Abstract

The adrenal enzyme deficiency leads pseudopuberty in later diagnosis or in the absence of treatment. The existence of testicular enlargement in boys can be related to adrenal inclusion. We report two observations about this pathology: A boy of 10 years old with 11β hydroxylase and another patients of 6 years old with 21 hydroxylase deficiency. The reason of consultation was the development of the penis and pubic hair with a testicular enlargement. Hormonal balance was in favor of early pseudo puberty. Testicular ultrasonography objectified increased volume and testicular hypoechoic nodules. Tumor markers (βHCG, ACE) were negative. Replacement and suppressive therapy by glucocorticoids is undertaken. The evolution was marked by regression of secondary sexual characteristics, reduced testicular size, increased its echogenecity and loss of nodules. During a reevaluation ten years later, a large heterogeneous testicular nodule is found in one patient. Tumor markers were elevated. Orchidopexy is decided. Histological study was in favor of bilateral Leydig tumor. The intratesticular adrenal inclusion is rare. It is the result of a cortical defect with a delay in diagnosis. Reduction of testicular volume after glucocorticoid therapy is a good predictor of disease control. However, a long term of follow up is necessary because a possibility of tumor degeneration. Keywords Congenital adrenal hyperplasia, Adrenal inclusion Intratesticular, Ultrasound, Tumor leydig, Glucocorticoids

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