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Testicular tumor of adrenogenital syndrome is a rare and benign anomaly usually presenting as bilateral testicular masses. It is the most important cause of infertility in adult male congenital adrenal hyperplasia. Distinction between testicular tumors of adrenogenital syndrome and Leydig cell tumors can be problematic; it is based on clinical, histopathologic, immunohistochemical and endocrine features. Biopsy is advised in cases of longstanding tumors in infertile patients and when surgery is indicated. Fertility preservation is a key management goal in testicular tumor of adrenogenital syndrome. In stages 2 and 3, intensified glucocorticoid treatment is recommended as a first step treatment. Sparing surgical approach is preferred for tumors of stage 4 and steroid unresponsive masses. Magnetic resonance imaging is recommended before surgery. The only indication of surgery in stage 5 is testicular pain. Copyright © 2017 Elsevier Masson SAS. All rights reserved.


Sahbi Naouar, Salem Braiek, Rafik El Kamel. Testicular tumors of adrenogenital syndrome: From physiopathology to therapy. Presse medicale (Paris, France : 1983). 2017 Jun;46(6 Pt 1):572-578

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PMID: 28549629

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