Azar C, Van de Stadt J, Rickaert F, Devière M, Baize M, Klöppel G, Gelin M, Cremer M
Department of Medico-Surgical Hepato-Gastroenterology, Université Libre de Bruxelles, Belgium.
Gut. 1996 Sep;39(3):457-64. doi: 10.1136/gut.39.3.457.
BACKGROUND/AIM: The clinical presentation, pancreatographic findings, and outcome of patients with intraductal papillary mucinous tumours have not been reported in a large patient series in the English literature. This study reviewed 32 patients diagnosed between 1980 and 1994, with special attention to these features.
PATIENTS/METHOD: Data on 24 operated and eight non-operated patients were abstracted from inpatient, outpatient, and procedure records.
Acute pancreatitis was the most common presentation seen in 56% of patients. Relapses occurred during an average of 43 months before diagnosis. A patulous papilla was observed in 55% of the cases. Endoscopic pancreatography showed communicating cysts, a diffusely dilated main pancreatic duct, and amorphous defects in 42, 71, and 97% respectively. An invasive carcinoma was found in nine of 24 (37.5%) of operated patients: six of the patients (66%) died or developed metastases within three years after surgery. No mortality was related to the tumour in absence of invasive carcinoma. Benign recurrence on the remaining pancreas was unusual and occurred late after surgery.
Intraductal papillary mucinous tumours must be considered in the differential diagnosis of relapsing pancreatitis. Despite slow growing, these tumours have an obvious malignant potential and a very poor prognosis when invasive carcinoma has developed. Early recognition and resection are the cornerstones of treatment.
背景/目的：英文文献中尚未有大型患者系列报道导管内乳头状黏液性肿瘤患者的临床表现、胰管造影结果及预后。本研究回顾了1980年至1994年间诊断的32例患者，并特别关注这些特征。
患者/方法：从住院、门诊及手术记录中提取了24例手术患者和8例非手术患者的数据。
急性胰腺炎是最常见的表现，见于56%的患者。在诊断前平均43个月期间出现复发。55%的病例观察到乳头扩张。内镜胰管造影分别显示交通性囊肿、主胰管弥漫性扩张及无定形充盈缺损的比例为42%、71%和97%。24例手术患者中有9例（37.5%）发现侵袭性癌：其中6例（66%）在手术后三年内死亡或发生转移。在无侵袭性癌的情况下，无死亡与肿瘤相关。剩余胰腺的良性复发不常见，且发生在手术后较晚时间。
复发性胰腺炎的鉴别诊断中必须考虑导管内乳头状黏液性肿瘤。尽管生长缓慢，但这些肿瘤具有明显的恶性潜能，当发生侵袭性癌时预后极差。早期识别和切除是治疗的基石。
