Uranaffin reaction in Merkel cells of fetal rat skin.
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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.
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Neuroendocrine carcinomas of the skin have recently been recognized, and clinicopathologic information on these tumors is accumulating rapidly. We studied 13 such lesions by light and electron microscopy, and eight were subjected to immunohistochemical analysis. The ages of the patients (five women and eight men) ranged from 24 to 84 years. Nine patients had neoplasms occurring in sun-exposed areas; one patient had metachronous symmetric lesions on the arms, and another had antecedent hypohidrotic ectodermal dysplasia. Eight tumors metastasized, and six spread to regional or distant lymph nodes. Three patients died with visceral metastases to the liver, bones, and brain. All patients had their primary tumor confined to the corium and subcutaneous tissue without involvement of overlying epidermis. Tumor cells were round and uniform in size, with delicate nuclear chromatin and a high mitotic rate. An organoid growth pattern was seen in all tumors, with focal trabeculation and rare rosette formation. Ultrastructurally, peripheral cytoplasmic dense-core granules were evident, and perinuclear filament whorls could be seen in all 13 tumors. The latter feature was stable, even in specimens taken from paraffin blocks for electron microscopy. Immunoperoxidase studies failed to reveal serotonin, calcitonin, or adrenocorticotrophin within tumor cells.
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