study suggest that the simultaneous administration of radiation and chemotherapy is an effective method of treatment of advanced female genital tract carcinoma.

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