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Report of a Kindred

Ann Danoff, MD; Susan Jormark, MD; Daniel Lorber, MD; Norman Fleischer, MD

Arch Intern Med. 1987;147(3):443-448.

Abstract
• In a family encompassing three generations, six of 11 evaluated members have two or three elements of a triad comprising adrenocortical micronodular dysplasia, mucocutaneous lentigines, and cardiac myxomas. Evaluation of the adrenals in affected members revealed characteristic pathologic lesions of micronodular adrenal hyperplasia and corticotropin-independent steroidogenesis that correlated with age, suggesting a progressive lesion that begins in early childhood. Since all subjects with micronodular hyperplasia and/or cardiac myxomas also had mucocutaneous lentigines, the skin lesions were markers for affected subjects. This family is one of the larger reported with this syndrome. Of special note was the finding of rare visceral tumors in affected family members, including melanocytic schwannomas and a fibrolamellar hepatoma, signaling another feature of the syndrome. Since 60% of this family encompassing three contiguous generations were affected, the syndrome appears to be inherited as an autosomal or X-linked dominant gene.

(Arch Intern Med 1987;147:443-448)

Author Affiliations
From the Division of Endocrinology, Department of Medicine, Albert Einstein College of Medicine, Bronx, NY (Drs Danoff, Lorber, and Fleischer), and the Department of Pathology, Booth Memorial Medical Center, Queens, NY (Dr Jormark).

Footnotes
Accepted for publication May 2, 1986.

Reprint requests to Albert Einstein College of Medicine, 1300 Morris Park Ave, Bronx, NY 10461 (Dr Fleischer).

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