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Differentiating Merkel cell carcinoma of lymph nodes without a detectable primary skin tumor from other metastatic neuroendocrine carcinomas: the ELECTHIP criteria
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International audience. BACKGROUND: Merkel cell carcinoma MCC) can present as a cutaneous tumor or a lymph node without primary tumor MCCWP). MCCWP can be misinterpreted on histologic examination as lymph node metastasis LNM) from other neuroendocrine carcinomas LNMNECs). However, this distinction is crucial for therapeutic management. OBJECTIVE: To determine the discriminative criteria for differential diagnosis between MCCWP, LNM from cutaneous MCC and LNMNECs. METHODS: Clinical, morphologic, immunohistochemical data expression of cytokeratin AE1/AE3, 7, 19, 20; chromogranin A, thyroid transcription factor-1 [TTF-1]) and presence of Merkel Cell Polyomavirus MCPyV) immunohistochemistry and PCR) were compared in patients with MCCWP n=17), LNM from cutaneous MCC n=11) and LNMNEC n=20: lung, n=8; thyroid, n=7; digestive tract, n=3; other, n=2). RESULTS: MCC MCCWP and LNM from cutaneous MCC) differed from LNMNEC by 7 discriminative criteria: Elderly age, Location of the tumor, Extent, Cytokeratin and TTF-1 expression, Histologic types and Polyomavirus MCPyV) detection, summarized under the acronym ELECTHIP. All MCC patients had at least 5 of the ELECTHIP criteria, whereas all LNMNEC patients but 1 had fewer than 3 criteria. LIMITATIONS: The discriminant ability of the ELECTHIP criteria should be validated in a second independent set. CONCLUSION: MCCWP can be distinguished from other LNMNEC by the ELECTHIP criteria.
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