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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

Abstract

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.

Keywords

IgG4-related disease MRI Musculoskeletal FDG-PET Soft tissue 

Notes

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflicts of interest.

References

  1. 1.
    Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51.CrossRefGoogle Scholar
  2. 2.
    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(9):1181–92.CrossRefGoogle Scholar
  3. 3.
    Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015;385(9976):1460–71.CrossRefGoogle Scholar
  4. 4.
    Palazzo E, Palazzo C, Palazzo M. IgG4-related disease. Joint Bone Spine. 2014;81(1):27–31.CrossRefGoogle Scholar
  5. 5.
    Priori R, Lucchino B, Cerbelli B, Alessandri C, Bottaro V, Zodda A, et al. An unusual manifestation of IgG4-related disease. Rheumatology (Oxford). 2018.  https://doi.org/10.1093/rheumatology/key050.CrossRefGoogle Scholar
  6. 6.
    Martinez-de-Alegria A, Baleato-Gonzalez S, Garcia-Figueiras R, Bermudez-Naveira A, Abdulkader-Nallib I, Diaz-Peromingo JA, et al. IgG4-related disease from head to toe. Radiographics. 2015;35(7):2007–25.CrossRefGoogle Scholar
  7. 7.
    Tan TJ, Ng YL, Tan D, Fong WS, Low AS. Extrapancreatic findings of IgG4-related disease. Clin Radiol. 2014;69(2):209–18.CrossRefGoogle Scholar
  8. 8.
    Katabathina VS, Khalil S, Shin S, Lath N, Menias CO, Prasad SR. Immunoglobulin G4-related disease: recent advances in pathogenesis and imaging findings. Radiol Clin N Am. 2016;54(3):535–51.CrossRefGoogle Scholar
  9. 9.
    Taniguchi Y, Kawano M, Zen Y, Aoyama N, Suehiro F, Terada Y. Immunoglobulin G4-related disease associated with extensive granulomatous changes. Rheumatology (Oxford). 2017;56(8):1430–3.CrossRefGoogle Scholar
  10. 10.
    Taki H, Matsui S, Shinoda K, Tobe K. Comment on: arthropathy with infiltrate IgG4-positive plasma cells in synovium. Rheumatology (Oxford). 2012;51(10):1922–4 author reply 1924–1925.CrossRefGoogle Scholar
  11. 11.
    Shinoda K, Matsui S, Taki H, Hounoki H, Ogawa R, Ishizawa S, et al. Deforming arthropathy in a patient with IgG4-related systemic disease: comment on the article by Stone et al. Arthritis Care Res. 2011;63(1):172.CrossRefGoogle Scholar
  12. 12.
    Umekita K, Kaneko Y, Yorita K, Hashiba Y, Matsuda M, Miyauchi S, et al. Arthropathy with infiltrate IgG4-positive plasma cells in synovium. Rheumatology (Oxford). 2012;51(3):580–2.CrossRefGoogle Scholar
  13. 13.
    Zhan Z, Lao M, Yang Z, Chen D, Yang X. Immunoglobulin G4-related disease presenting as bilateral arthritis of the hip joints. J Clin Rheumatol. 2018;24(7):398–401.CrossRefGoogle Scholar
  14. 14.
    Ando W, Yukioka F, Yamamoto K, Koyama T, Hashimoto Y, Yasui Y, et al. Immunoglobulin G4-related disease of the hip. Orthopedics. 2018;41(6):e876–9.CrossRefGoogle Scholar
  15. 15.
    Wu JS, Hochman MG. Soft-tissue tumors and tumorlike lesions: a systematic imaging approach. Radiology. 2009;253(2):297–316.CrossRefGoogle Scholar
  16. 16.
    Gruber L, Loizides A, Luger AK, Glodny B, Moser P, Henninger B, et al. Soft-tissue tumor contrast enhancement patterns: diagnostic value and comparison between ultrasound and MRI. AJR Am J Roentgenol. 2017;208(2):393–401.CrossRefGoogle Scholar
  17. 17.
    Nakamura T, Matsumine A, Matsubara T, Asanuma K, Yada Y, Hagi T, et al. Infiltrative tumor growth patterns on magnetic resonance imaging associated with systemic inflammation and oncological outcome in patients with high-grade soft-tissue sarcoma. PLoS One. 2017;12(7):e0181787.CrossRefGoogle Scholar
  18. 18.
    Robinson E, Bleakney RR, Ferguson PC, O’Sullivan B. Oncodiagnosis panel: 2007: multidisciplinary management of soft-tissue sarcoma. Radiographics. 2008;28(7):2069–86.CrossRefGoogle Scholar
  19. 19.
    Costa FM, Martins PH, Canella C, Lopes F. Multiparametric MR imaging of soft tissue tumors and pseudotumors. Magn Reson Imaging Clin N Am. 2018;26(4):543–58.CrossRefGoogle Scholar
  20. 20.
    Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, Tirumani SH, Shinagare AB, Hornick JL, et al. Role of imaging in management of desmoid-type fibromatosis: a primer for radiologists. Radiographics. 2016;36(3):767–82.CrossRefGoogle Scholar
  21. 21.
    Yoo HJ, Hong SH, Kang Y, Choi JY, Moon KC, Kim HS, et al. MR imaging of myxofibrosarcoma and undifferentiated sarcoma with emphasis on tail sign; diagnostic and prognostic value. Eur Radiol. 2014;24(8):1749–57.CrossRefGoogle Scholar
  22. 22.
    Surabhi VR, Chua S, Patel RP, Takahashi N, Lalwani N, Prasad SR. Inflammatory myofibroblastic tumors: current update. Radiol Clin N Am. 2016;54(3):553–63.CrossRefGoogle Scholar
  23. 23.
    Savvidou OD, Sakellariou VI, Papakonstantinou O, Skarpidi E, Papagelopoulos PJ. Inflammatory myofibroblastic tumor of the thigh: presentation of a rare case and review of the literature. Case Rep Orthop. 2015;2015:814241.PubMedPubMedCentralGoogle Scholar

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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