Concomitant intraductal papillary mucinous neoplasm and neuroendocrine tumor of the pancreas
ABSTRACT Intraductal papillary mucinous neoplasm (IPMN) and neuroendocrine tumor (NET) of the pancreas are rare tumors and their association is not expected to be frequent. However, certain studies have suggested that the concomitant occurence of these tumors may be more frequent than previously thought. In the current study, we describe a case of concomitant occurrence of IPMN and NET of the pancreas and review the clinicopathological features of previously published cases and the current one. A 68-year-old female was incidentally found to have dilatation of the main pancreatic duct. A distal pancreatectomy was performed under the clinical diagnosis of IPMN. Histopathological analysis revealed concomitant IPMN (low-grade) and NET G1 of the pancreas. Review of the clinicopathological features of the 15 previously reported cases of concomitant IPMN and NET of the pancreas as well as the present one indicated that: i) this condition mainly affects middle-aged females; ii) the main symptom is abdominal or back pain, or no symptoms; iii) a hormone production symptom was observed in only one case; iv) the most common degree of dysplasia of IPMN is low grade; and v) the size of the NET is not particularly large (average 15.1 mm), although the clinical behavior is not always indolent (metastasis was observed in 3 cases). It is well known that IPMNs are associated with a high incidence of extrapancreatic malignancies, including colorectal and gastric carcinomas. Concomitant pancreatic NET and extrapancreatic malignancies may occur, therefore, systemic surveillance of extrapancreatic neoplasms and detection of concomitant NETs of the pancreas are necessary for patients with IPMN.
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ABSTRACT: Intraductal papillary mucinous neoplasms (IPMNs) are uncommon tumours which can be associated with pancreatic and extrapancreatic malignancies. The association of IPMN and neuroendocrine tumours of the pancreas has been reported previously but is exceedingly rare. We report three cases of IPMN treated with total pancreatectomy/extended distal pancreatectomy. Histopathological analysis of the resected specimens revealed concomitant IPMN and neuroendocrine tumour. Two patients had adenocarcinoma as well. The presence of an IPMN may place the entire pancreas at risk of developing other tumour types and vigilance during all stages of management is necessary to ensure optimal treatment.JOP: Journal of the pancreas 01/2013; 14(4):423-427.
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ABSTRACT: Solid pseudopapillary neoplasm (SPN) is a rare and low-grade malignant pancreatic neoplasm composed of poorly cohesive monomorphic neoplastic cells forming solid and pseudopapillary structures with frequent hemorrhagic-cystic degeneration. Intraductal papillary mucinous neoplasm (IPMN) is a pancreatic exocrine tumor composed of intraductal papillary growth of mucin containing neoplastic cells in the main pancreatic duct or its major branches. In the case presented here, a 53-year-old, Japanese man was found to have multiple cystic lesions and dilatation of the main pancreatic duct in the neck of the pancreas. Histological examination revealed a main-duct and branch-duct type IPMN, of the gastric-type, involving the neck of the pancreas, associated with a 0.5 cm SPN in the caudal side of the IPMN. We diagnosed this case as synchronous SPN and IPMN. As far as we know, only one other case of synchronous SPN and IPMN has been reported. Both the present case and the previously reported case showed abnormal nuclear expression of β-catenin in SPN, whereas IPMN showed no abnormal nuclear expression. These results suggest that β-catenin abnormality is not a common pathogenetic factor of synchronous SPN and IPMN.World Journal of Gastroenterology 06/2013; 19(21):3358-63. DOI:10.3748/wjg.v19.i21.3358
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ABSTRACT: There are no established guidelines for pathologic diagnosis/reporting of intraductal papillary mucinous neoplasms (IPMNs). An international multidisciplinary group, brought together by the Verona Pancreas Group in Italy-2013, was tasked to devise recommendations. (1) Crucial to rule out invasive carcinoma with extensive (if not complete) sampling. (2) Invasive component is to be documented in a full synoptic report including its size, type, grade, and stage. (3) The term "minimally invasive" should be avoided; instead, invasion size with stage and substaging of T1 (1a, b, c; ≤0.5, >0.5-≤1, >1 cm) is to be documented. (4) Largest diameter of the invasion, not the distance from the nearest duct, is to be used. (5) A category of "indeterminate/(suspicious) for invasion" is acceptable for rare cases. (6) The term "malignant" IPMN should be avoided. (7) The highest grade of dysplasia in the non-invasive component is to be documented separately. (8) Lesion size is to be correlated with imaging findings in cysts with rupture. (9) The main duct diameter and, if possible, its involvement are to be documented; however, it is not required to provide main versus branch duct classification in the resected tumor. (10) Subtyping as gastric/intestinal/pancreatobiliary/oncocytic/mixed is of value. (11) Frozen section is to be performed highly selectively, with appreciation of its shortcomings. (12) These principles also apply to other similar tumoral intraepithelial neoplasms (mucinous cystic neoplasms, intra-ampullary, and intra-biliary/cholecystic). These recommendations will ensure proper communication of salient tumor characteristics to the management teams, accurate comparison of data between analyses, and development of more effective management algorithms.Annals of Surgery 03/2015; DOI:10.1097/SLA.0000000000001173