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A Case of Gastrointestinal Stromal Tumor (GIST) of Jejunum Presenting as Hollow Organ Perforation

以腹腔中空臟器破裂為表徵之空腸胃腸道基質腫瘤的病例報告

摘要


引言:胃腸道基質腫瘤是最常見的胃腸道間質細胞腫瘤。詳盡的影像學檢查有助於各部門的醫療團隊決定最合適的治療計劃,但此腫瘤的臨床表徵具多樣性,常導致手術前做出正確的診斷十分困難。在此我們提出一個空腸胃腸道基質腫瘤破裂合併廣泛性腹膜炎的病例。 病例報告:一位六十九歲男性病患因疑似腹腔中空臟器破裂合併廣泛性腹膜炎而接受緊急開腹手術。術中發現了一顆位於Treitz's ligament遠端十五公分處已破裂的巨大空腸腫瘤以及三顆小於一公分的散佈性腹膜腫瘤。我們對此段空腸施行了局部腸道切除及端對端吻合手術,所有可見的散佈性腹膜腫瘤也一併予以完全切除。此病患於手術後順利康復出院。病理報告顯示了一顆七點五公分乘七公分乘六公分源自空腸外肌肉層的胃腸道基質腫瘤合併有明顯的出血性梗塞。此腫瘤因大於五公分以及顯微鏡底下高細胞分裂計數而被歸類為高度危險性胃腸道基質腫瘤。其餘的散佈性腹膜腫瘤均被證實為胃腸道基質腫瘤。 討論:腫瘤破裂為胃腸道基質腫瘤病患一種罕見的臨床表徵,占有症狀的胃腸道基質腫瘤病例大約百分之零到八。對於此類病患,手術中確定所有的腫瘤均被完全切除,以及評估有否轉移性病灶以決定疾病分期是十分重要的。故術前腹部電腦斷層檢查適用於被懷疑可能合併有腹內惡性腫瘤的急性腹症病患。把所有可見的腫瘤完全切除已被證實不足以治癒轉移性胃腸道基質腫瘤患者。終生接受口服Imatinib藥物治療伴隨定期腹部電腦斷層檢查對於這類病患是必要的。

並列摘要


Introduction: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. A detailed imaging study can help a multidisciplinary team to decide on an appropriate treatment strategy, but variability in clinical presentation often obscures a pre-operative diagnosis of GIST before laparotomy. We describe a case of perforated jejunal GIST with diffuse peritonitis. Case Report: A 69-year-old man with hollow organ perforation and diffuse peritonitis received emergency laparotomy. During the procedure, a large perforated jejunal tumor 15 cm distal to Treitz's ligament and three peritoneal-seeding tumors each less than 1 cm in size were identified. Segmental resection of the diseased jejunum with safe margin, end-to-end anastomosis, and total excision of gross peritoneal-seeding tumors were performed. The patient recovered uneventfully after surgery. Pathology revealed a 7.5×7×6 cm GIST with prominent hemorrhagic infarct arising from the external muscular layer of jejunum. The tumor was classified as high risk by its large size and high mitotic count. The peritoneal tumors were confirmed as seeding of GIST. Discussion: Tumor perforation is a rare presentation of patients with GISTs, accounting for 0-8% of symptomatic cases. In these patients, it is important to confirm that the disease has been completely resected, assess for metastases, and determine stage at laparotomy. A pre-operative computed tomography (CT) is indicated in selected patients with acute abdomen when malignancy is suspected. Complete resection has been proven insufficient for patients with metastatic GISTs. In this case, life-long Imatinib therapy after surgery with regular CT follow-up is necessary.

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