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Pancreatic Mucinous-Cystic Neoplasm, High Grade

MedGen UID:
459411
•Concept ID:
C2987185
•
Neoplastic Process
Synonyms: Pancreatic Mucinous Cystic Neoplasm with High Grade Dysplasia; Pancreatic Mucinous-Cystic Neoplasm with High Grade Dysplasia; Pancreatic Mucinous-Cystic Neoplasm, High-Grade

Definition

A non-invasive mucinous cystic neoplasm that arises from the pancreas and is characterized by the presence of severe dysplasia. The neoplastic columnar mucin-producing epithelial cells form papillae with irregular branching and budding. There is nuclear stratification, prominent nucleoli, and cellular pleomorphism. Mitotic activity is present and the mitoses may be atypical. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic Mucinous-Cystic Neoplasm, High Grade

Professional guidelines

PubMed

European evidence-based guidelines on pancreatic cystic neoplasms.
European Study Group on Cystic Tumours of the Pancreas
Gut 2018 May;67(5):789-804. Epub 2018 Mar 24 doi: 10.1136/gutjnl-2018-316027. PMID: 29574408Free PMC Article
Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.
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
A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas.
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article

Recent clinical studies

Etiology

Association of high-risk stigmata and worrisome features with advanced neoplasia in intraductal papillary mucinous neoplasms (IPMN): A systematic review.
Ohno E, Balduzzi A, Hijioka S, De Pastena M, Marchegiani G, Kato H, Takenaka M, Haba S, Salvia R
Pancreatology 2024 Feb;24(1):48-61. Epub 2023 Dec 7 doi: 10.1016/j.pan.2023.12.002. PMID: 38161091
Pancreatic Cyst Surveillance.
Lennon AM, Vege SS
Clin Gastroenterol Hepatol 2022 Aug;20(8):1663-1667.e1. Epub 2022 Apr 6 doi: 10.1016/j.cgh.2022.03.002. PMID: 35397230Free PMC Article
European evidence-based guidelines on pancreatic cystic neoplasms.
European Study Group on Cystic Tumours of the Pancreas
Gut 2018 May;67(5):789-804. Epub 2018 Mar 24 doi: 10.1136/gutjnl-2018-316027. PMID: 29574408Free PMC Article
Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.
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
A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas.
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article

Diagnosis

Association of high-risk stigmata and worrisome features with advanced neoplasia in intraductal papillary mucinous neoplasms (IPMN): A systematic review.
Ohno E, Balduzzi A, Hijioka S, De Pastena M, Marchegiani G, Kato H, Takenaka M, Haba S, Salvia R
Pancreatology 2024 Feb;24(1):48-61. Epub 2023 Dec 7 doi: 10.1016/j.pan.2023.12.002. PMID: 38161091
Surveillance of Pancreatic Cystic Neoplasms.
Chhoda A, Schmidt J, Farrell JJ
Gastrointest Endosc Clin N Am 2023 Jul;33(3):613-640. Epub 2023 Apr 24 doi: 10.1016/j.giec.2023.03.010. PMID: 37245939
European evidence-based guidelines on pancreatic cystic neoplasms.
European Study Group on Cystic Tumours of the Pancreas
Gut 2018 May;67(5):789-804. Epub 2018 Mar 24 doi: 10.1136/gutjnl-2018-316027. PMID: 29574408Free PMC Article
Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.
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
A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas.
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article

