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Pancreatic intraductal papillary-mucinous carcinoma

MedGen UID:
235451
Concept ID:
C1335304
Neoplastic Process
Synonyms: intraductal papillary mucinous carcinoma of pancreas; Intraductal papillary mucinous carcinoma of pancreas; intraductal papillary-colloid carcinoma of pancreas; intraductal papillary-colloid carcinoma of the pancreas; intraductal papillary-colloidal carcinoma of pancreas; intraductal papillary-colloidal carcinoma of the pancreas; intraductal papillary-mucinous carcinoma of pancreas; intraductal papillary-mucinous carcinoma of the pancreas; IPMN; Pancreatic intraductal papillary mucinous carcinoma; pancreatic intraductal papillary mucinous carcinoma; pancreatic intraductal papillary-colloid carcinoma; pancreatic intraductal papillary-colloidal carcinoma; Pancreatic Intraductal Papillary-Mucinous Carcinoma; pancreatic intraductal papillary-mucinous carcinoma
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0004285
Orphanet: ORPHA424058

Definition

Intraductal papillary mucinous carcinoma of pancreas is a rare epithelial tumor of pancreas characterized by malignant, mucin-producing cystic mass, originating from the pancreatic ductal system, associated with local invasion and metastatic spread, composed of mucin-producing, columnar epithelial cells covering the dilated pancreatic ducts with a papillary structure. The presenting symptoms are non-specific and include abdominal pain, pancreatitis, steatorrhea, jaundice and diabetes. Many patients are asymptomatic at the time of diagnosis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic intraductal papillary-mucinous carcinoma

Professional guidelines

PubMed

Ohtsuka T, Fernandez-Del Castillo C, Furukawa T, Hijioka S, Jang JY, Lennon AM, Miyasaka Y, Ohno E, Salvia R, Wolfgang CL, Wood LD
Pancreatology 2024 Mar;24(2):255-270. Epub 2023 Dec 28 doi: 10.1016/j.pan.2023.12.009. PMID: 38182527
McGuigan A, Kelly P, Turkington RC, Jones C, Coleman HG, McCain RS
World J Gastroenterol 2018 Nov 21;24(43):4846-4861. doi: 10.3748/wjg.v24.i43.4846. PMID: 30487695Free PMC Article
Tanaka M, Fernández-Del Castillo C, Kamisawa T, Jang JY, Levy P, Ohtsuka T, Salvia R, Shimizu Y, Tada M, Wolfgang CL
Pancreatology 2017 Sep-Oct;17(5):738-753. Epub 2017 Jul 13 doi: 10.1016/j.pan.2017.07.007. PMID: 28735806

Supplemental Content

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