IGG4 Related Inflammatory Pseudotumor of the Liver: Particularities of a Rare Diagnosis

Authors

  • Raquel Maia Serviço de Medicina IV; Hospital Prof. Doutor Fernando da Fonseca; Amadora; Portugal
  • Marta Jonet Serviço de Medicina IV; Hospital Prof. Doutor Fernando da Fonseca; Amadora; Portugal
  • João Serôdio Serviço de Medicina IV; Hospital Prof. Doutor Fernando da Fonseca; Amadora; Portugal
  • Catarina Favas Serviço de Medicina IV; Hospital Prof. Doutor Fernando da Fonseca; Amadora; Portugal
  • António Alves Serviço de Anatomia Patológica; Hospital Prof. Doutor Fernando da Fonseca; Amadora; Portugal/Instituto de Anatomia Patológica; Faculdade de Medicina da Universidade de Lisboa; Lisboa; Portugal

DOI:

https://doi.org/10.24950/rspmi/CC/205/1/2018

Keywords:

Immunoglobulin G, Liver Neoplasms

Abstract

Liver´s inflammatory pseudotumor (LIP) is a rare, benign,
nonneoplastic mass, that constitutes an exaggerated inflammatory response to an aggression – infection/trauma/autoimmune disease. Recently, an IgG4-related LIP has been
recognized. We present a 57-year-old male with prostatic adenocarcinoma and chronic alcoholism with fever and abdominal pain. Laboratory tests showed leucocytosis (16100/uL),
elevated C-reactive protein (52.7 mg/dL), high sedimentation
rate (130 mm/h) and hepatic cytolysis. Hepatic nodules, suspicious of secondary/primary lesions were observed by computerized tomography. Alpha-fetoprotein and serotypes for
hepatotropic viruses were negative. Streptococcus viridans
was isolated in the blood cultures and targeted antibiotic
therapy was started. Fever disappeared but hepatic lesions
remained. Increased level of serum IgG4 (188 mg/dL) was
observed. The liver biopsy showed myofibroblast proliferation with storiform pattern, suggesting LIP, and IgG4+ plasmocytes. Steroid therapy was initiated successfully. The diagnosis of IgG4 related LIP is rare, however, the prognosis is
usually good after a course of steroid therapy

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References

Shibata M, Matsubayashi H, Aramaki T, Uesaka K, Tsutsumi N, Sasaki K, et al. A case of IgG4-related hepatic inflammatory pseudotumor replaced by an abscess after steroid treatment. BMC Gastroenterol. 2016;16:89.

Narla LD, Newman B, Spottswood SS, Narla S, Kolli R. Inflammatory pseudotumor. Radiographics. 2003;23:719-29.

Levy S, Sauvanet A, Diebold MD, Marcus C, Da Costa N, Thiefin G. Spontaneous regression of an inflammatory pseudotumor of the liver presenting as an obstructing malignant biliary tumor. Gastrointest Endosc. 2001;53:371-4.

Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181-92.

Le Borgne J, Lehur PA, Bitar O, Prunet D, Rochedreux A, Lenne Y. Les pseudo-tumeurs inflammatoires du foie. A propos de deux cas. Chirurgie. 1991;117:840-6; discussion 6-7.

Grouls V. Pseudolymphoma (inflammatory pseudotumor) of the liver. Zentralbl Allg Pathol. 1987;133:565-8.

Collina G, Baruzzi G, Eusebi V. Inflammatory pseudotumor of the liver: report of two cases. Tumori. 1987;73:407-12.

Lupovitch A, Chen R, Mishra S. Inflammatory pseudotumor of the liver. Report of the fine needle aspiration cytologic findings in a case initially misdiagnosed as malignant. Acta Cytol. 1989;33:259-62.

Rosa B, Moutinho-Ribeiro P, Pereira JM, Fonseca D, Lopes J, Amendoeira I, et al. Ghost tumor: an inflammatory pseudotumor of the liver. Gastroenterol Hepatol. 2012;8:630-3.

Balabaud C, Bioulac-Sage P, Goodman ZD, Makhlouf HR. Inflammatory pseudotumor of the liver: a rare but distinct tumor-like lesion. Gastroenterol Hepatol. 2012;8:633-4.

Kawaguchi T, Mochizuki K, Kizu T, Miyazaki M, Yakushijin T, Tsutsui S, et al. Inflammatory pseudotumor of the liver and spleen diagnosed by percutaneous needle biopsy. World J Gastroenterol. 2012;18:90-5.

Pieringer H, Parzer I, Wohrer A, Reis P, Oppl B, Zwerina J. IgG4- related disease: an orphan disease with many faces. Orphanet J Rare Dis. 2014;9:110.

Umehara H, Nakajima A, Nakamura T, Kawanami T, Tanaka M, Dong L, et al. IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity. Int Immunol. 2014;26:585-95.

Pfeifer L, Agaimy A, Janka R, Boxberger F, Wein A, Neurath MF, et al. Complete long-term remission of an inflammatory pseudotumor under corticosteroid therapy. Case Rep Oncol. 2011;4:304-10.

Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22:1-14.

Published

2018-03-13

How to Cite

1.
Maia R, Jonet M, Serôdio J, Favas C, Alves A. IGG4 Related Inflammatory Pseudotumor of the Liver: Particularities of a Rare Diagnosis. RPMI [Internet]. 2018 Mar. 13 [cited 2024 Nov. 23];25(1):43-6. Available from: https://revista.spmi.pt/index.php/rpmi/article/view/438

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Section

Case Reports

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