Androgen-Secreting Tumor with Oncocytic Features and Myelolipoma Arising from Ectopic Adrenal Cortical Tissue Located in
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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 Ghosh1 • Partha Pratim Chakraborty1
•
Anirban Sinha1 • Animesh Maiti1
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 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 17ahydroxyprogesterone (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 Adrenal rest tumor Myelolipoma PCOS
Introduction Polycystic ovary syndrome (PCOS) is the underlying cause of hirsutism in majority (70–80%) of women of reproductive age group, followed by idiopathic ones and nonclassic congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase (CYP21A2) deficiency [1]. Androgen-secreting 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
& Partha Pratim Chakraborty [email protected] Sayan Ghosh [email protected] Anirban Sinha [email protected] Animesh Maiti [email protected] 1
Department of Endocrinology and Metabolism, Medical College, Kolkata, West Bengal, India
ridge structures and detected in more than 30% of unselected autopsy cases [2]. Ectopic adrenal tissue, however, has been localized in celiac plexus, kidney, testis, epididymis, 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 accompanying 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 orig
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