Crosby J A, Catton C N, Davis A, Couture J, O'Sullivan B, Kandel R, Swallow C J
Department of Surgical Oncology, Princess Margaret Hospital, Toronto, Ontario, Canada.
Ann Surg Oncol. 2001 Jan-Feb;8(1):50-9. doi: 10.1007/s10434-001-0050-4.
Malignant gastrointestinal stromal tumors (M-GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal (GI) tract. Previous studies have included limited numbers of patients, and most included malignant and benign cases from throughout the GI tract. We reviewed the experience of a single tertiary cancer care center with M-GIST of the small intestine only.
A prospective database identified all patients seen from 1989 to 1998. Clinical and pathological data, treatment, and outcome were analyzed. Overall median follow-up time was 24 months (range, 1-176 months).
Fifty patients (31 male, 19 female) were identified. Mean age at diagnosis was 55 years. Disease was localized in 11 patients, locally advanced (invasion into adjacent organs/peritoneum) in 24 patients, perforated in 4 patients, multiple primary lesions in 2 patients, and distant metastases in 9 patients. All patients underwent resection, which was complete in 70%. Locoregional recurrence (LR) developed in 43% (median, 25 months), and distant metastases in 59% (median, 21 months) of patients at risk. At last follow-up, 14 patients were alive (6 disease-free), 2 had died disease-free, and 34 died with recurrent disease. Overall survival (OS) was similar for localized and locally advanced disease; OS also was similar for patients with multiple primaries and distant metastases at diagnosis. Patients were grouped into three stages: (I) patients with localized and locally advanced disease; (II) patients with perforated; and (III) patients with multiple primaries and distant metastases. Actuarial OS at 5 years was 41% (n = 50)--42% for those with complete resection and 8% for incomplete resection. Univariable analysis showed that earlier stage at diagnosis (P = .001) and completeness of resection (P = .004) predicted for longer OS.
Most patients with M-GIST of the small intestine relapse following resection, but survival may be prolonged. In univariable analysis, stage at presentation and complete resection were significant prognostic variables for OS; grade was not significant. Localized and locally advanced M-GIST of the small intestine have a mean OS > 5 years. Complete resection should be the goal of initial surgical treatment.
恶性胃肠道间质瘤（M-GIST）是起源于胃肠道（GI）壁的罕见间叶组织肿瘤。以往的研究纳入的患者数量有限，且大多数研究纳入了整个胃肠道的恶性和良性病例。我们回顾了一家单一的三级癌症治疗中心仅针对小肠M-GIST的治疗经验。
通过前瞻性数据库确定了1989年至1998年期间诊治的所有患者。分析了临床和病理数据、治疗方法及治疗结果。总体中位随访时间为24个月（范围1 - 176个月）。
共确定了50例患者（男性31例，女性19例）。诊断时的平均年龄为55岁。疾病局限于11例患者，局部进展期（侵犯相邻器官/腹膜）24例，穿孔4例，多发原发性病变2例，远处转移9例。所有患者均接受了手术切除，其中70%切除完整。有复发风险的患者中，43%发生了局部区域复发（LR，中位时间25个月），59%发生了远处转移（中位时间21个月）。在最后一次随访时，14例患者存活（6例无疾病复发），2例无疾病复发死亡，34例因疾病复发死亡。局限性和局部进展期疾病的总生存期（OS）相似；诊断时多发原发性病变和远处转移患者的OS也相似。患者被分为三个阶段：（I）局限性和局部进展期患者；（II）穿孔患者；（III）多发原发性病变和远处转移患者。5年精算OS为41%（n = 50），切除完整的患者为42%，切除不完整的患者为8%。单因素分析显示，诊断时分期较早（P = .001）和切除完整性（P = .004）预示着更长的OS。
大多数小肠M-GIST患者术后复发，但生存期可能延长。在单因素分析中，就诊时分期和完整切除是OS的重要预后变量；分级不具有显著性。小肠局限性和局部进展期M-GIST的平均OS>5年。完整切除应是初始手术治疗的目标。
