Rossi Roberta Elisa, Luong Tu-Vinh, Caplin Martyn Evan, Thirlwell Christina, Meyer Tim, Garcia-Hernandez Jorge, Baneke Alex, Conte Dario, Toumpanakis Christos
Neuroendocrine Tumor Unit, Centre of Gastroenterology, Royal Free Hospital, London, UK; Gastroenterology and Endoscopy Unit, Fondazione IRCCS Cà Granda - Ospedale Maggiore Policlinico and Department of Pathophysiology and Transplantation, Università degli Studi Milano, Italy.
Department of Histopathology, Royal Free Hospital, London, UK.
Surg Oncol. 2015 Mar;24(1):47-53. doi: 10.1016/j.suronc.2015.01.001. Epub 2015 Jan 28.
Appendiceal Goblet cell tumors (GCTs) are clinically more aggressive, and have a worse outcome than midgut neuroendocrine tumors (mNETs). Guidelines for management of GCTs are limited.
A retrospective case-study analysis was performed in patients with a diagnosis of GCT, confirmed on histological review. Patients were evaluated clinically, biochemically, and radiologically.
48 patients were identified (TNM stage I-II: 27, stage III: 15, stage IV: 6). Median follow-up was 44 months and was complete in all patients. 68.8% presented with acute appendicitis. 44/48 patients had initial appendectomy, followed by prophylactic right hemicolectomy in 41. 10/48 patients had recurrent disease [median time to recurrence 28 months (range 4-159)]. Of those, 9 received systemic chemotherapy (FOLFOX/FOLFIRI), which was also given in 5/48 patients with disseminated disease at diagnosis. Partial response, stable disease and disease progression was noted in 22%, 22% and 56%, respectively. Adjuvant chemotherapy was also administered in 9/48 patients with stage III disease after right hemicolectomy, however in 3/9 the disease recurred. Median progression/disease-free-survival was 44 months (range 3-159) and overall 5-year survival rate was 41.6%.
The clinical behaviour of GCTs is more similar to colorectal adenocarcinomas than to NETs. A prophylactic right hemicolectomy is recommended to reduce the risk of recurrence. Systemic chemotherapy, using colorectal adenocarcinoma regimens, is indicated for advanced or recurrent disease and has encouraging results. Prospective studies are needed to define the role of adjuvant chemotherapy and the optimal chemotherapy regimen.
阑尾杯状细胞肿瘤（GCTs）在临床上侵袭性更强，预后比中肠神经内分泌肿瘤（mNETs）更差。GCTs的管理指南有限。
对经组织学检查确诊为GCT的患者进行回顾性病例研究分析。对患者进行临床、生化和影像学评估。
共纳入48例患者（TNM分期I-II期：27例，III期：15例，IV期：6例）。中位随访时间为44个月，所有患者均完成随访。68.8%的患者表现为急性阑尾炎。44/48例患者最初接受了阑尾切除术，随后41例接受了预防性右半结肠切除术。10/48例患者出现疾病复发[复发的中位时间为28个月（范围4-159个月）]。其中，9例接受了全身化疗（FOLFOX/FOLFIRI），5/48例诊断时患有播散性疾病的患者也接受了该化疗。分别观察到部分缓解、疾病稳定和疾病进展的比例为22%、22%和56%。9/48例III期疾病患者在右半结肠切除术后也接受了辅助化疗，但其中3/9例疾病复发。中位无进展生存期/无病生存期为44个月（范围3-159个月），总体5年生存率为41.6%。
GCTs的临床行为与结直肠癌比与NETs更相似。建议进行预防性右半结肠切除术以降低复发风险。对于晚期或复发性疾病，使用结直肠癌方案的全身化疗是有效的，且结果令人鼓舞。需要进行前瞻性研究以确定辅助化疗的作用和最佳化疗方案。
