ABSTRACT
Neuroendocrine tumors (NETs) are neoplasms arising from neuroendocrine cells. There are a minimum of 17 different types of neuroendocrine cells recognized and found distributed throughout the pancreas and gastrointestinal tract. Tumors can arise from these precursor cells and overproduce one dominant hormone and other in fewer quantities. Clinical syndromes are caused by the excess hormones produced. Tumors are referred to by the name of the excess hormone whose effects dominate the clinical picture appended with “-oma.” In the pancreas, they are located in the islets of Langerhans and produce five biologically active peptides insulin, glucagon, somatostatin, pancreatic polypeptide, and ghrelin. Tumors of any of these cells that produce excess hormone are called as pancreatic NETs (PNETs). These are indolent tumors with heterogeneous presentation and pathology. They may present as asymptomatic, incidentally discovered masses or debilitating hormonal syndromes or diffuse liver metastases. They have varying degrees of aggressiveness and varying malignant potential. The diagnostic workup includes biochemical testing for NET markers, imaging (computed tomography scans, MRI, and endoscopic ultrasound), and gallium-68 positron emission tomography. The diagnosis is confirmed by immunohistochemistry positivity and tumor is graded for classification and treatment purposes. Clinical management requires a multidisciplinary approach. Surgery is the most efficient approach for long-term benefits.
