Fernandes Michel Ribeiro, Ghezzi Caroline Lorenzoni Almeida, Grezzana-Filho Tomaz Jm, Feier Flávia Heinz, Leipnitz Ian, Chedid Aljamir Duarte, Cerski Carlos Thadeu Schmidt, Chedid Marcio Fernandes, Kruel Cléber Rosito Pinto
Department of Gastrointestinal Surgery and Transplantation, Hospital de Clínicas de Porto Alegre, Porto Alegre 90035-007, RS, Brazil.
Division of Radiology, Hospital de Clínicas de Porto Alegre, Porto Alegre 90035-903, RS, Brazil.
World J Gastrointest Surg. 2021 Mar 27;13(3):315-322. doi: 10.4240/wjgs.v13.i3.315.
Primary extra-gastrointestinal stromal tumors (E-GIST) of the liver are rare. The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect. Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care. However, under specific circumstances, a cytoreductive approach may represent a therapeutic option. We describe herein the case of an 84-year-old woman who presented with a tender, protruding epigastric mass. Abdominal computed tomography scan revealed a large, heterogeneous mass located across segments III, IV, V, and VIII of the liver. The initial approach was transarterial embolization of the tumor, which elicited no appreciable response. Considering the large size and central location of the tumor and the advanced age of the patient, non-anatomic complete resection was indicated. Due to substantial intraoperative bleeding and hemodynamic instability, only a near-complete resection could be achieved. Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver. Considering the risk/benefit ratio for therapeutic options, debulking surgery may represent a strategy to control pain and prolong survival.
Here, we present a case report of a patient diagnosed with E-GIST primary of the liver, which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.
E-GIST primary of the liver is a rare conditional, the treatment is with systemic therapy and total resection surgery. However, a cytoreductive surgery will be necessary when a complete resection is no possible.
原发性肝脏胃肠道外间质瘤（E-GIST）较为罕见。临床表现可从无症状到出血或出现占位效应的表现。肿瘤切除术后辅助使用伊马替尼治疗是标准的治疗方案。然而，在特定情况下，减瘤手术可能是一种治疗选择。我们在此描述一名84岁女性患者的病例，该患者上腹部出现压痛性突出肿块。腹部计算机断层扫描显示肝脏第III、IV、V和VIII段有一个巨大的、不均匀的肿块。最初的治疗方法是对肿瘤进行经动脉栓塞，但未取得明显效果。考虑到肿瘤体积大、位于中央以及患者年龄较大，建议进行非解剖性完整切除。由于术中出血量大且血流动力学不稳定，仅实现了近完整切除。组织病理学和免疫组化染色确诊为原发性肝脏E-GIST。考虑到治疗方案的风险/获益比，减瘤手术可能是控制疼痛和延长生存期的一种策略。
在此，我们报告一例被诊断为原发性肝脏E-GIST的患者病例，该病例接受了减瘤手术及伊马替尼辅助治疗。
原发性肝脏E-GIST是一种罕见病症，治疗方法是全身治疗和全切除手术。然而，当无法进行完整切除时，减瘤手术将是必要的。
