CASE REPORT 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! Sayan Ghosh 1 • Partha Pratim Chakraborty 1 • Anirban Sinha 1 • Animesh Maiti 1 Received: 22 July 2020 / Revised: 21 August 2020 / Accepted: 25 August 2020 Ó Association of Gynecologic Oncologists of India 2020 Abstract Hirsutism or excess growth of androgen-dependent hairs in a male pattern distribution associated with menstrual irregu- larities 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 17a- 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. Keywords Congenital adrenal hyperplasia Á Adrenal rest tumor Á Myelolipoma Á PCOS Introduction Polycystic ovary syndrome (PCOS) is the underlying cause of hirsutism in majority (70–80%) of women of repro- ductive age group, followed by idiopathic ones and non- classic congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase (CYP21A2) deficiency [1]. Androgen-se- creting tumors of ovarian or adrenal origin are rare causes of hyperandrogenism. Adrenal rest, also termed as ectopic adrenal gland, is commonly found along the urogenital ridge structures and detected in more than 30% of unse- lected autopsy cases [2]. Ectopic adrenal tissue, however, has been localized in celiac plexus, kidney, testis, epi- didymis, broad ligament of the uterus, canal of Nuck, hernial and hydrocele sacs, the mesoappendix, liver, lung, intradural space and brain. Adrenocortical tissue is the sole component of these ectopic adrenal rests without accom- panying medullary tissue [3, 4]. Few and far between, these ectopic rests undergo marked hyperplasia and develop into ectopic adrenocortical adenomas and carcinomas which may be hormonally active or silent. Virilizing adrenal adenomas themselves is very rare, even rarer are the androgen-secreting adenomas of ectopic adrenal origin. Case Report A 22-year-old lady, married for 10 years, consulted us for primary infertility, menstrual irregularity in the form of oligo/amenorrhea and male pattern hair growth. She had experienced menarche at 12 years of age. She used to have cyclic predictable menses till 18 years of age when she had & Partha Pratim Chakraborty docparthapc@yahoo.co.in Sayan Ghosh sayan1810@gmail.com Anirban Sinha anirban.doc@gmail.com Animesh Maiti animeshmaiti73@gmail.com 1 Department of Endocrinology and Metabolism, Medical College, Kolkata, West Bengal, India 123 Indian Journal of Gynecologic Oncology (2020)18:103 https://doi.org/10.1007/s40944-020-00450-3