title: Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1.
abstract: Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors. Presurgical diagnosis of multiple endocrine neoplasia type 1 is therefore crucial to plan a proper intervention. Of note, hyperparathyroidism/multiple endocrine neoplasia type 1 should be surgically treated before pancreatic endocrine tumors/multiple endocrine neoplasia type 1 resection, apart from insulinoma. Non-functioning pancreatic endocrine tumors/multiple endocrine neoplasia type 1 >1 cm have a high risk of malignancy and should be treated by a pancreatic resection associated with lymphadenectomy. The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. The usual surgical treatment for insulinoma/multiple endocrine neoplasia type 1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of tumor lesions in the pancreatic head. Surgical procedures for glucagonomas, somatostatinomas, and vipomas/ multiple endocrine neoplasia type 1 are similar to those applied to sporadic pancreatic endocrine tumors. Some of these surgical strategies for pancreatic endocrine tumors/multiple endocrine neoplasia type 1 still remain controversial as to their proper extension and timing. Furthermore, surgical resection of single hepatic metastasis secondary to pancreatic endocrine tumors/multiple endocrine neoplasia type 1 may be curative and even in multiple liver metastases surgical resection is possible. Hepatic trans-arterial chemo-embolization is usually associated with surgical resection. Liver transplantation may be needed for select cases. Finally, pre-surgical clinical and genetic diagnosis of multiple endocrine neoplasia type 1 syndrome and localization of multiple endocrine neoplasia type 1 related tumors are crucial for determining the best surgical strategies in each individual case with pancreatic endocrine tumors.
authors.affiliation: Department of Surgery, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. mccm37@uol.com.br
authors.forename: Marcel Cerqueira Cesar
authors.initials: MC
authors.lastname: Machado
mesh_terms: D003937:Diagnosis, Differential; D015408:Gastrinoma / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007340:Insulinoma / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*