Therapy

Solid pseudopapillary neoplasm of pancreas: Two case reports.
Gurzu S, Bara T, Sincu M, Gabos S, Vlad DM, Bara T Jr, Beres H, Jung I
Medicine (Baltimore) 2019 Jul;98(29):e16455. doi: 10.1097/MD.0000000000016455. PMID: 31335701Free PMC Article
Prevalence and outcomes of cystic lesions of the transplant pancreas: The University of Wisconsin Experience.
Al-Qaoud TM, Martinez EJ, Sollinger HW, Kaufman DB, Redfield RR 3rd, Welch B, Leverson G, Odorico JS
Am J Transplant 2018 Feb;18(2):467-477. Epub 2017 Nov 15 doi: 10.1111/ajt.14540. PMID: 29024476
Patterns of recurrence and long-term outcomes in patients who underwent pancreatectomy for intraductal papillary mucinous neoplasms with high grade dysplasia: implications for surveillance and future management guidelines.
Blackham AU, Doepker MP, Centeno BA, Springett G, Pimiento JM, Malafa M, Hodul PJ
HPB (Oxford) 2017 Jul;19(7):603-610. Epub 2017 May 9 doi: 10.1016/j.hpb.2017.03.007. PMID: 28495434
Vascular endothelial growth factor, a novel and highly accurate pancreatic fluid biomarker for serous pancreatic cysts.
Yip-Schneider MT, Wu H, Dumas RP, Hancock BA, Agaram N, Radovich M, Schmidt CM
J Am Coll Surg 2014 Apr;218(4):608-17. Epub 2013 Dec 24 doi: 10.1016/j.jamcollsurg.2013.12.019. PMID: 24491241

Prognosis

Association of high-risk stigmata and worrisome features with advanced neoplasia in intraductal papillary mucinous neoplasms (IPMN): A systematic review.
Ohno E, Balduzzi A, Hijioka S, De Pastena M, Marchegiani G, Kato H, Takenaka M, Haba S, Salvia R
Pancreatology 2024 Feb;24(1):48-61. Epub 2023 Dec 7 doi: 10.1016/j.pan.2023.12.002. PMID: 38161091
State-of-the-art surgical treatment of IPMNs.
Salvia R, Burelli A, Perri G, Marchegiani G
Langenbecks Arch Surg 2021 Dec;406(8):2633-2642. Epub 2021 Nov 4 doi: 10.1007/s00423-021-02349-9. PMID: 34738168Free PMC Article
Precursor Lesions of Pancreatic Cancer.
Kim JY, Hong SM
Oncol Res Treat 2018;41(10):603-610. Epub 2018 Sep 28 doi: 10.1159/000493554. PMID: 30269131
Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.
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
A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas.
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article

Clinical prediction guides

Association of high-risk stigmata and worrisome features with advanced neoplasia in intraductal papillary mucinous neoplasms (IPMN): A systematic review.
Ohno E, Balduzzi A, Hijioka S, De Pastena M, Marchegiani G, Kato H, Takenaka M, Haba S, Salvia R
Pancreatology 2024 Feb;24(1):48-61. Epub 2023 Dec 7 doi: 10.1016/j.pan.2023.12.002. PMID: 38161091
Clinical outcomes of minimally invasive duodenum-preserving pancreatic head resection.
Zu G, Chen W, Wu D, Zhang Y, Chen X
BMC Surg 2023 Sep 21;23(1):288. doi: 10.1186/s12893-023-02170-9. PMID: 37735367Free PMC Article
State-of-the-art surgical treatment of IPMNs.
Salvia R, Burelli A, Perri G, Marchegiani G
Langenbecks Arch Surg 2021 Dec;406(8):2633-2642. Epub 2021 Nov 4 doi: 10.1007/s00423-021-02349-9. PMID: 34738168Free PMC Article
Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.
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
A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas.
Basturk O, Hong SM, Wood LD, Adsay NV, Albores-Saavedra J, Biankin AV, Brosens LA, Fukushima N, Goggins M, Hruban RH, Kato Y, Klimstra DS, Klöppel G, Krasinskas A, Longnecker DS, Matthaei H, Offerhaus GJ, Shimizu M, Takaori K, Terris B, Yachida S, Esposito I, Furukawa T; Baltimore Consensus Meeting
Am J Surg Pathol 2015 Dec;39(12):1730-41. doi: 10.1097/PAS.0000000000000533. PMID: 26559377Free PMC Article

Recent systematic reviews

Association of high-risk stigmata and worrisome features with advanced neoplasia in intraductal papillary mucinous neoplasms (IPMN): A systematic review.
Ohno E, Balduzzi A, Hijioka S, De Pastena M, Marchegiani G, Kato H, Takenaka M, Haba S, Salvia R
Pancreatology 2024 Feb;24(1):48-61. Epub 2023 Dec 7 doi: 10.1016/j.pan.2023.12.002. PMID: 38161091

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