Department of Surgery, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506, Japan.
Surg Today. 2012 Dec;42(12):1234-9. doi: 10.1007/s00595-012-0135-7. Epub 2012 Feb 4.
This report presents the extremely rare case of an adult patient who presented with peritonitis caused by the perforation of an ileal duplication, associated with a gastrointestinal stromal tumor (GIST) arising within the duplication. A 70-year-old female was admitted to the hospital with lower abdominal pain. Abdominal computed tomography showed free air in the peritoneal cavity. An emergency laparotomy was performed, presuming diffuse peritonitis caused by a perforation of the gastrointestinal tract. The surgical findings showed that the peritonitis was caused by the perforation of an ileal duplication, 90 cm proximal to the ileocecal valve, with an extrinsic tumor protruding from the duplication. A segmental resection of the ileum-including the tumor and ileal duplication-was performed. Histopathology showed that the tumor was composed of spindle-shaped and epithelioid cells. The tumor was immunohistochemically positive for KIT, with a deletion mutation spanning the intron 10-exon 11 boundary. Accordingly, a diagnosis was made of a GIST arising within a duplication of the ileum. Duplications of the alimentary tract are encountered only occasionally in adults. Although quite rare, malignant change should therefore be considered as a possible complication when a solid portion is observed within a duplication of the alimentary tract.
本报告介绍了一例极为罕见的成年患者,其因回肠重复畸形穿孔导致腹膜炎,同时伴有起源于重复畸形的胃肠道间质瘤(GIST)。一名 70 岁女性因下腹疼痛入院。腹部 CT 显示腹腔内有游离气体。行急诊剖腹探查术,考虑为胃肠道穿孔引起的弥漫性腹膜炎。手术发现腹膜炎是由回肠重复畸形穿孔引起的,位于回盲瓣近端 90cm 处,一个外生性肿瘤从重复畸形中突出。行包括肿瘤和回肠重复畸形在内的回肠节段切除术。组织病理学显示肿瘤由梭形和上皮样细胞组成。肿瘤免疫组织化学 KIT 阳性,存在跨越内含子 10-外显子 11 边界的缺失突变。因此,诊断为回肠重复畸形内的 GIST。成人消化道重复畸形较为罕见。虽然很少见,但当消化道重复畸形中出现实性部分时,应考虑恶性变作为可能的并发症。