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Primary Extra-Gastrointestinal Stromal Tumor (GIST) arising from mesentery of small bowel and presenting as abdominal mass: A rare entity

机译:小肠系膜引起的原发性胃肠道间质瘤(GIST),表现为腹部肿块:一种罕见的实体

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Introduction: Majority of mesenchymal tumors of gastrointestinal tract are Gastrointestinal Stromal Tumor (GIST). It is, however, a rare tumor, accounting for less than 1% of primary gastrointestinal (GI) neoplasms. Though, these tumors are refractory to conventional chemotherapy or radiotherapy but show a good response to targeted adjuvant chemotherapy with tyrosine kinase inhibitors following surgical resection. Case Report: we report here a case of primary Extra-GIST tumor arising from mesentry of small bowel near duodeno-jejunal junction in a 69 years old male patient. The patient presented with a palpable mass in upper abdomen for past 15 days. On examination, a non-tender mobile lump of size around 17 × 10 cm, with bosselated surface and firm in consistency was palpable involving epigastric, left hypochondrium and umbilical region. Contrast enhanced computed tomography of abdomen revealed a heterogenous mesentric mass. On surgical intervention a mass was found involving mesentery near dudenojejunal junction without involvement of gastrointestinal tract. Complete surgical resection of the tumor was done and adjuvant chemotherapy with Imatinib mesylate was started as HPE revealing GIST with mitotic index of >10/50 HPF and 17 × 10 cm size placed the patient in high risk category. Patient was discharged on 12th of post-operative day with advice of regular follow-up. Conclusion: GIST occurrence is not restricted to bowel but can involve unusual sites also. The mainstay of treatment remains surgical resection with adequate margin. In cases where tumour has malignant potential (high mitotic figures on histopathology) adjuvent treatment with tyrosine kinase may prevent or delay relapse.
机译:简介:胃肠道间质肿瘤多数是胃肠道间质瘤(GIST)。但是,它是一种罕见的肿瘤,占原发性胃肠道(GI)肿瘤的不到1%。虽然,这些肿瘤对常规化学疗法或放射疗法是难治性的,但在手术切除后对酪氨酸激酶抑制剂的靶向辅助化学疗法表现出良好的反应。病例报告:我们在此报告了一名69岁男性患者因十二指肠-空肠交界处附近小肠肠系膜引起的原发Extra-GIST肿瘤的病例。过去15天,患者出现上腹部明显肿块。经检查,可触及的非招标性移动性肿块约17×10 cm,表面呈凸起状,结实牢固,涉及上腹,左软骨下和脐带区域。腹部增强对比计算机断层扫描显示不均匀的肠系膜肿块。在外科手术干预下,发现一个肿物累及十二指肠空肠交界处的肠系膜,而没有胃肠道的累及。进行了完整的肿瘤手术切除,并开始使用甲磺酸伊马替尼辅助化疗,因为HPE显示GIST的有丝分裂指数> 10/50 HPF,尺寸为17×10 cm,使患者处于高风险类别。病人在术后第12天出院,并建议定期随访。结论:GIST的发生不仅限于肠道,还可能涉及异常部位。治疗的主要内容仍然是手术切除,并留有足够的余量。在肿瘤具有恶性潜能的情况下(组织病理学上有丝分裂高的数字),酪氨酸激酶的辅助治疗可预防或延迟复发。

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