Sasmal Prakash K, Sharma Rakesh, Patra Susama, Mishra Tushar S, Mishra Pritinanda, Rout Bikram
Department of General Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Department of Pathology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Surg J (N Y). 2019 Aug 1;5(3):e65-e68. doi: 10.1055/s-0039-1693040. eCollection 2019 Jul.
Gastrointestinal stromal tumors (GISTs), the commonest mesenchymal tumors of gastrointestinal tract are often described to take origin from the interstitial cells of Cajal (ICC) or its precursor cells. Rarely these tumors do arise in structures other than the alimentary tract like omentum, mesentery, retroperitoneum, etc., of varying malignant potential and are known as extra-gastrointestinal stromal tumors (eGISTs). This is a case report of a 70-year-old female with multicentric malignant eGISTs arising in the mesentery of ileum. On laparotomy, a large mass of 20 × 15 cm was found in the small bowel mesentery without involvement of the adjacent ileum, with multiple other small nodules resembling lymph nodes, present adjacent to it. Histopathological study of the excised lump, confirmed the mass to be malignant eGIST without involvement of the adjacent ileum, with cluster differentiation (CD)117 positive and of high-risk stratification. The mesenteric nodule was confirmed on histopathology to be malignant eGIST, similar to that of that of the primary, without any lymphoid tissue. Adjuvant imatinib mesylate treatment was started immediately postoperation with the patient doing well at 1 year of follow-up. We report this case, due to the rare occurrence of multifocal malignant eGISTS of small bowel mesentery, which is yet to be reported. The existing literature is unclear regarding the clinicopathology and management of multifocal malignant stromal tumors of the mesentery.
胃肠道间质瘤（GISTs）是胃肠道最常见的间叶组织肿瘤，通常被认为起源于 Cajal 间质细胞（ICC）或其前体细胞。这些肿瘤很少发生在消化道以外的结构，如网膜、肠系膜、腹膜后等，具有不同的恶性潜能，被称为胃肠道外间质瘤（eGISTs）。本文报告一例 70 岁女性，患有多中心恶性 eGISTs，起源于回肠系膜。剖腹探查时，在小肠系膜中发现一个 20×15cm 的大肿块，未累及相邻的回肠，其附近还有多个类似淋巴结的小结节。对切除肿块的组织病理学研究证实，该肿块为恶性 eGIST，未累及相邻回肠，伴有簇分化（CD）117 阳性且为高危分层。肠系膜结节经组织病理学证实为恶性 eGIST，与原发灶相似，无任何淋巴组织。术后立即开始辅助甲磺酸伊马替尼治疗，患者随访 1 年情况良好。我们报告此病例，是因为小肠系膜多灶性恶性 eGISTs 罕见，此前尚未见报道。关于肠系膜多灶性恶性间质瘤的临床病理学和治疗，现有文献尚无明确阐述。
