Juvenile Granulosa Cell Tumor Mimicking HAIR-AN in a 4-year-old: A Case Report
Rachel Choe Kim1, Ilya Goldberg2, Trevor Van Brunt1, Hamama Tul-Bushra3, Rebecca Batiste3, Andrew H. Lane4, Helen Hsieh51Stony Brook University, Renaissance Faculty of Medicine, New York, USA2Stony Brook University, Renaissance Faculty of Medicine, Department of Surgery, New York, USA
3Stony Brook University, Renaissance Faculty of Medicine, Department of Pathology, New York, USA
4Stony Brook Children’s Hospital, Clinic of Pediatrics, New York, USA
5Stony Brook University, Renaissance Faculty of Medicine, Department of Surgery, Division of Pediatric Surgery, New York, USA
Predominantly androgen secreting juvenile granulosa cell tumors (JGCT) are uncommon and few reports have been published. We present a case of a JGCT that presented with signs of prepubertal hyperandrogenism and insulin resistance to highlight the possible interaction between hyperandrogenemia and hyperinsulinism. A 4-year-old girl presented with acanthosis nigricans and hyperinsulinism, mimicking the hyperandrogenism, insulin resistance and acanthosis nigricans 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 1A JGCT. This case highlights the importance of including ovarian tumors in the differential diagnosis when considering causes of virilization and insulin resistance. This case also suggests a 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 resistanceCorresponding Author: Helen Hsieh, United States of America
Manuscript Language: English
