Division of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada.
Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
Ann Surg Oncol. 2018 Aug;25(8):2391-2399. doi: 10.1245/s10434-018-6560-0. Epub 2018 Jun 18.
Goblet cell carcinoids (GCCs) of the appendix are rare mucinous neoplasms, for which optimal therapy is poorly described. We examined prognostic clinical and treatment factors in a population-based cohort.
Patients diagnosed with GCC from 1984 to 2014 were identified from the British Columbia Cancer Agency and the Vancouver Lower Mainland Pathology Archive.
Of 88 cases with confirmed appendiceal GCCs, clinical data were available in 86 cases (annual population incidence: 0.66/1,000,000). Median age was 54 years (range 25-91) and 42 patients (49%) were male. Metastasis at presentation was the strongest predictor of overall survival (OS), with median OS not reached for stage I-III patients, and measuring 16.2 months [95% confidence interval (CI) 9.1-29] for stage IV patients. In 67 stage I-III patients, 51 (76%) underwent completion hemicolectomy and 9 (17%) received adjuvant 5-fluorouracil-based chemotherapy. No appendicitis at initial presentation and Tang B histology were the only prognostic factors, with inferior 5-year recurrence-free survival (53 vs. 83% with appendicitis, p = 0.02; 45% Tang B vs. 89% Tang A, p < 0.01). Of 19 stage IV patients, 10 (62.5%) received 5-fluorouracil-based chemotherapy and 11 (61%) underwent multiorgan resection (MOR) ± hyperthermic intraperitoneal chemotherapy (HIPEC). Low mitotic rate and MOR ± HIPEC were associated with improved 2-year OS, but only MOR ± HIPEC remained significant on multivariate analysis (hazard ratio 5.4, 95% CI 1.4-20.9; p = 0.015).
In this population-based cohort, we demonstrate excellent survival outcomes in stage I-III appendiceal GCCs and clinical appendicitis. Hemicolectomy remains the standard treatment. In metastatic disease, outcomes remain poor, although MOR ± HIPEC may improve survival.
阑尾杯状细胞类癌（GCC）是一种罕见的黏液性肿瘤，其最佳治疗方法描述甚少。我们在基于人群的队列中研究了预后相关的临床和治疗因素。
从不列颠哥伦比亚癌症协会和温哥华低陆平原病理档案中确定了 1984 年至 2014 年间确诊的阑尾 GCC 患者。
在 88 例经证实的阑尾 GCC 病例中，有 86 例（年人群发病率：0.66/100 万）获得了临床数据。中位年龄为 54 岁（范围 25-91 岁），42 例（49%）为男性。就诊时的转移是总生存（OS）的最强预测因素，I-III 期患者的中位 OS 未达到，而 IV 期患者的中位 OS 为 16.2 个月（95%置信区间 9.1-29）。在 67 例 I-III 期患者中，51 例（76%）行半结肠切除术，9 例（17%）接受了基于 5-氟尿嘧啶的辅助化疗。初始表现无阑尾炎和 Tang B 组织学是唯一的预后因素，5 年无复发生存率（有阑尾炎者为 53%，无阑尾炎者为 83%，p=0.02；Tang B 者为 45%，Tang A 者为 89%，p<0.01）。在 19 例 IV 期患者中，10 例（62.5%）接受了基于 5-氟尿嘧啶的化疗，11 例（61%）行多器官切除术（MOR）+腹腔内热灌注化疗（HIPEC）。低有丝分裂率和 MOR+HIPEC 与 2 年 OS 改善相关，但仅在多变量分析中 MOR+HIPEC 仍具有显著意义（风险比 5.4，95%CI 1.4-20.9；p=0.015）。
在本基于人群的队列中，我们证明了 I-III 期阑尾 GCC 患者和伴有临床阑尾炎的患者具有极好的生存结果。半结肠切除术仍然是标准治疗方法。在转移性疾病中，预后仍然较差，尽管 MOR+HIPEC 可能改善生存。
