Background: In children, congenital hyperinsulinism is the most common cause for endogenous hyperinsulinaemic hypoglycaemia (HH). Beyond infancy other diagnoses may be considered, such as insulinoma, an insulin-secreting neuroendocrine tumour (NET) arising mostly from the pancreas. The latter is a rare cause of HH in children. The estimated incidence of insulinoma is 1:250000 person-years of all age groups with a median age at diagnosis of 47 years. Herein, we report an adolescent girl with malignant insulinoma, which is extremely rare in children.
Case presentation: A previously healthy 15-year-old girl presented at the emergency department with a history of vertigo, fatigue, recurrent feeling of faintness, intermittent inadequate behaviour and amnesia for the event for the last 6 months. Physical and neurological examinations were normal. No evidence for organic brain disease was present and symptoms were interpreted to be functional. The patient was hospitalised for further evaluation. At the first night, the patient fell unconscious and hypoglycaemia was diagnosed. She rapidly recovered under intravenous glucose supply. Further evaluation revealed increased serum insulin and C-peptide levels. An MRI scan of the abdomen demonstrated a solid mass in the pancreatic tail as well as two liver lesions, suspicious for metastases. Surgical resection of the tumour and the pancreatic tail was performed. The intrahepatic lesions were identified by ultrasound and resected as well. Histological examination confirmed the diagnosis of a NET with liver metastases. MEN-Typ1 was excluded. A follow-up 8 months later revealed no evidence of hypoglycaemia, but a new suspicious liver lesion on MRI scan.
Conclusion: Unclear behavioural changes should prompt blood glucose measurement. Malignant insulinoma is an extremely rare finding in children and its management in case of progression or relapse remains mainly experimental.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology