Q: Carney complex includes which of the following components?
a.Large cell calcifying Sertoli cell tumors
b.Cardiac myxomas
c.Primary pigmented adrenocortical disease
d.Autosomal dominant transmission
e.All of the above
Explanation: This autosomal dominant disorder is mapped to chromosome 2p16 and may be due to a gain-of-function mutation. The adrenal disorder is a pigmented nodular adrenocortical disease with associated features such as lentigines, blue nevi, cardiac and skin myxomas, and sexual precocity in boys with large cell calcifying Sertoli tumors and melanotic schwannomas. (See Chapter 571 in Nelson
Textbook of Pediatrics, 17th ed.)
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