EPITHELIOID BLUE NEVUS AND PSAMMOMATOUS MELANOTIC SCHWANNOMA - THE UNUSUAL PIGMENTED SKIN TUMORS OF THE CARNEY COMPLEX

Carney complex, a familial multitumoral syndrome, comprises spotty ski n pigmentation (lentigines and blue nevi), myxomas (heart, skin, and b reast), endocrine tumors (adrenal cortex, pituitary, testis, and thyro id), and schwannomas. The skin pigmentary abnormality included two unu sual conditions, epithelioid blue nevus and psammomatous melanotic sch wannoma. The former tumor occurred on the extremities and trunk, less frequently on the head and neck; was multiple; and did not recur or me tastasize. Clinically, it was pigmented, domed, and small (<1 cm). Mic roscopically, it displayed two types of melanocytes-one intensely pigm ented, globular, and fusiform and the other lightly pigmented, polygon al, and spindle. Nuclei of the latter cells were vesicular, with pale chromatin and single prominent nucleolus. None recurred or metastasize d. The psammomatous melanotic schwannoma occurred in posterior spinal nerve roots, upper alimentary tract, bone, and skin. Microscopically, it was circumscribed but incompletely encapsulated and contained spind le and epithelioid cells, melanin, psammoma bodies, and fat. The spind le cells were arranged in interlacing fascicles, with whorling and occ asional nuclear palisading. Twenty-one patients (68%) were alive witho ut evidence of the neoplasm; two of these each had two local recurrenc es. Seven patients died, three (10%) as a result of metastasis. Copyri ght (C) 1998 by W.B. Saunders Company.