Pancreatic neuroendocrine tumors (PNETs) include both functioning and non-functioning neoplasms, with the latter constituting the largest subgroup. Non-functioning PNETs pose morbidity risks due to potential tissue invasion and metastasis. Functioning PNETs, comprising 1% to 2% of pancreatic tumors, induce hormonal symptoms through excessive hormone secretion. Advanced imaging techniques have increased their detection frequency, with insulinoma, the most prevalent functioning PNET, constituting 35-40% of cases. Insulinoma presents with hyperinsulinemia and subsequent hypoglycemia. Glucagonoma, the fourth most common functioning PNET, accounting for 5% of cases, results in hyperglycemia due to excessive glucagon secretion. Concurrent existence of two PNETs is exceedingly rare, prompting this report to detail cases involving simultaneous insulinomas and glucagonomas.

We present a case of a young male patient who was presented with hypoglycemic symptoms. A 72-hour fast revealed insulinoma. Imaging (ultrasound, CT, MRI, octreotide, gallium scintigraphy) detected a 4 cm cystic lesion in the pancreatic tail. Endoscopic ultrasound confirmed a pancreatic neuroendocrine neoplasm (PNEN). Distal pancreatectomy was performed based on cytological results. Moreover, we conducted a literature review on the PubMed database and Cochrane library, aimed to identify documented instances of concurrent pancreatic insulinoma and glucagonoma.

Seven cases of simultaneous pancreatic insulinoma and glucagonoma were identified within the 1992-2021 timeframe.

Concomitant insulinoma and glucagonoma pose rare and intricate challenges. A multidisciplinary approach is imperative for accurate diagnosis, prognosis, and therapeutic management.