MULTIPLE HAMARTOMA SYNDROME (COWDENS DISEASE) ASSOCIATED WITH RENAL-CELL CARCINOMA AND PRIMARY NEUROENDOCRINE CARCINOMA OF THE SKIN (MERKEL CELL-CARCINOMA)

A case of multiple hamartoma syndrome (Cowden's disease) associated with renal cell adenocarcinoma and primary neuroendocrine carcinoma of the skin is described. Neither of these neoplasms has been documented previously in association with this genodermatosis. A search for epidermal growth factor receptor (c-erb-B protooncogene) gene abnormalities in the kidney, liver, and thyroid, as well as in tissue of the primary neuroendocrine carcinoma, was negative. Serum obtained from the patient before his death contained elevated levels of both chromogranin A (2641 ng/mL; normal level, < 20 ng/mL) and calcitonin (517 pg/mL; normal level, < 200 pg/mL), suggesting that the patient's principal tumor was neuroendocrine in origin.