ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume: 15 Issue: 2 Year: 2023

Abstracting & Indexing
Turkish Society for Pediatric Endocrinology and Diabetes
Juvenile Granulosa Cell Tumor Mimicking HAIR-AN in a 4-Year-Old: A Case Report [J Clin Res Pediatr Endocrinol]
J Clin Res Pediatr Endocrinol. Ahead of Print: JCRPE-96168 | DOI: 10.4274/jcrpe.galenos.2022.2022-4-17

Juvenile Granulosa Cell Tumor Mimicking HAIR-AN in a 4-Year-Old: A Case Report

Rachel Choe Kim1, Iliya Goldberg2, Trevor Van Brunt1, Hamama Tul-Bushra3, Rebecca Batiste3, Andrew H. Lane4, Helen Hsieh5
1Renaissance School of Medicine, Stony Brook University, NY, United States
2Department of Surgery, Stony Brook Medicine, NY, United States
3Department of Pathology, Stony Brook Medicine, NY, United States
4Department of Pediatrics, Stony Brook Children’s Hospital, NY, United States
5Division of Pediatric Surgery, Department of Surgery, NY, United States

Predominantly androgen secreting juvenile granulosa cell tumors (JGCT) are uncommon and few reports exist in the literature. We present a case of a JGCT which presented with signs of prepubertal hyperandrogenism and insulin resistance to highlight the possible interaction between hyperandrogenemia and hyperinsulinism. We conducted chart review of a rare androgen secreting JGCT accompanied by hyperinsulinemia in a prepubertal patient. A 4-year-old girl presented with acanthosis nigricans and hyperinsulinism mimicking the Hyperandrogenism Insulin Resistance and Acanthosis Nigricans (HAIR-AN) syndrome at an age much younger than is typical for this diagnosis. Laboratory studies revealed elevated insulin, inhibin A and B, and total testosterone. All laboratory results normalized after unilateral salpingo-oophorectomy. The final diagnosis was Stage IA JGCT. This case highlights the importance of including ovarian tumors in the differential diagnosis when considering causes of virilization and insulin resistance. Our case illustrates the potential relationship between excess testosterone secretion and hyperinsulinemia and strengthens evidence that hyperandrogenemia may promote hyperinsulinism in ovarian disease.

Keywords: Juvenile granulosa cell tumor, hyperandrogenism, insulin resistance

Corresponding Author: Helen Hsieh, United States of America
Manuscript Language: English
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