胰管内乳头状黏液性肿瘤的临床特征和预后分析

Clinical features and prognosis of intraductal papillary mucinous neoplasms of pancreas:analysis of 38 cases

目的 总结胰管内乳头状黏性肿瘤（IPMN）的诊治经验，提高对该病的认识和诊治水平。方法回顾性分析1999年5月至2005年7月底经手术切除且病理证实的38例胰管内乳头状黏性肿瘤病例的临床表现、病理特征和随访资料。结果本组38例IPMN，男23例、女15例；平均64．1岁±10．7岁（41～81岁）；主要临床表现为黄疸和腹痛。胰十二指肠切除术32例；胰体尾和脾切除术3例；全胰切除术1例；活检2例。围手术期死亡1例。全组主胰管型15例、分枝胰管型14例、混合型1例，分型不明8例，均有不同程度的胰管扩张（平均4．6mm）。按肿瘤侵袭性分，侵袭性组30例，非侵袭性组8例（良性、交界性和原位癌）。侵袭性IPMN与非侵袭性IPMN相比血清CAl9．9值明显较高。全组的中位生存期为18．5个月。侵袭性组的中位生存期为16．1个月；1、2和5年的生存率分别为54％、31％和21％。非侵袭性组的中位生存期为24．3个月，1和2的生存率分别为58％和38％，组间差异无统计学意义。按TNM分期，全组34例恶性肿瘤中，0期6例，Ⅰ期15例，Ⅱ期9例，Ⅲ期4例。0期、Ⅰ期、Ⅱ期、Ⅲ期的中位生存期分别为31．3、27、9．1和8．9个月（P=0．0124）。结论胰管内乳头状黏性肿瘤无特异性临床表现，胰管扩张是特征性影像学表现；侵袭性胰管内乳头状黏液性肿瘤血清CA19-9明显升高。不同TMN分期间的胰管内乳头状黏液性肿瘤的生存率有明显差异。

Objective To explore the diagnosis, clinical manifestation, treatment, and prognosis of intraductal papillary mucinous neoplasms （IPMNs） of pancreas. Methods The clinical data of 38 patients with IPMNs, 23 nudes and 15 females, aged 64. 1±10.7 （41 ±81 ）, were analyzed respectively. Results The main symptoms included abdominal pain and jaundice. Pancreaticeduedenectomy was performed on 32 patients, total pancreatectomy on 1 patient, distal pancreatectomy on 3 patients; and pancreatic biopsy on 2 patients. One patient died during the peri-operational period. Pathology showed 15 cases of main-duct type, 14 cases of branch-duct type, 1 case of mixed type, and 8 cases being un-differentiated, all with dilatation of pancreatic duct at different degrees 4.6 mm in diameter on average. There were 30 cases of invasive IPMNs, with significantly higher level of carbohydrate antigen 19-9 （ CA19-9 ） , and 8 non-invasive. The median survival time was 18.5 months in general. In the invasive IPMN group the general median survival time was 16.1 months, and the 1,2, and 5-year survival times were 54% , 31%, and 21% respectively; and in the non-invasive IPMN group the median survival time was 24.3 months, and the 1, 2, and 5-year survival times were 58% and 38% respectively; without significant differences in the survival times between these 2 groups. TMN staging showed 6 cases of stage 0, 15 cases of stage Ⅰ , 9 cases of stage Ⅱ , and 4 cases of stage Ⅲ among the 34 patients of malignant IPMNs. The median survival times of the patients of the stages 0, Ⅰ , Ⅱ , and Ⅲ were 31.3, 27, 9.1, and 8.9 months respectively with significant differences among them （P = 0. 0124）. Conclusion IPMN of pancreas has no specific clinical manifestation. Dilatation of pancreatic duct is a manifestation in imaging examination characteristic of IPMN. The serum CA19-9 level is significantly higher in the patients with invasive IPMN. There are significant differences in survival rate among different groups according to TMN staging.