Intraductal mucin-hypersecreting neoplasm "mucinous ductal ectasia": endoscopic recognition and management.
ABSTRACT 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.
Article: Genetic progression and heterogeneity in intraductal papillary-mucinous neoplasms of the pancreas.[show abstract] [hide abstract]
ABSTRACT: Intraductal papillary-mucinous neoplasms (IPMNs) of the pancreas are ideal neoplasms to study clonal progression and genetic diversity because of their large size and prominent intraductal component. We microdissected 55 histologically defined areas from 13 IPMNs, extracted the DNA from each, and performed polymerase chain reaction (PCR)-based microsatellite analysis to detect loss of heterozygosity on chromosome arms 1p, 3p, 6q, 8p, 9p, 17p, 18q, and 22q. LOH was identified at 1p in two cases, at 3p in four cases, at 6q in seven cases, at 8p in four cases, at 9p in eight cases, at 17p in five cases, at 18q in five cases, and at 22q in one of the IPMNs examined. In one of the IPMNs, the allelic losses were uniform throughout multiple microdissected areas, and in four of the IPMNs, there was evidence of clonal progression. In contrast, in three of the IPMNs, substantial allelic heterogeneity was seen. This remarkable heterogeneity may, in part, be due to the slow growth rate of these neoplasms.American Journal Of Pathology 12/1997; 151(5):1447-54. · 4.89 Impact Factor
Article: Intraductal papillary mucinous tumors of the pancreas: imaging studies and treatment strategies.[show abstract] [hide abstract]
ABSTRACT: We analyzed clinicopathologic and imaging features and the prognosis of intraductal papillary mucinous tumor (IPMT) of the pancreas to identify imaging findings indicative of malignancy and to establish the optimal treatment strategy. In IPMT, preoperative differentiation between adenoma and adenocarcinoma is often difficult. Appropriate treatment based on pathologic study and surgical outcome has not been adequately documented. Forty-one patients with IPMT underwent surgery; 15 with adenoma and 26 with adenocarcinoma; main duct type in 13, combined type in 12, and branch duct type in 16. In malignant IPMT, deep invasion was found in 62% and lymph node metastasis in 23% (peripancreatic nodes in 19% and distant nodes in 4%). Tumors with mural nodules (86%) had a significantly higher incidence of carcinoma than tumors without nodules (37%). IPMT with a main pancreatic duct > or =15 mm or tumor diameter > or =30 mm (branch duct type) showed a high prevalence of adenocarcinoma. Main duct (54%) and combined (58%) type tumor, and tumors with mural nodules (64%) often showed invasion. All five branch duct tumors less than 30 mm without nodules were adenomas. However, imaging studies could not definitely distinguish adenocarcinomas from adenomas. Complete resection was possible for all adenomas and 88% of adenocarcinomas. Five-year survival rates for patients with adenomas and adenocarcinomas were 100% and 82%, respectively. IPMT has a favorable prognosis, regardless of deep invasion or node metastasis. IPMT requires peripancreatic node dissection in addition to complete tumor excision. Node dissection may be omitted for branch duct tumors less than 30 mm without mural nodules.Annals of Surgery 12/1998; 228(5):685-91. · 7.49 Impact Factor