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Background <p>Dedifferentiated 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.</p> Case presentation <p>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.</p> Conclusion <p>DDLPS 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.</p>

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Perihepatic dedifferentiated liposarcoma mimicking IgG4-related disease

  • 期刊论文

  • 发表日期：

    2026年5月7日

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  • Soushi Shibusawa,
  • Yuki Yamashita,
  • Tomoyuki Tanaka,
  • Takefumi Kimura,
  • Shusei Sano,
  • Hisashi Tamada,
  • Yusuke Oka,
  • Yoshiki Ozawa,
  • Hiromitsu Mori,
  • Shuichi Wada

摘要

Background

Dedifferentiated 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.

Conclusion

DDLPS 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.