Clinical Experience of Intraductal Papillary Mucinous Neoplasms of the Pancreas

胰管內黏液乳突狀腫瘤之臨床經驗

陳建富(Chien-Fu Chen) ； 邱正堂(Cheng-Tang Chiu)

《臺灣消化醫學雜誌》 24卷1期 (2007/03) Pp. 1-12

https://doi.org/10.6557/GJT.200703_24(1).0001

摘要

胰管內黏液乳突狀腫瘤是一種罕見的胰臟腫瘤。其與其他的胰臟腫瘤有不同的臨床表現及病理特徵，並有較好的預後。追溯五年收集開刀病理組織中證實腫瘤由胰管所長出且胰管內有黏液的案例（5例）或者ERCP看到十二指腸乳頭所黏液流出且影像研究可看到胰管擴大並排除其餘黏液腫瘤的案例（1例）。我們分析其臨床表徵、影像檢查、腫瘤標記與預後。五例接受開刀。平均年紀為72.7歲(68~79)男女比例為2:1。大多的病人主訴為腹痛(5/6)。有胰臟炎者有二例。多數的患者有偏高的血糖及鹼性磷酸鹽。二位患者有黃疸。CA199升高者有五例(5/6)。影像檢查方面：ERCP可看到不規則或擴大的胰管，有時總膽管會有外源壓跡，ERCP無法定出腫瘤的位置。而電腦斷層及MRCP除了胰管的擴大病變外，可以辨認出囊狀腫瘤位置，但無法辨認出胰管內的腫瘤分佈。EUS檢查有三例皆可找到主腫瘤位置，但僅有一例可排除，其餘胰管內無腫瘤。開刀之病理組織，發現主胰管型(main duct type)有一例，支胰管型(branch duct type)有二例，合併型(combined type)有二例。三例主腫瘤在胰頭部其開刀法為Whipple procedure，一例在胰尾部其開法為尾端切除，一例為瀰漫型由頭部延伸至尾部，其開法為nearly total pancreatectomy。有二例有胰外器官侵犯，其中一例可開刀完全切除。另三例無胰外器官侵犯，但其中二例胰管邊緣有殘存腫瘤。所有的開刀案例皆惡性，但無一例有鄰近腹內淋巴轉移。所有開刀的案例目前皆存活，追蹤最久的一例為期五年。胰管內黏液乳突狀腫瘤的臨床表徵與平常腫瘤無甚大差異，可藉由影像得到診斷，針對腫瘤在整個胰管內分部狀況則不易掌握，積極的檢查與治療則是必需的，因其預後較佳。

關鍵字

胰管內黏液乳突狀腫瘤 ； 胰臟腫瘤

並列摘要

Intraductal papillary mucinous neoplasm is a rare disease. This study reviewed imaging and pathological studies in last 5 years. All patients with imaging study showing dilated main pancreatic duct or major branch duct with mucoid secreted from the papilla identified by ERCP and patients with an intraductal pancreatic tumor formed by papillary proliferations by mucin-producing cells were enrolled. Patients with mucin-producing tumors of the biliary tree and mucinous cystic tumors of the pancreas were excluded. Six patients matched inclusion criteria. Five patients underwent surgery and 1 received conservative treatment. Mean patient age was 72.7 years. The male-to-female ratio was 4:2. Abdominal pain was the most common presentation; 2 patients presented with an acute pancreatitis. Jaundice (n=2) was relatively less common. Four patients had hyperglycemia. Elevated blood CA19-9 was much more common than elevated blood CEA in this series. Magnetic resonance imaging (MRI) or computed tomography (CT) detected the cystic main tumor and not ductal spreading of tumors. ERCP showed patulous papilla extruding mucus, and dilated pancreatitic duct with an amorphous filling defect in all ERCP patients. However, ERCP failed to locate the main tumors. EUS and MRCP also did not detect ductal spreading. Five cases underwent surgery and all tumors were resectable although residual tumors at the resection margin were noted in 3 cases. All operated tumors were well- to moderately-differentiated adenocarcinomas. No patient had lymph node metastasis; however, 2 cases had adjacent organ invasion, 1 to the common bile duct, and 1 to the spleen and mesocolon. Patient prognosis was good. All patients survived over a mean observation period of 15.2 months; longest follow-up was 54 months. An intraductal papillary mucinous neoplasm is a rare disease of pancreas that requires a high index of suspicion for diagnosis. Clinically, the neoplasm may mimics pancreatitis and causes pancreatic insufficiency because of its chronic nature. Patulous papilla and cystic tumors along with dilated pancreatic duct are important imaging findings and are path gnomonic. Ductal spreading, which is important to resection decisions is not well demonstrated by imaging studies. Prognosis is good. Following complete resection, most patients have a favorable course.

並列關鍵字

intraductal papillary mucinous neoplasms ； pancreatic tumors