Merkel's cell (neuroendocrine) carcinoma of the vulva

Abstract

Background: Merkel's cell carcinoma rarely arises in the vulva. It is unclear whether those cases arising in the vulva behave differently from the usual Merkel's cell carcinomas.

Methods: A Merkel's cell carcinoma of the vulva was studied by light microscopy, immunohistochemistry, and electron microscopy. The clinical data of this case and of other reported cases are summarized and compared with those of Merkel's cell carcinoma in general.

Results: This tumor showed the characteristic trabecular pattern of Merkel's cell carcinoma. The tumor cells were immunoreactive to low-molecular-weight cytokeratin and neuron-specific enolase. This patient was treated initially with local excision. She died 17 months later with progressive metastatic disease unresponsive to chemotherapy. This case study and the review of six other cases indicate that Merkel's cell carcinoma of the vulva is universally metastatic, both to the regional lymph nodes and distant sites, and that it invariably follows a rapidly fatal course.

Conclusion: Merkel's cell carcinoma of the vulva appears to have a biologic behavior more aggressive than that of Merkel's cell carcinoma in general. An initial modality combining chemotherapy, with or without irradiation, with aggressive surgery should be tried in future cases.