Adrenocortical Micronodular Dysplasia, Cardiac Myxomas, Lentigines, and Spindle Cell Tumors: Report of a Kindred

Ann Danoff, Susan Jormark, Daniel Lorber, Norman Fleischer

Research output: Contribution to journalArticle

45 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)443-448
Number of pages6
JournalArchives of internal medicine
Volume147
Issue number3
DOIs
StatePublished - Mar 1987

ASJC Scopus subject areas

  • Internal Medicine

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