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Lymphoplasmacytic inflammatory pseudotumor of the liver

MedGen UID:
1843135
Concept ID:
C5680170
Neoplastic Process
Synonyms: IgG4-related inflammatory pseudotumor of the liver; lymphoplasmacytic inflammatory pseudotumor of the liver
 
Monarch Initiative: MONDO:0035313
Orphanet: ORPHA555437

Definition

A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with diffuse lymphoplasmacytic infiltration with histological features of IgG4-related disease (numerous IgG4-positive plasma cells, prominent eosinophils, stromal fibrosis, fibroblastic proliferations and, frequently, obliterative phlebitis), and that is likely located around the hepatic hilum. Most often it is discovered as an incidental finding. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphoplasmacytic inflammatory pseudotumor of the liver

Recent clinical studies

Etiology

Kaneko R, Mitomi H, Nakazaki N, Yano Y, Ogawa M, Sato Y
J Gastrointestin Liver Dis 2017 Sep;26(3):299-304. doi: 10.15403/jgld.2014.1121.263.eko. PMID: 28922443
Zen Y
Semin Liver Dis 2016 Aug;36(3):242-56. Epub 2016 Jul 28 doi: 10.1055/s-0036-1584319. PMID: 27466794
Ahn KS, Kang KJ, Kim YH, Lim TJ, Jung HR, Kang YN, Kwon JH
J Hepatobiliary Pancreat Sci 2012 Jul;19(4):405-12. doi: 10.1007/s00534-011-0436-z. PMID: 21894477
Zen Y, Fujii T, Sato Y, Masuda S, Nakanuma Y
Mod Pathol 2007 Aug;20(8):884-94. Epub 2007 Jun 15 doi: 10.1038/modpathol.3800836. PMID: 17571078
Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y
Am J Surg Pathol 2004 Sep;28(9):1193-203. doi: 10.1097/01.pas.0000136449.37936.6c. PMID: 15316319

Diagnosis

Primitivo A, Oliveira MH, Gonçalves A
BMJ Case Rep 2021 Aug 23;14(8) doi: 10.1136/bcr-2021-244572. PMID: 34426430Free PMC Article
Lee HE, Zhang L
Semin Diagn Pathol 2019 Nov;36(6):423-433. Epub 2019 Jul 24 doi: 10.1053/j.semdp.2019.07.007. PMID: 31358425
Zen Y
Semin Liver Dis 2016 Aug;36(3):242-56. Epub 2016 Jul 28 doi: 10.1055/s-0036-1584319. PMID: 27466794
Hirabayashi K, Zamboni G
Pathologica 2012 Apr;104(2):43-55. PMID: 22953500
Ahn KS, Kang KJ, Kim YH, Lim TJ, Jung HR, Kang YN, Kwon JH
J Hepatobiliary Pancreat Sci 2012 Jul;19(4):405-12. doi: 10.1007/s00534-011-0436-z. PMID: 21894477

Therapy

Wan Q, Xu Z, Liu X, Wu Z, Zhong Q, Wu C
Front Immunol 2024;15:1376276. Epub 2024 Apr 30 doi: 10.3389/fimmu.2024.1376276. PMID: 38745658Free PMC Article
Prayson RA
J Clin Neurosci 2015 Nov;22(11):1848-9. Epub 2015 Jul 15 doi: 10.1016/j.jocn.2015.04.007. PMID: 26188666

Prognosis

Baber A, Legendre P, Palmic P, Lupo-Mansuet A, Burroni B, Azoulay C, Szwebel TA, Costedoat-Chalumeau N, Leroy K, Blons H, Blay JY, Boudou-Rouquette P, Terrier B
Int J Surg Pathol 2024 Feb;32(1):150-154. Epub 2023 May 8 doi: 10.1177/10668969231168345. PMID: 37157817
Hastir D, Verset L, Lucidi V, Demetter P
Liver Int 2014 Jul;34(6):961. Epub 2013 Oct 1 doi: 10.1111/liv.12319. PMID: 24118880
Ahn KS, Kang KJ, Kim YH, Lim TJ, Jung HR, Kang YN, Kwon JH
J Hepatobiliary Pancreat Sci 2012 Jul;19(4):405-12. doi: 10.1007/s00534-011-0436-z. PMID: 21894477
Cheuk W, Chan JK, Shek TW, Chang JH, Tsou MH, Yuen NW, Ng WF, Chan AC, Prat J
Am J Surg Pathol 2001 Jun;25(6):721-31. doi: 10.1097/00000478-200106000-00003. PMID: 11395549

Clinical prediction guides

Miyagi A, Fujimoto D, Yoshikawa A, Okushi S, Miyagi J, Matsumoto R, Ogata S, Bando Y, Ogawa H, Tanahashi T
Clin J Gastroenterol 2022 Oct;15(5):975-980. Epub 2022 Jun 30 doi: 10.1007/s12328-022-01663-z. PMID: 35773570
Itazaki Y, Einama T, Konno F, Fujinuma I, Takihata Y, Iwasaki T, Ogata S, Tsujimoto H, Ueno H, Kishi Y
Clin J Gastroenterol 2021 Dec;14(6):1733-1739. Epub 2021 Sep 27 doi: 10.1007/s12328-021-01526-z. PMID: 34570358
Kaneko R, Mitomi H, Nakazaki N, Yano Y, Ogawa M, Sato Y
J Gastrointestin Liver Dis 2017 Sep;26(3):299-304. doi: 10.15403/jgld.2014.1121.263.eko. PMID: 28922443
Hastir D, Verset L, Lucidi V, Demetter P
Liver Int 2014 Jul;34(6):961. Epub 2013 Oct 1 doi: 10.1111/liv.12319. PMID: 24118880
Zen Y, Fujii T, Sato Y, Masuda S, Nakanuma Y
Mod Pathol 2007 Aug;20(8):884-94. Epub 2007 Jun 15 doi: 10.1038/modpathol.3800836. PMID: 17571078

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