Mixed neuroendocrine and endometrioid carcinoma of the endometrium: a rare aggressive malignancy

Correspondence to Dr Sonia Badwal, Pathology (Histopathology division), Sir Ganga Ram Hospital, New Delhi, Delhi, 110060, India; soniabadwal06@gmail.com Uterine neuroendocrine carcinomas are rare, accounting for <1% of endometrial carcinomas, and include both large cell and small cell types.1 Pro...

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Published inInternational journal of gynecological cancer Vol. 34; no. 11; p. 1828
Main Authors Bakshi, Neha, Modi, Rahul D, Srivastava, Mala, Badwal, Sonia
Format Journal Article
LanguageEnglish
Published England BMJ Publishing Group LTD 04.11.2024
Subjects
Cancer
Cytokeratin
Cytoplasm
Editing
Endometrial cancer
Endometrium
Estrogens
Females
Medical prognosis
Metastasis
Morphology
Pathology
Proteins
Tumors
Neuroendocrine Tumors
Endometrial Neoplasms
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Summary:Correspondence to Dr Sonia Badwal, Pathology (Histopathology division), Sir Ganga Ram Hospital, New Delhi, Delhi, 110060, India; soniabadwal06@gmail.com Uterine neuroendocrine carcinomas are rare, accounting for <1% of endometrial carcinomas, and include both large cell and small cell types.1 Prognosis is distinctly worse, even in early stage tumors, with strong propensity for rapid recurrence and distant metastasis.2 3 Accurate diagnosis is therefore crucial to improve patient survival. According to the World Health Organization (WHO) 2020 classification, diagnosis of large cell neuroendocrine carcinoma requires a combination of this high grade neuroendocrine histomorphology and expression of at least one neuroendocrine marker (synaptophysin, chromogranin, or CD56) in >10% tumor cells.2 3 Chromogranin positivity is more specific, as synaptophysin expression is reported in both endometrioid (21%) and serous (13%) endometrial carcinomas, while CD56 can stain endometrioid endometrial and ovarian carcinomas.2 INSM1 is a novel immunohistochemistry marker, with superior performance to conventional neuroendocrine markers in cervical tumors.4 Cytokeratin is usually positive, but rare cytokeratin negative cases are documented in the literature.2 Up to two thirds of neuroendocrine carcinomas are negative for PAX8, and these tumors may be associated with microsatellite instability (up to 44% of cases).2 Uterine neuroendocrine carcinomas may be histologically pure, but are frequently (50–80%) admixed with non-neuroendocrine carcinoma (endometrioid >serous) components, and such cases are classified by WHO as combined or mixed neuroendocrine carcinomas. Twitter @, @ModiRahul86 Contributors NB: design, literature search, data acquisition, data analysis, manuscript preparation, and manuscript editing.
ISSN:1048-891X
1525-1438
DOI:10.1136/ijgc-2023-004929