Back
Management of pancreatic neuroendocrine tumors in patients with MEN 1.
Journal article

Management of pancreatic neuroendocrine tumors in patients with MEN 1.

  • Sadowski SM 1 Endocrine Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA ; 2 Thoracic and Endocrine Surgery, University Hospitals of Geneva, 1211 Geneva, Switzerland.
  • Triponez F 1 Endocrine Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA ; 2 Thoracic and Endocrine Surgery, University Hospitals of Geneva, 1211 Geneva, Switzerland.
  • 2015-02-26
Published in:
  • Gland surgery. - 2015
Pancreatic neuroendocrine tumors (PNETs)
multiple endocrine neoplasia type 1 (MEN1)
surgical management
English Pancreatic neuroendocrine tumors (PNETs) are frequent and can be non-functional (NF) in patients with multiple endocrine neoplasia type 1 (MEN1). Their identification is of clinical importance because malignant PNETs are reported to be the most common cause of death in patients with MEN1. Once the diagnosis of MEN1 is established in an individual based on clinical manifestations and/or genetic testing results, an active surveillance program is instituted for early detection and treatment of MEN1-associated disease. Ultrasonography, endoscopic ultrasonography (EUS), CT, MRI, selective arterial angiography and somatostatin receptor scintigraphy are all used for localization of tumors. Managing PNETs can be challenging and includes diagnosis, surveillance, adequate staging, and interdisciplinary, multimodal treatments to optimize patient outcome. Treatment includes surgical resection for loco-regional disease, as well as liver directed and targeted chemotherapies for advanced progressive disease. To date, the recommendation for surgical resection in NF-PNETs is based on tumor size, as a higher rate of metastases was found in patients with larger tumors. This review summarizes key concepts in managing PNETs in patients with MEN1.
Language
  • English
Identifiers
Persistent URL
https://sonar.ch/global/documents/232895
Statistics
Document views: 67 File downloads: