Malignant insulinoma in a child

Abstract

Insulinomas are rare tumors with an estimated incidence of one per 250,000 person-years. Most insulinomas are benign with less than 10% demonstrating malignant behavior, the vast majority of which occur in adults. A systemic review of the literature revealed only nine cases of malignant insulinomas occurring in children. Herein, we present a case of metastatic malignant insulinoma in a 12-year-old child. The occurrence of this diagnosis in a child, its unusual pattern of metastases and the challenging management of severe hypoglycemia make this case worth reporting.

The surgical management of insulinomas in children

“…This is consistent with the literature in which enucleation was performed in 34–54% of cases and was the procedure of choice in the absence of possible injury to the main pancreatic duct or when malignancy was not suspected [1,4,25]. None of the patients in the current series had a malignant insulinoma which is rare (<10% of insulinomas) but may require a more extensive resection and even liver transplantation secondary to lymph node involvement, invasion into adjacent structures or liver involvement [26]. The spleen can be preserved in patients undergoing a distal pancreatectomy as advised by reports suggesting patients undergoing spleen-preserving procedures have less postoperative complications including infection [4,27].…”

Pancreatic surgery for tumors in children and adolescents

“…The most common histology was insulinoma, associated with MEN1 in one case. One patient was diagnosed with a malignant insulinoma, seen in 4-14 % of cases [42], with only ten cases described in children [43]. Lymph node involvement, local invasion or metastases are present at the initial diagnosis in the vast majority of cases and portend aggressive behavior [44].…”

Sporadic Insulinoma as a Rare Cause of Recurrent Hypoglycemia in Children

“…As our patient, neuropsychiatric manifestations are sometimes confusing and can cause delay in diagnosis. Most insulinomas are benign and less than 10% show malignant behaviour and majority of them occur in adults [3, 6]. Early diagnosis of insulinoma is obviously important for ensuring proper treatment and preventing serious adverse neurological consequences and permanent damage [7].…”