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24 September 2014 - Case of the Week #327
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Thanks to Dr. Jamie Shutter, Women's Care Florida Laboratory (USA), for contributing this case.
This case was reviewed in May 2020 by Dr. Jennifer Bennett, University of Chicago and Dr. Carlos Parra-Herran, University of Toronto.
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Case of the Week #327
A 27 year old woman had a 6 cm cervical mass, which was biopsied. The outside diagnosis was poorly differentiated carcinoma, suggestive of adenocarcinoma.
What is your diagnosis?
Neuroendocrine carcinoma of the cervix, small cell type
The biopsy shows a poorly differentiated carcinoma with neuroendocrine histology, including a vague ribbon pattern and salt and pepper chromatin. There is hyperchromasia and nuclear molding, as well as conspicuous apoptosis and mitotes. Immunohistochemistry also confirmed the tumor's neuroendocrine features:
Additional immunostain results were focal p63 staining, and negative staining for HMB45, LCA / CD45, CK20, NSE, polyclonal CEA, vimentin and p53. The morphologic features and positive (albeit not strong) neuroendocrine immunostains are most consistent with a poorly differentiated malignancy, namely small cell neuroendocrine carcinoma. The clinical presentation and the overexpression of p16 (acting as surrogate of HPV infection) are most in keeping with a primary cervical origin.
Cervical neuroendocrine carcinomas, whether small cell or large cell type, are aggressive tumors with rapid metastases. They are frequently treated with a combination of chemotherapy and radiation, so the initial biopsy may be the only time to get diagnostic tissue.
The differential diagnosis includes:
• metastatic neuroendocrine carcinoma from the lung or another primary site (clinical history, negative p16 / HPV molecular studies)
• carcinoid tumor (distinct architecture with insular, trabecular, tubular morphology, and absence of high-grade cytomorphology)
• basaloid squamous cell carcinoma (distinct nesting, absence of nuclear molding and brisk apoptosis / mitoses, strong p63 staining, no neuroendocrine features)
Nat Pernick, M.D., President
and Shivani Thakore, Associate Medical Editor
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
Alternate email: NatPernick@gmail.com