Androgen-Secreting Tumor with Oncocytic Features and Myelolipoma Arising from Ectopic Adrenal Cortical Tissue Located in Broad Ligament of the Uterus: Too Rare a Disease!

Hirsutism or excess growth of androgen-dependent hairs in a male pattern distribution associated with menstrual irregularities is frequently encountered in clinical practice. Polycystic ovary syndrome and non-classic congenital adrenal hyperplasia are the common underlying causes; androgen-secreting ovarian or adrenal neoplasms also need to be ruled out in appropriate settings, particularly in presence of virilization and grossly elevated circulatory androgens. Elevated 17α-hydroxyprogesterone (17-OH-P), though highly suggestive of congenital adrenal hyperplasia, is not specific for the disease, and grossly elevated values are occasionally encountered in androgen-producing tumors. Ectopic adrenocortical tissue or adrenal rest at times may undergo tumor formation and become hormonally active. In this article, androgen-secreting tumor of adrenal rest located in broad ligament of the uterus is reported as the underlying cause of severe hyperandrogenism in a young lady who was initially diagnosed with simple virilizing form of congenital adrenal hyperplasia due to 21 hydroxylase deficiency following elevated circulatory 17-OH-P levels.