You Y Nancy, Thompson Geoffrey B, Young William F, Larson Dirk, Farley David R, Richards Melanie, Grant Clive S
Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Surgery. 2007 Dec;142(6):829-36; discussion 836.e1. doi: 10.1016/j.surg.2007.09.010.
Pancreatoduodenal (PD) neoplasms represent the principal disease-specific lethality in multiple endocrine neoplasia type 1 (MEN1). Potential oncologic benefits of PD resection must be weighed against operative morbidities, compromised pancreatic function, and quality of life (QOL).
Fifty MEN1 patients underwent PD resections during 1984-2004. Postoperative pancreatic function and QOL were assessed by EORTC QLQ-C30 and a disease-specific questionnaire (response rate, 78%).
Twelve patients (24%) had asymptomatic disease detected by screening; 38 patients (76%) were symptomatic. All gross neoplasm was resected in 80% of patients. No patients died; 21 patients (42%) had complications. At 5 years postoperatively, 60% of patients were alive without disease, 24% of patients were with disease, 10% of patients died of PD neoplasms, 4% of patients died of other malignancies, and 2% of patients died of an unknown cause. Diabetes that requires insulin or oral hypoglycemics developed in 20% of patients. Frequent steatorrhea (>once/week) occurred in 25% of patients, early dumping occurred in 25% of patients, bloating occurred in 25% of patients, late dumping occurred in 7% of patients, hypoglycemia occurred in 7% of patients, and vomiting occurred in 4% of patients. Global QOL did not differ from that of the reference population (72.8 vs 75.3; P = .58).
PD resections in MEN1 are associated with perioperative risks and altered pancreatic function. The moderate compromise in patient-perceived QOL suggests that most patients accept and adapt to these trade-offs for the potential of prolonged survival.
胰十二指肠（PD）肿瘤是1型多发性内分泌肿瘤（MEN1）中主要的疾病特异性致死原因。PD切除潜在的肿瘤学益处必须与手术并发症、胰腺功能受损以及生活质量（QOL）相权衡。
1984年至2004年间，50例MEN1患者接受了PD切除术。通过欧洲癌症研究与治疗组织生活质量核心问卷（EORTC QLQ-C30）和一份疾病特异性问卷对术后胰腺功能和生活质量进行评估（应答率为78%）。
12例患者（24%）通过筛查发现无症状疾病；38例患者（76%）有症状。80%的患者所有肉眼可见肿瘤均被切除。无患者死亡；21例患者（42%）出现并发症。术后5年时，60%的患者无病存活，24%的患者患有疾病，10%的患者死于PD肿瘤，4%的患者死于其他恶性肿瘤，2%的患者死因不明。20%的患者出现需要胰岛素或口服降糖药治疗的糖尿病。25%的患者出现频繁脂肪泻（>每周1次），25%的患者出现早期倾倒综合征，25%的患者出现腹胀，7%的患者出现晚期倾倒综合征，7%的患者出现低血糖，4%的患者出现呕吐。总体生活质量与参照人群无差异（72.8对75.3；P = 0.58）。
MEN1患者的PD切除与围手术期风险及胰腺功能改变有关。患者自我感知的生活质量有中度受损，这表明大多数患者为了延长生存的可能性接受并适应了这些权衡。
