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Persistent Hyperinsulinemic Hypoglycemia with Pancreatic Teratoma in Infancy: A Case Report

Unusual clinical course, Challenging differential diagnosis

Ayse Pinar Cemeroglu, Faik Sarialioglu, Fatma Burcu Belen-Apak, Yunus Kasim Terzi

Turkey Deparment of Pediatric Endocrinology, Faculty of Medicine, Baskent University, Ankara, Turkey

Am J Case Rep 2020; 21:e925273

DOI: 10.12659/AJCR.925273

Available online: 2020-06-25

Published: 2020-08-12


#925273

BACKGROUND: Pediatric intraabdominal pancreatic teratomas have been rarely reported. This is the first case of severe hyperinsulinemic hypoglycemia in a 6-month-old infant secondary to an intraabdominal teratoma. The hypoglycemia resolved after surgical removal.
CASE REPORT: A 6-month-old infant was seen in a pediatric emergency department with complaints of lethargy and abnormal eye movements. She was diagnosed with hyperinsulinemic hypoglycemia and started on diazoxide. A CT and MRI of the abdomen revealed a 165×77×72 mm cyst with a 51×45×30 mm solid structure connecting to the wall of the cyst by a stalk, raising suspicion of a fetus in fetu. The mass had no connection to her pancreas. Following total excision of the intraabdominal mass, her hypoglycemia resolved. Histopathological examination showed immature fetal pancreatic tissue consistent with a mature teratoma. Whole exon sequencing of the infant’s peripheral blood showed a negative mutation of ABCC8 and presence of heterozygous variations of HNF1ß and IRS1 genes.
CONCLUSIONS: This is the first case report of an infant with severe hyperinsulinemic hypoglycemia secondary to a pancreatic teratoma. The heterozygous variations of HNF1ß and IRS1 genes likely played a role in the embryogenesis, causing a pancreatic teratoma and hyperinsulinemic hypoglycemia.

Keywords: Hyperinsulinism, Hypoglycemia, Infant, Newborn, Teratoma



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