Pigmented Adrenal Hyperplasia with Myelolipomatous Changes and Bilateral Testicular Enlargement in an Untreated Man with 21-Hydroxylase Deficiency

Objective: To report the occurrence of a giant left adrenal tumor and bilateral testicular masses (adenomatous hyperplasia of Leydig cells) in a young man with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Methods: The clinical, radiologic, endocrinologic, and pathologic features of this case are correlated with the findings in the literature. Results: The interesting elements in this case are the rare pathologic features of the left adrenal lesion (pigmented adrenal hyperplasia with myelolipomatous changes) and the association with the infrequent testicular adrenal rest tumors. The absence of enlargement of the right adrenal gland was unexplained. Conclusion: The presence of these two rare complications seemed to be associated with poor adherence to medical treatment recommendations for congenital adrenal hyperplasia. (Endocr Pract. 2000;6:260-263)