Feminizing adrenocortical adenoma in a girl from a resource-limited setting: a case report

Abstract

Background An adrenocortical tumor is a rare tumor in pediatrics, which can be functional or nonfunctional. Functional tumors present with virilization, feminization, or hypercortisolism. Feminizing adrenal tumors, though rare in pediatrics, need to be excluded in any child presenting with features of feminization. Case presentation We report a case of a 4-year-old Sudanese girl who presented with gradually progressive bilateral breast enlargement and accelerated growth since the age of 6 months. The family had sought medical advice several times in numerous health facilities without much gain. Investigations showed pubertal luteinizing hormone levels, high estradiol E2, and dehydroepiandrosterone sulfate, with normal early morning cortisol level. Abdominal ultrasound revealed a right-sided hypoechoic suprarenal mass. Abdominal computed tomography scan showed a right adrenal mass. The diagnosis of feminizing adrenal neoplasm was confirmed and right adrenalectomy was done. Histopathological examination of the resected adrenal gland showed adrenocortical adenoma. The patient was started on gonadotrophin-releasing hormone agonist for secondary central precocious puberty. Conclusion Adrenocortical tumors, though rare in pediatrics, are a documented cause of precocious puberty; biochemical and imaging screening protocol should be adopted for patients with precocious puberty, even in a resource-limited setting, for early detection and treatment.

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

“…A complication caused by the increased secretion of steroids due to an adrenocorticotropic tumor leads to a defect in the sex of the fetus resulting in ambiguous genitalia 24 . A disruption in the ovulation process, hypomenorrhea, irregular periods, or menopause are all consequences of the increased cortisol production, which prevents the pituitary gland from releasing gonadotropin 25 , and Peripheral precocious puberty is more likely when there is an excess of estrogen 26 . Dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, 11-deoxycortisol, androstenedione (A4), testosterone, and cortisol are the adrenal hormones used to measure basal secretion to diagnose adrenocortical tumors 3 , 7 .…”