Epidermotropic primary neuroendocrine (Merkel cell) carcinoma of the skin with Pautrier-like microabscesses: Report of three cases and review of the literature

Abstract

Three cases of primary cutaneous neuroendocrine carcinoma involving the face are reported. In the first patient the tumor developed in the scar of a previously excised basal cell carcinoma of the nose, recurred locally, and metastasized to a regional lymph node 2 1/2 years after initial treatment. In the second and third patients the tumors were located on the forehead and were removed with a wide excision. In the second patient the tumor metastasized to regional lymph nodes 1 month after treatment. Light microscopic examination in all patients showed an undifferentiated, small cell, dermal tumor with prominent epidermotropism, forming Pautrier-like microabscesses. Immunohistochemical staining for neuron-specific enolase was positive, and ultrastructural examination revealed numerous cytoplasmic neurosecretory granules and paranuclear aggregates of intermediate-sized filaments. The literature concerning epidermal involvement by primary neuroendocrine carcinoma of the skin is reviewed; the relation with basal cell carcinoma and treatment are discussed.