Background: Hypoglycemia in the neonate occurs in approximately one to five per 1000 live births. Common causes may be due to sepsis, IUGR or LGA. Rarer causes are congenital hyperinsulinism, panhypopituitarism, GHD, cortisol deficiency, insulinoma, insulin-receptor stimulating antibodies, BWS, and congenital disorders of glycosylation. We present a case of hypoglycemia in a neonate secondary to intraductal papillary mucinous neoplasm of the pancreas (IPMN). IPMN was first reported by Ohashi et al. (1982). The majority of IMPNs occur in the sixth to seventh decade of life with rare reports in children.
Objective and hypotheses: Patient presented to the ER following a seizure with bg of 55. UA revealed many WBCs resulting in US evaluation of kidneys with abdominal mass identified. Our hypothesis was that the seizure may have been due an insulin secreting mass resulting in hypoglycemia. To further confirm this, we evaluated the patient biochemically and obtained more accurate imaging.
Method: A critical sample was obtained when bg <40 followed by administration of glucagon. A dedicated abdominal US followed by an MRI of the abdomen was obtained.
Results: Insulin level at the time of hypoglycemia was with bg rise to within half an hour of glucagon, consistent with hyperinsulinism. Abdominal US identified mass as a mixed solid/cystic structure, medial to the right kidney. MRI further delineated a retroperitoneal, multiloculated cystic mass, located in the RUQ of the abdomen appearing to arise from the pancreas. Dimensions were 2.2 ×3.7 ×3.9 cm. The mass was resected with pathological analysis revealing multiple cystic lesions described as multifocal intraductal papillary mucinous neoplasm (IPMNs) with high-grade dysplasia. Insulin immunostain of the tumor was positive.
Conclusion: The final diagnosis was an isolated IPMN with high-grade dysplasia. This is the first known reported case of this diagnosis in a neonate.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology