| 特集名 | 膵神経内分泌腫瘍 update | |
|---|---|---|
| 題名 | 膵神経内分泌腫瘍のUS，EUS | |
| 発刊年月 | 2018年 08月 | |
| 著者 | 肱岡 範 | 国立がん研究センター中央病院肝胆膵内科 |
| 著者 | 坂本 康成 | 国立がん研究センター中央病院肝胆膵内科 |
| 著者 | 近藤 俊輔 | 国立がん研究センター中央病院肝胆膵内科 |
| 著者 | 森実 千種 | 国立がん研究センター中央病院肝胆膵内科 |
| 著者 | 水口 安則 | 国立がん研究センター中央病院放射線診断科 |
| 著者 | 奥坂 拓志 | 国立がん研究センター中央病院肝胆膵内科 |
| 【 要旨 】 | 膵神経内分泌腫瘍（pancreatic neuroendo- crine tumors/neoplasm；PNETs/NEN）は，US，EUSの典型例では境界明瞭で内部均一な腫瘍として捉えられ，比較的小さくても見つけやすい印象がある一方で，インスリノーマやガストリノーマのように，機能性腫瘍によるホルモン症状を呈しながらも，腫瘍が小さいために発見できないこともまれではない．また，NET G3やNEC G3の典型例は高分化PNETsの典型像とはかけ離れ，浸潤性膵管癌や腺房細胞癌に類似した画像所見を呈する．このようにPNETsは，G1からNECまでスペクトラムの広い疾患であるため，画像所見が多岐にわたり，また非典型例も多く存在する．最終的には，画像診断のみではなく，EUS-FNAなどの組織診断と併せた総合的な診断が必要な場合も多い．また，PNETsの組織診断には悪性度診断も要求される．昨年改訂されたWHO 2017分類でのNET G1～G3とNEC G3を正しく理解することが， もっとも重要である． | |
| Theme | Update on Pancreatic Neuroendocrine Tumors | |
|---|---|---|
| Title | US,EUS of PNETs | |
| 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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