Primary Cutaneous Neuroendocrine Tumor of the Vulva: A Case Report

Primary cutaneous neuroendocrine tumors (CNET) are extremely rare. Only a few cases have been reported so far. CNET have an indolent clinical course and usually present as a single flesh-colored nodule with a predilection for the scalp and trunk in elderly patients. While primary CNET have characteristic histological and immunohistochemical features akin to other low-grade neuroendocrine tumors elsewhere in the body, diagnosing these tumors on skin biopsies can be challenging as they are particularly mistaken for other, more commonly diagnosed, entities. In the current report we present a unique case of primary CNET of the vulva. The clinical presentation will be discussed as well as the histopathologic and immunohistochemical features and most importantly the possible pitfalls in microscopic examination.