文献考察1):GIST(Gastro-Intestinal Stromal Tumor).間葉系腫瘍(GIMT:Gastro-Intestinal Mesenchymal Tumor)241例(表).
【術前・術後のサーベイランスプログラム】 十二指腸・小腸 GIST・平滑筋肉腫
Author:西田俊朗(大阪大学 大学院医学系研究科臓器制御外科学病態制御外科), 門田守人
Source:外科(0016-593X)65巻12号 Page1424-1431(2003.11) 要旨:消化管の間葉系腫瘍(GIMT:Gastro-Intestinal Mesenchymal Tumor)は大きく分けてGIST, 筋原性腫瘍(平滑筋腫, 平滑筋肉腫), 神経原性腫瘍(神経鞘腫, 神経線維腫等)に分けられる. 肉眼的には鑑別はむずかしく, 組織学的にはHE染色でspindleないしepithelioidの形態をとる腫瘍細胞からなる. 免疫組織染色でKIT蛋白質を発現しているもの, あるいはKITを発現していなくてもCD34を他のマーカーに比し優位に発現しているものをGISTと定義する. KITを発現せずS-100蛋白質を発現しているものを神経原性腫瘍, KITを発現せずデスミンを優位に発現している腫瘍を筋原性腫瘍と診断する. これら3種GIMTの臨床的特徴・振る舞い・予後に大きな差は認めず, 原則的に術前・術後のサーベイランスは同じように考えればよい. 治療指針は図を参照.
文献考察2):malignant GIST 116例
Radiology. 2003 Feb;226(2):527-32. Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread.
Burkill GJ, Badran M, Al-Muderis O, Meirion Thomas J, Judson IR, Fisher C, Moskovic EC.
PURPOSE: To investigate and describe the anatomic distribution, imaging features, and pattern of metastatic spread of malignant gastrointestinal stromal tumors (GISTs). MATERIALS AND METHODS: The medical records of all patients at our institution with a histologic diagnosis of GIST were reviewed. Two radiologists with knowledge of the diagnosis reviewed the radiologic findings by means of consensus. Sixty-seven patients underwent computed tomography, and scans of the primary tumor were available in 38 patients. RESULTS: One hundred sixteen patients with malignant GISTs were identified (76 men and 40 women; mean age, 54.6 years +/- 13.5 [SD]). The primary tumor locations in descending order of frequency were the small bowel (n = 49), stomach (n = 43), colon (n = 7), rectum (n = 6), other (n = 3), and not specified (n = 8). Mean primary tumor size was 13 cm +/- 6. Tumors were typically well defined (31 of 36 [86%]), with a heterogeneous rim of soft tissue with lower signal intensity than that of the contrast material-enhanced liver. Central fluid attenuation was seen in 24 of 36 (67%) patients. Metastases were seen in 23 of 38 (61%) patients at presentation and in 53 of 61 (87%) patients during follow-up. Spread was usually to the liver or peritoneum. Visceral obstruction rarely occurred, even in the presence of extensive peritoneal metastatic disease. Ascites was an unusual finding. CONCLUSION: Malignant GISTs are typically large, well-circumscribed, heterogeneous, centrally necrotic tumors that arise in the wall of the small bowel or stomach. They rarely obstruct viscera, despite their large size and propensity to metastasize to the liver and peritoneum. PMID: 12563150(full text)
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