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lymphoplasmacytic inflammatory pseudotumor of the liver - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

lymphoplasmacytic inflammatory pseudotumor of the liver

MONDO:0035313

Also known as:IgG4-related inflammatory pseudotumor of the liver

Overview

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.

Diagnosis

Biomarker and diagnostic research for lymphoplasmacytic inflammatory pseudotumor of the liver has been reported in the published literature.

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for lymphoplasmacytic inflammatory pseudotumor of the liver.

15 publications have been identified in PubMed for lymphoplasmacytic inflammatory pseudotumor of the liver. Research spans Case Report / Case Series (67%), Review / Meta-Analysis (20%), and Diagnostic / Biomarker (7%).

Research Type	Count	% of Total
Patient case studies	10	67%

Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 5:36 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:555437

GARD

Genetic and Rare Diseases Info Center

GARD:22240

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lymphoplasmacytic inflammatory pseudotumor of the liver

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Recent Publications