Mucin-Producing Neoplasms of the Pancreas: An Analysis of Distinguishing Clinical and Epidemiologic Characteristics

Department of Surgery, Policlinico GB Rossi, University of Verona, Verona, Italy.
Clinical gastroenterology and hepatology: the official clinical practice journal of the American Gastroenterological Association (Impact Factor: 7.9). 10/2009; 8(2):213-9. DOI: 10.1016/j.cgh.2009.10.001
Source: PubMed


Mucin-producing neoplasms (MPNs) of the pancreas include mucinous cystic neoplasms (MCNs) and main-duct, branch-duct, and combined intraductal papillary mucinous neoplasms (IPMNs). MCNs and branch-duct IPMNs are frequently confused; it is unclear whether main-duct, combined, and branch-duct IPMNs are a different spectrum of the same disease. We evaluated their clinical and epidemiologic characteristics.
Patients who underwent resection for histologically confirmed MPNs were identified (N = 557); specimens were reviewed and eventually reclassified.
One hundred sixty-eight patients (30%) had MCNs, 159 (28.5%) had branch-duct IPMNs, 149 (27%) had combined IPMNs, and 81 (14.5%) had main-duct IPMNs. Patients with MCNs were significantly younger and almost exclusively women; 44% of patients with main-duct or combined IPMNs and 57% of those with branch-duct IPMNs were women. MCNs were single lesions located in the distal pancreas (95%); 11% were invasive. IPMNs were more frequently found in the proximal pancreas; invasive cancer was found in 11%, 42%, and 48% of branch-duct, combined, and main-duct IPMNs, respectively (P = .001). Patients with invasive MCN and those with combined and main-duct IPMNs were older than those with noninvasive tumors. The 5-year disease-specific survival rate approached 100% for patients with noninvasive MPNs. The rates for those with invasive cancer were 58%, 56%, 51%, and 64% for invasive MCNs, branch-duct IPMNs, main-duct IPMNs, and combined IPMNs, respectively.
MPNs comprise 3 different neoplasms: MCNs, branch-duct IPMNs, and main-duct IPMNs, including the combined type. These tumors have specific clinical, epidemiologic, and morphologic features that allow a reasonable degree of accuracy in preoperative diagnosis.

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Available from: Stefano Crippa
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    • "Furthermore, multifocal lesions can be found both in MD-, combined-, and BD-IPMNs [9]. BD-IPMNs present multifocal lesions in about half cases [3] [9]. Main pancreatic duct (MPD) can be entirely involved by the tumor that may extend along it or by synchronous skip lesions that are present in around 20% of the patients [10] [11]. "
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    ABSTRACT: Appropriate surgical strategies for management of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are a matter of debate. Preoperative and intraoperative evaluation of malignant potential of IPMN and of patient's comorbidities is of paramount importance to balance potential complications of surgery with tumors' risk of being or becoming malignant; the decision about the extent of pancreatic resection and the eventual total pancreatectomy needs to be determined on individual basis. The analysis of frozen-section margin of pancreas during operation is mandatory. The goal should be the complete resection of IPMN reaching negative margin, although there is still no agreed definition of "negative margin." Of note, the presence of deepithelization is often wrongly interpreted as absence of neoplasia. Management of resection margin status and stratification of surveillance of the remnant pancreas, based on characteristics of primary tumour, are of crucial importance in the management of IPMNs in order to decrease the risk of tumor recurrence after resection. Although risk of local and distant recurrence for invasive IPMNs is increased even in case of total pancreatectomy, also local recurrence after complete resection of noninvasive IPMNs is not negligible. Therefore, a long-term/life-time follow-up monitoring is of paramount importance to detect eventual recurrences.
    Full-text · Article · Sep 2014 · Gastroenterology Research and Practice
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    • "Depending on the site of the disease, IPMNs are classified as either main-duct (IPMN-MD) or branch-duct (IPMN-BD) type [2]. Mixed IPMNs, defined by a simultaneous involvement of both main pancreatic duct and its secondary branches, show close similarities with IPMN-MD in regard to clinico-pathologic and epidemiologic characteristics [3]. "
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    ABSTRACT: BACKGROUND: There are few data on the outcome of patients with intraductal papillary mucinous neoplasms of the pancreas meeting criteria for resection (Sendai-positive), and not operated. AIM: To evaluate outcome of patients with a resectable, Sendai-positive intraductal papillary mucinous neoplasm, and not operated. METHODS: Multicentre, retrospective analysis of prospectively enrolled patients, with resectable Sendai-positive, not-operated intraductal papillary mucinous neoplasm. Overall-survival and disease-specific survival were the primary end-point, and progression-free survival secondary. RESULTS: Thirty-five patients (60% male, median age 77) enrolled: 40% main-duct, 60% branch-duct intraductal papillary mucinous neoplasms. In 19 patients surgery was ruled out due to comorbidities, in 7 because aged>80, 9 refused surgery. Twelve (34.3%) patients died after a mean of 32.5 months, 8 due to disease progression, 4 due to comorbidities. The median overall, disease-specific and progression-free survival were 52, 55, and 44 months respectively. Main duct involvement and age at diagnosis were associated with worse overall and progression-free survival, only main duct involvement with worse disease-specific survival (52 months main duct vs. 64 branch duct; P=0.04). CONCLUSION: These results suggest that in elderly and comorbid patients with Sendai-positive intraductal papillary mucinous neoplasms, especially of the branch duct, a conservative approach could be reasonable, as associated with a relatively good outcome, and should be carefully discussed with the patients.
    Full-text · Article · Feb 2013 · Digestive and Liver Disease
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    • "In fact IPMNs-P can affect the ductal system of the pancreas with two distinct forms: neoplasms arising from the main duct with or without a synchronous involvement of the branch ducts (main-duct IPMNs-P, including " combined " IPMNs) or IPMNs involving only the secondary branches (branchduct IPMNs-P) [3]. Increasing evidence indicates that branch-duct IPMNs-P are much less likely to harbour cancer than main-duct IPMNs-P, and while guidelines put forth by the International Association of Pancreatology (IAP) suggest surgical resection for all main-duct IPMNs-P, they recommend observation alone for asymptomatic patients with branch-duct IPMNs-P less than 30 mm and without nodules [3] [4]. "

    Full-text · Article · Mar 2012 · Digestive and Liver Disease
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