ArticleClinical journal of gastroenterology2026
Perihepatic dedifferentiated liposarcoma mimicking IgG4-related disease.
Article in Clinical journal of gastroenterology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundDedifferentiated liposarcoma (DDLPS) may originate in perihepatic adipose tissue, but can radiologically present as a hepatic mass. Moreover, dense Immunoglobulin G4 (IgG4)-positive plasma cell infiltration may mimic IgG4-related disease (IgG4-RD) and delay curative-intent resection. We herein describe a unique case that exemplifies this diagnostic pitfall. CASE PRESENTATION: A 67-year-old woman was incidentally found to harbor a hepatic mass in segment 6 along with small mesenteric nodules. Cross-sectional imaging showed heterogeneous non-fat containing lesions, while needle biopsy revealed abundant IgG4-positive plasma cells. Prednisolone treatment was commenced for presumed IgG4-related inflammatory pseudotumor. Although the lesions initially shrank slightly, they both enlarged during steroid tapering. Segment 6 hepatectomy with limited bowel resection was subsequently performed. Pathohistological examination revealed atypical stromal cells arising in perihepatic adipose tissue, and Mouse double minute protein 2 (MDM2) amplification confirmed liposarcoma infiltration into the liver and mesentery. Following the discontinuation of steroids, the patient has remained recurrence-free for fourteen months after surgery.
conclusionDDLPS can masquerade as IgG4-RD and even show temporary steroid responsiveness. When clinical or imaging treatment findings are atypical, comprehensive tissue evaluation with molecular analysis is essential to prevent diagnostic delay.
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