Tenner S, Carr-Locke D L, Banks P A, Brooks D C, Van Dam J, Farraye F A, Turner J R, Lichtenstein D R
Center for Pancreatic Disease, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
Am J Gastroenterol. 1996 Dec;91(12):2548-54.
Intraductal mucin-hypersecreting neoplasm (IMHN), also termed mucinous ductal ectasia, is a rare disorder of the pancreas characterized by distension of the pancreatic duct with mucus. This study attempted to clarify the clinical, radiographic, histological, and treatment approaches to this entity.
The medical records, radiological imaging studies, and pathology specimens of eight patients with IMHN seen during a 3-yr period were reviewed. The diagnosis of IMHN was established by findings during ERCP, which included mucin plugging of the papilla, mucin extrusion from the papillary orifice after intraductal injection of contrast medium, mucinous filling defects in the main pancreatic duct, and dilated main and branch pancreatic ducts in the absence of obstructing ductal strictures.
All patients presented with an initial clinical diagnosis of acute or chronic pancreatitis, suspected cystic neoplasm, or biliary obstruction. Noninvasive imaging studies such as transabdominal ultrasonography or CT and laboratory evaluation did not seem to help in defining the disease. Five patients underwent Whipple resection; pathology included papillary ductal hyperplasia in one, dysplastic mucinous epithelium in two, and mucinous cystadenocarcinoma in two. All five patients had associated histological evidence of chronic pancreatitis. All patients are alive and well after 21-53 months without evidence of residual disease.
IMHN has a wide spectrum of clinical, radiological, and histological features. The indolent biologic behavior and favorable prognosis of IMHN suggest that it is one of the most curable forms of pancreatic malignancy.
导管内黏液高分泌性肿瘤（IMHN），也称为黏液性导管扩张症，是一种罕见的胰腺疾病，其特征为胰管被黏液扩张。本研究试图阐明该疾病的临床、影像学、组织学及治疗方法。
回顾了3年期间收治的8例IMHN患者的病历、放射影像学研究及病理标本。IMHN的诊断通过内镜逆行胰胆管造影（ERCP）检查结果确立，包括乳头黏液堵塞、导管内注射造影剂后乳头开口处黏液挤出、主胰管内黏液性充盈缺损以及在无导管狭窄梗阻情况下主胰管和分支胰管扩张。
所有患者最初的临床诊断为急性或慢性胰腺炎、疑似囊性肿瘤或胆道梗阻。经腹超声或CT等非侵入性影像学检查及实验室评估似乎无助于明确该疾病。5例患者接受了胰十二指肠切除术；病理结果包括1例乳头导管增生、2例发育异常的黏液上皮以及2例黏液性囊腺癌。所有5例患者均有慢性胰腺炎的相关组织学证据。所有患者在21至53个月后均存活且状况良好，无残留疾病迹象。
IMHN具有广泛的临床、影像学和组织学特征。IMHN的惰性生物学行为和良好预后表明它是胰腺恶性肿瘤中最可治愈的类型之一。
