Bidirectional differentiation in a large cell pleomorphic primary endocrine carcinoma of the skin (a variant of malignant Merkel cell tumour).

A primary endocrine carcinoma of the skin is described in which the tumour cells showed the unusual features of large size and marked nuclear pleomorphism. Ultrastructural examination revealed combined features of squamous and endocrine differentiation in the same cells, a phenotype which has been previously anticipated but only recently described in endocrine carcinomas of the skin. In addition, immunocytochemical staining showed the co-expression of cytokeratin and neurofilament. The globular masses of cytokeratin corresponded to paranuclear whorls of intermediate filaments and there was also strong and diffuse staining for neuron specific enolase. Many tumour cells stained for calcitonin, VIP, ACTH, and S100 protein. Large tumour cells with markedly pleomorphic nuclei and co-expression of cytokeratin and neurofilament add to the expanding morphological spectrum of primary endocrine carcinomas of the skin.