不完全型Carney triadに対する反復腹腔鏡下胃局所切除の経験：1例報告

Repeated laparoscopic gastric local resection for incomplete Carney triad : A case report

◆要旨：Carney triadはGIST・肺軟骨腫・副腎外傍神経節腫の3病変のうち，2病変以上を合併する症例を1つの症候群として捉えたもので稀な疾患である．今回筆者らはGISTに対する腹腔鏡下胃局所切除後約8年で残胃に発生した計7個のGISTに対し胃局所切除術を施行した1例を経験した．腹腔鏡下胃局所切除術を2か所で行い，集簇した5個の腫瘍はLECS手技で一括切除しえた．Carney triadは複数回の手術を要する疾患である．腹腔鏡下手術は癒着も少なく，今後の手術を行ううえでも適した術式と考える．また最小限の胃切除に留めることで，患者希望があれば胃温存の手術を複数回行える可能性も示唆された．〔なお本報告は2012年に坪井らが報告した症例（日呼外会誌26 : 79-84, 2012）の経過観察中に再切除を行ったものである．〕

　Carney triad, as originally described in 1977, is a combination of three tumors: gastric epithelioid leiomyosarcoma ［later renamed gastrointestinal stromal tumor （GIST）］, extra-adrenal paraganglioma, and pulmonary chondroma. The disorder affects mostly young women and is not familial. Zhang et al. reported that gastric lesions in the Carney triad occur in multiple nodules/multiple locations, with recurrence observed in 47％ of cases. They also reported that the mean age of the patients at first gastric operation is 22 years, and the mean interval between primary and secondary surgery is 10.3 years. Therefore, it is necessary to consider the quality of life of patients when choosing a treatment strategy. A 33-year-old female with Carney triad who had undergone right upper and middle lobectomy and laparoscopic partial gastrectomy with pulmonary chondroma and GIST at the age of 25 was found to have seven stromal tumors during follow-up observation. Two tumors were resected via laparoscopic local gastrectomy. Five colonized tumors were resected as a group via laparoscopy and endoscopy cooperative surgery （LECS）. Laparoscopic surgery or LECS leads to few adhesions and is a suitable technique for future surgery. By minimizing gastrectomy, it preserves the function of the stomach and allows performing multiple stomach-preserving surgeries depending on patient preference.