Pseudoprecocity Resulting from Tumors of the Testes

Published on 25/03/2015 by admin

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Last modified 25/03/2015

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Chapter 578 Pseudoprecocity Resulting from Tumors of the Testes

Omar Ali, Patricia A. Donohoue

Leydig cell tumors of the testes are rare causes of precocious pseudopuberty and cause asymmetric enlargement of the testes. Leydig cells are sparse before puberty and tumors derived from them are more common in the adult, but rare cases do occur in children and the youngest reported case was in a 1 yr old boy. These tumors are usually unilateral and benign; while up to 10% of adult tumors may be malignant, metastasizing malignant tumors have not been reported in children. Some tumors may be due to somatic activating mutations of the luteinizing hormone (LH) receptor.

The clinical manifestations are those of puberty in the male; onset usually occurs usually from 5 to 9 years of age. Gynecomastia has been described. The tumor of the testis can usually be readily felt; the contralateral unaffected testis is normal in size for the age of the patient.

Plasma levels of testosterone are markedly elevated, and follicle stimulating hormone and LH levels are suppressed. Ultrasonography may aid in the detection of small nonpalpable tumors. Fine-needle aspiration biopsy may help define the diagnosis.

Treatment consists of surgical removal of the affected testis. These tumors are generally resistant to chemotherapy. Progression of virilization ceases after removal of the tumor, and partial reversal of the signs of precocity may occur.

Testicular adrenal rests

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