남성화 증상으로 내원한 16개월 남아에서진단된 부신겉질종양 1례
A Case of Adrenocortical Tumor in a16-Month-Old Boy Presenting with Virilization
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A 16-month-old boy had signs of virilization such as penile enlargement and pubic hair since 13 months of age. Hormone evaluation indicated increased levels of 17-OH-progesterone, dehydroepiandrosterone- sulfate (DHEA-S) and testosterone. Initially he was diagnosed as simple virilizing type of congenital adrenal hyperplasia and hydrocortisone was given to him. After 2 months, his penile enlargement continued and we did not find gene mutation for 21-hydroxylase deficiency. Adrenal magnetic resonance imaging revealed a 3.5×2.7×4.4 cm size of adrenal mass consistent with an adrenocortical tumor. The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormone levels. Childhood adrenocortical tumors are rare and are not well characterized. Surgery is the definitive treatment for adrenocortical tumors and prognosis is often poor. We report a case of adrenocortical tumor in a 16-month-old boy presenting with virilization with review of literature.
Abstract
A 16-month-old boy had signs of virilization such as penile enlargement and pubic hair since 13 months of age. Hormone evaluation indicated increased levels of 17-OH-progesterone, dehydroepiandrosterone- sulfate (DHEA-S) and testosterone. Initially he was diagnosed as simple virilizing type of congenital adrenal hyperplasia and hydrocortisone was given to him. After 2 months, his penile enlargement continued and we did not find gene mutation for 21-hydroxylase deficiency. Adrenal magnetic resonance imaging revealed a 3.5×2.7×4.4 cm size of adrenal mass consistent with an adrenocortical tumor. The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormone levels. Childhood adrenocortical tumors are rare and are not well characterized. Surgery is the definitive treatment for adrenocortical tumors and prognosis is often poor. We report a case of adrenocortical tumor in a 16-month-old boy presenting with virilization with review of literature.
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