Should PNETs occurring in patients with MEN 1 be approached differently from PNETs occurring sporadically?
Yes. Approximately 70% of patients with MEN 1 develop PNETs, with gastrinoma being the most common. Gastrinomas are commonly multifocal in these patients and, thus, aggressive surgery is often necessary to achieve a biochemical cure; however, this is not routinely recommended. The morbidity and mortality rates of aggressive surgical resection for multifocal gastrinomas combined with the availability of effective medical management of gastric acid hypersecretion sway many clinicians toward avoiding surgical resection unless there is suspicion of malignancy. It is recommended that surgery be avoided for tumors < 2 cm in size because of the low risk of hepatic metastases. For tumors > 2 cm in size, the risk of hepatic metastases is much higher. Enucleation of tumors in the pancreatic head combined with distal pancreatectomy is the recommended procedure. Pancreaticoduodenectomy is recommended in select cases of large tumors in the pancreatic head that are not amenable to enucleation.