Department of Oncology, Mayo Clinic, Rochester, MN 55905, USA.
Pancreas. 2010 Oct;39(7):972-5. doi: 10.1097/MPA.0b013e3181df273b.
Limited data are available to guide the management of very rare exocrine neoplasms of the pancreas (VREP). Available evidence suggests that VREP have different risk factors and prognoses from those of adenocarcinoma of the pancreas. The primary objectives for this study were to determine the survival, comorbidities, and response to treatment of patients seen at Mayo Clinic with VREP.
We reviewed patients from 1975 to 2005 who had VREP and compared them to patients with adenocarcinomas that were matched for TNM, grade, and decade of treatment.
Sixty-six patients with VREP were identified. The most commonly identified neoplasms were acinar cell carcinoma (n = 15), small cell carcinoma (n = 12), and squamous cell carcinoma (n = 8). Abdominal discomfort and jaundice were the most common presenting symptoms. The median overall survival for patients with VREP, 10.4 months (range, 3.7-23 months), was better than that for matched controls, 8.2 months (range, 4-15.4 months) (P = 0.01). There was no difference in the survival of patients with stage 4 disease between cases, 8 months (range, 2.3-21.8 months), and controls, 6.7 months (range, 2.3-10.8 months) (P = 0.17).
We present one of the largest series of VREP to date. The overall survival of all patients with VREP was better than matched controls, but no statistical difference was seen between the groups with stage 4 disease.
关于胰腺外分泌罕见肿瘤（VREP）的治疗方法，目前仅有少量数据可供参考。现有证据表明，VREP 的风险因素和预后与胰腺腺癌不同。本研究的主要目的是确定在梅奥诊所就诊的 VREP 患者的生存情况、合并症和治疗反应。
我们回顾了 1975 年至 2005 年期间患有 VREP 的患者，并将其与 TNM、分级和治疗十年相匹配的胰腺腺癌患者进行了比较。
共确定了 66 例 VREP 患者。最常见的肿瘤是腺泡细胞癌（n=15）、小细胞癌（n=12）和鳞状细胞癌（n=8）。腹部不适和黄疸是最常见的首发症状。VREP 患者的中位总生存期为 10.4 个月（范围 3.7-23 个月），优于匹配对照组的 8.2 个月（范围 4-15.4 个月）（P=0.01）。在 4 期疾病患者中，病例组的 8 个月（范围 2.3-21.8 个月）和对照组的 6.7 个月（范围 2.3-10.8 个月）之间的生存差异无统计学意义（P=0.17）。
我们目前报告了最大的 VREP 系列之一。所有 VREP 患者的总体生存率均优于匹配对照组，但 4 期疾病患者之间无统计学差异。
