Skeletal Radiology

, Volume 49, Issue 1, pp 155–160 | Cite as

IgG4-related disease: rare presentation as a soft-tissue mass in the thigh of an adolescent

  • Maud CrezeEmail author
  • Sofia Boussebaa
  • Thierry Lazure
  • Sylvain Briand
  • Charles Court
Case Report


We report a case of a 16-year–old boy who presented a soft-tissue mass in the anterior compartment of the right thigh discovered by positron emission tomography/computed tomography within the work-up of unexplained prolonged inflammatory syndrome. The mass has no calcification. Subsequently, magnetic resonance imaging of the femoral triangle was carried out. Axial short tau inversion recovery images showed a 3.5-cm ill-defined mass in the femoral triangle with focal areas of hypointensity, which suggests that there might be fibrosis or hemosiderin within the tumor. Axial T1-weighted images showed a slight hyperintense mass involving the iliopsoas muscle. Contrast-enhanced fat-suppressed T1-weighted imaging showed a heterogeneous solid enhancement. Adjacent thick fascia enhancement of the vastus intermedius and the vastus lateralis muscles extending from the mass as a tail-like margin suggested the infiltrative spread of the tumor along the fascial plane. The mass and the lymphadenopathy were excised. Immunohistochemically, tumor cells were staining for muscle actin and desmin. Many plasma cells were IgG4+ (175per high-power field) with a ratio IgG4+/IgG+ of 50%. The diagnosis of IgG4-related disease (IgG4-RD) was made. Although a diffuse array of musculoskeletal symptoms has been observed in IgG4-related disease, reports of biopsy-proven musculoskeletal involvement of the limb are rare. We showed the radiological features of IgG4-RD presenting as a soft-tissue mass of the thigh. Musculoskeletal involvement, clinical significance, and treatment of IgG4-RD are also discussed.


IgG4-related disease MRI Musculoskeletal FDG-PET Soft tissue 


Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflicts of interest.


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Copyright information

© ISS 2019

Authors and Affiliations

  • Maud Creze
    • 1
    Email author
  • Sofia Boussebaa
    • 1
  • Thierry Lazure
    • 2
  • Sylvain Briand
    • 3
  • Charles Court
    • 3
  1. 1.Radiology DepartmentCHU de BicêtreLe Kremlin-BicêtreFrance
  2. 2.Anatomopathology DepartmentBicêtre Hospital, APHPLe Kremlin-BicêtreFrance
  3. 3.Orthopaedic DepartmentBicêtre Hospital, APHPLe Kremlin-BicêtreFrance

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