| Theme | Update on Pancreatic Neuroendocrine Tumors | |
|---|---|---|
| Title | US,EUS of PNETs | |
| Publish Date | 2018/08 | |
| Author | Susumu Hijioka | Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital |
| Author | Yasunari Sakamoto | Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital |
| Author | Shunsuke Kondo | Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital |
| Author | Chigusa Morizane | Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital |
| Author | Yasunori Mizuguchi | Department of Diagnostic Radiology, National Cancer Center Hospital |
| Author | Takuji Okusaka | Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital |
| [ Summary ] | Although more than 90 % of pancreatic neuroendocrine neoplasms (PNETs/NEN) manifest sporadically, they arise at times as a partial disease with the background of multiple endocrine neoplasia type 1 (MEN-1 syndrome). MEN-1 syndrome is an autosomal dominant genetic disorder primarily affecting the three tissues of parathyroid gland, pituitary gland and pancreatic endocrine gland, and its causative gene is MEN1. The morbidity of pancreatic/gastrointestinal NET in MEN-1 syndrome patients is about 60 %, among which there are many cases having complications with gastrinoma and insulinoma. Since the treatment policy for PNETs complicated with MEN-1 syndrome is different from that of sporadic PNETs, and because correspondence and management of blood relatives is also very important, it is very important to diagnose whether or not there is MEN-1 syndrome complication, when examining NET. Furthermore, due to the relatively high number of cases, determination of the treatment policy will require multiple tests, including endoscopy, ultrasonic endoscopy, SACI test and somatostatin receptor scintigraphy etc. As many of the MEN-1 syndrome-related deaths are caused by NET, and as there are cases of MEN-1 syndrome identified due to pancreatic or gastrointestinal NET, the diagnosis and management of MEN-1 syndrome is paramount. |
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