| Theme |
Pancreatic Endocrine Tumors |
| Title |
Endocrine Tumors of the Pancreas in Multiple Endocrine Neoplasia Type 1 |
| Author |
Masaaki Izukura |
Department of Surgical Oncology, Osaka University Medical School |
| Author |
Shin-ichiro Takai |
Department of Surgical Oncology, Osaka University Medical School |
| [ Summary ] |
Pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1 (MEN 1) are usually evaluated for diffuse hyperplasia or multiple microadenomas. These histological features and extrapancreatic involvement have caused difficulty in achieving complete tumor resection. The surgical strategy for gastrinoma in MEN 1 has been controversial, because of the development of medical treatments for peptic ulcer as well as the slow growth of gastrinomas. However, the resection of intrapancreatic tumors and of submucosal tumors in the duodenum with peripancreatic lymphnode dissection have resulted in the normalization of serum gastrin levels and in a decreased incidence of hepatic metastasis, implying improved overall survival. Therefore, aggressive surgical procedures based on meticulous localization methods, such as the selective arterial secretin infusion test (SASI test), percutaneous transhepatic portal vein sampling (PTPVS) and intraoperative ultrasonography, are recommended for eradication and biochemical cure. Insulinomas associated with MEN 1 are usually multiple and cannot be managed with medical treatments. Subtotal pancreatectomy with enucleation of tumors in the pancreatic head is recommended for curative resection of insulinomas. |