This case series highlights the diverse clinical manifestations of Carney complex, including cardiac myxomas and potentially fatal metastatic melanotic schwannomas.
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Raises caution for fatal metastatic schwannoma in Carney complex; leaves open optimal surveillance strategies.
The authors report three patients with features of the Carney complex: cardiac myxomas (n = 2), multicentric (n = 1) or recurrent (n = 1); pigmented skin lesions (n = 2); cutaneous myxomas (n = 2); melanotic schwannoma (n = 2); and sonographic evidence of multicentric, large cell, calcifying Sertoli cell tumors of the testes (n = 1). The initial component of the complex was cutaneous myxoma (n = 1), cardiac myxoma (n = 1), or psammomatous melanotic schwannoma (n = 1). Two patients died of metastatic schwannoma.
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Radin et al. (1995) studied this question.
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