Malignant Insulinoma in Childhood

Abstract

Pancreatic tumors constitute a rare surgical problem in infancy and childhood. Insulinomas are rare in all age groups with an estimated incidence of one per 250,000 person-years and even rarer in childhood. We report a 10 year-old girl with malignant insulinoma. The presenting symptom was hypoglycemic attacks. Laboratory investigation demonstrated that the hypoglycemia was due to hyperinsulinism. MRI of the abdomen revealed a mass at the tail of the pancreas. Distal pancreatectomy with splenectomy was performed. Histological examination showed malignant insulinoma with peripancreatic lymph node metastases. One month later abdominal MRI revealed the existence of multiple small metastatic foci in the liver, which were confirmed by In111 octreoscan. Treatment with octreotide was started and the disease is stable after 12 months of therapy.

Metastatic Insulinoma in a 16-Year-Old Adolescent Male With Men-1: A Case Report and Review of the Literature

“…Only 11 cases of malignant insulinomas have been reported in the pediatric age group (3)(4)(5)(6)(7)(8)(9)(10)(11). The youngest patient reported with malignant insulinoma was 1.5 years old (4,5), however, most cases reported are children 8 years or older (3,(5)(6)(7)(8)(9)(10)(11). In the pediatric age group, patients most commonly present with symptoms of severe hypoglycemia, including loss of consciousness, confusion, or seizures (5).…”

“…Multiple endocrine neoplasia type 1 (MEN-1) is an autosomal dominant condition associated with various combinations of 3 groups of endocrine tumors: (1) parathyroid adenomas (most common of all endocrine tumors in MEN-1), (2) pituitary tumors, including prolactinoma (most common of all pituitary tumors in MEN-1), acromegaly, Cushing disease, and (3) gastro-entero-pancreatic tumors such as gastrinoma, insulinoma, and glucagonoma, and nonendocrine tumors, including carcinoid, facial angiofibroma, lipoma, meningioma, ependymoma, and leiomyoma (1)(2)(3). Clinical diagnostic criteria for MEN-1 include presence of at least 2 of the 3 groups of endocrine tumors as listed above (1).…”

“…Insulinomas are rare endocrine tumors in the pediatric age group and may be associated with MEN-1 in 10% of cases (2). Malignant insulinomas only constitute 5 to 10% of all insulinomas and are exceedingly rare in children (3)(4)(5)(6)(7)(8)(9)(10)(11).…”

Management Challenges in a Child with Hyperinsulinemic Hypoglycemia

“…Out of these, only three cases have been reported as malignant (12,13,14). Our patient had the tumor enucleated and underwent subtotal pancreatectomy five months later because of persistent hypoglycemia and inadequate response to octreotide and diazoxide.…”