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Intraductal tubulopapillary neoplasm of pancreas

MedGen UID:
459415
Concept ID:
C2987189
Neoplastic Process
Synonyms: Intraductal Tubulopapillary Neoplasm, Pancreatic; Pancreatic Intraductal Tubulopapillary Neoplasm
 
Monarch Initiative: MONDO:0034987
Orphanet: ORPHA580572

Definition

A rare epithelial tumor of pancreas characterized by a solid, nodular mass growing within dilated pancreatic ducts, histologically composed of nodules of back-to-back tubular glands forming large cribriform structures, with high-grade dysplasia and ductal differentiation. There is no overt production of mucin. About half of the tumors occur in the head of the pancreas, one third involve the gland diffusely. Patients present with nonspecific symptoms including abdominal pain, vomiting, weight loss, steatorrhea, and diabetes mellitus, while obstructive jaundice is uncommon. This tumor type accounts for less than 1% of exocrine neoplasms and 3% of intraductal neoplasms of the pancreas. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntraductal tubulopapillary neoplasm of pancreas

Professional guidelines

Recent clinical studies

Etiology

Yu C, Lokan J, Jackett L, Nikfarjam M, Mills C
BMJ Case Rep 2022 Dec 22;15(12) doi: 10.1136/bcr-2021-246398. PMID: 36549752Free PMC Article
Furukawa T
Pathol Int 2022 Nov;72(11):529-540. Epub 2022 Sep 26 doi: 10.1111/pin.13272. PMID: 36161420Free PMC Article
Paolino G, Esposito I, Hong SM, Basturk O, Mattiolo P, Kaneko T, Veronese N, Scarpa A, Adsay V, Luchini C
Histopathology 2022 Sep;81(3):297-309. Epub 2022 May 27 doi: 10.1111/his.14698. PMID: 35583805Free PMC Article
Seki T, Watanabe A, Harimoto N, Shirabe K
Surg Today 2022 Apr;52(4):532-541. Epub 2021 Jul 1 doi: 10.1007/s00595-021-02324-3. PMID: 34196805
Klöppel G, Basturk O, Schlitter AM, Konukiewitz B, Esposito I
Semin Diagn Pathol 2014 Nov;31(6):452-466. Epub 2014 Sep 2 doi: 10.1053/j.semdp.2014.08.005. PMID: 25282472

Diagnosis

Yu C, Lokan J, Jackett L, Nikfarjam M, Mills C
BMJ Case Rep 2022 Dec 22;15(12) doi: 10.1136/bcr-2021-246398. PMID: 36549752Free PMC Article
Kuan LL, Dennison AR, Garcea G
Pancreas 2020 Apr;49(4):498-502. doi: 10.1097/MPA.0000000000001518. PMID: 32282762
Aşkan G, Bağci P, Memiş B, Baştürk O
Turk Patoloji Derg 2017;33(2):87-102. doi: 10.5146/tjpath.2017.01386. PMID: 28272684
Rooney SL, Shi J
Arch Pathol Lab Med 2016 Oct;140(10):1068-73. doi: 10.5858/arpa.2016-0207-RA. PMID: 27684978
Adsay V, Jang KT, Roa JC, Dursun N, Ohike N, Bagci P, Basturk O, Bandyopadhyay S, Cheng JD, Sarmiento JM, Escalona OT, Goodman M, Kong SY, Terry P
Am J Surg Pathol 2012 Sep;36(9):1279-301. doi: 10.1097/PAS.0b013e318262787c. PMID: 22895264

Therapy

Kölby D, Thilén J, Andersson R, Sasor A, Ansari D
Int J Clin Exp Pathol 2015;8(8):9672-80. Epub 2015 Aug 1 PMID: 26464736Free PMC Article

Prognosis

Paolino G, Esposito I, Hong SM, Basturk O, Mattiolo P, Kaneko T, Veronese N, Scarpa A, Adsay V, Luchini C
Histopathology 2022 Sep;81(3):297-309. Epub 2022 May 27 doi: 10.1111/his.14698. PMID: 35583805Free PMC Article
Kuan LL, Dennison AR, Garcea G
Pancreas 2020 Apr;49(4):498-502. doi: 10.1097/MPA.0000000000001518. PMID: 32282762
Kim H, Ro JY
Arch Pathol Lab Med 2018 Mar;142(3):420-423. doi: 10.5858/arpa.2016-0405-RSR2. PMID: 29494224
Rooney SL, Shi J
Arch Pathol Lab Med 2016 Oct;140(10):1068-73. doi: 10.5858/arpa.2016-0207-RA. PMID: 27684978
Adsay V, Jang KT, Roa JC, Dursun N, Ohike N, Bagci P, Basturk O, Bandyopadhyay S, Cheng JD, Sarmiento JM, Escalona OT, Goodman M, Kong SY, Terry P
Am J Surg Pathol 2012 Sep;36(9):1279-301. doi: 10.1097/PAS.0b013e318262787c. PMID: 22895264

Clinical prediction guides

Khristenko E, Hank T, Gaida MM, Kauczor HU, Hackert T, Klauß M, Mayer P
Sci Rep 2022 Sep 16;12(1):15557. doi: 10.1038/s41598-022-19517-6. PMID: 36114217Free PMC Article
Mafficini A, Simbolo M, Shibata T, Hong SM, Pea A, Brosens LA, Cheng L, Antonello D, Sciammarella C, Cantù C, Mattiolo P, Taormina SV, Malleo G, Marchegiani G, Sereni E, Corbo V, Paolino G, Ciaparrone C, Hiraoka N, Pallaoro D, Jansen C, Milella M, Salvia R, Lawlor RT, Adsay V, Scarpa A, Luchini C
Mod Pathol 2022 Dec;35(12):1929-1943. Epub 2022 Sep 2 doi: 10.1038/s41379-022-01143-2. PMID: 36056133Free PMC Article
Seki T, Watanabe A, Harimoto N, Shirabe K
Surg Today 2022 Apr;52(4):532-541. Epub 2021 Jul 1 doi: 10.1007/s00595-021-02324-3. PMID: 34196805
Akita M, Hong SM, Sung YN, Kim MJ, Ajiki T, Fukumoto T, Itoh T, Zen Y
Histopathology 2020 Jun;76(7):1005-1012. Epub 2020 May 15 doi: 10.1111/his.14103. PMID: 32181510
Shi C, Hruban RH
Hum Pathol 2012 Jan;43(1):1-16. Epub 2011 Jul 20 doi: 10.1016/j.humpath.2011.04.003. PMID: 21777948

Recent systematic reviews

Paolino G, Esposito I, Hong SM, Basturk O, Mattiolo P, Kaneko T, Veronese N, Scarpa A, Adsay V, Luchini C
Histopathology 2022 Sep;81(3):297-309. Epub 2022 May 27 doi: 10.1111/his.14698. PMID: 35583805Free PMC Article
Seki T, Watanabe A, Harimoto N, Shirabe K
Surg Today 2022 Apr;52(4):532-541. Epub 2021 Jul 1 doi: 10.1007/s00595-021-02324-3. PMID: 34196805
Date K, Okabayashi T, Shima Y, Iwata J, Sumiyoshi T, Kozuki A, Morita S, Hata Y, Noda Y, Nishioka A, Matsumoto M
Langenbecks Arch Surg 2016 Jun;401(4):439-47. Epub 2016 Mar 21 doi: 10.1007/s00423-016-1391-6. PMID: 27001682

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