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The Complex of Myxomas, Spotty Pigmentation, and Endocrine Overactivity

J. Aidan Carney, MD, PhD, FRCPI
Arch Intern Med. 1987;147(3):418-419. doi:10.1001/archinte.1987.00370030022003.
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The syndrome discussed by Danoff and associates1 in this issue of the Archives is a newly recognized condition1 that was "synthesized" from some personal observations, along with bits and pieces and good-sized chunks of information gleaned from the literature. The complex includes myxomas (cardiac, cutaneous, and mammary), spotty mucocutaneous pigmentation, and endocrine overactivity (Cushing's syndrome, sexual precocity, and acromegaly or gigantism). Most of the constituents of the complex are probably now known, but there are still some candidate components—such as uterine myxoma, acoustic neuroma, taillike appendage, thyroid neoplasms, and fibrolamellar hepatoma—that have occurred singly in affected patients (tumors in the complex are usually multicentric).

Among the 40 patients included in our article on the complex2 were two genetically unrelated patients who, in addition to other components of the syndrome, had a peculiar pigmented neural tumor with features of schwannoma. Although the neoplasm was single in both, we suggested that it


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