Skip to main content

Advertisement

SpringerLink
Log in

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

  • Case Report
  • Published:
Skeletal Radiology Aims and scope Submit manuscript

A Correction to this article was published on 27 December 2019

This article has been updated

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Change history

  • 27 December 2019

    Unfortunately in Volume 49, Issue 1 had been published online with an incorrect date (2001 instead of 2020).

References

  1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51.

    Article  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  3. Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015;385(9976):1460–71.

    Article  CAS  Google Scholar 

  4. Palazzo E, Palazzo C, Palazzo M. IgG4-related disease. Joint Bone Spine. 2014;81(1):27–31.

    Article  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  7. Tan TJ, Ng YL, Tan D, Fong WS, Low AS. Extrapancreatic findings of IgG4-related disease. Clin Radiol. 2014;69(2):209–18.

    Article  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  15. Wu JS, Hochman MG. Soft-tissue tumors and tumorlike lesions: a systematic imaging approach. Radiology. 2009;253(2):297–316.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Radiology Department, CHU de Bicêtre, 78 rue du Général Leclerc, 94270, Le Kremlin-Bicêtre, France

    Maud Creze & Sofia Boussebaa

  2. Anatomopathology Department, Bicêtre Hospital, APHP, Le Kremlin-Bicêtre, France

    Thierry Lazure

  3. Orthopaedic Department, Bicêtre Hospital, APHP, Le Kremlin-Bicêtre, France

    Sylvain Briand & Charles Court

Authors
  1. Maud Creze
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sofia Boussebaa
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Thierry Lazure
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sylvain Briand
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Charles Court
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maud Creze.

Ethics declarations

Conflicts of interest

The authors declare that they have no conflicts of interest.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Creze, M., Boussebaa, S., Lazure, T. et al. IgG4-related disease: rare presentation as a soft-tissue mass in the thigh of an adolescent. Skeletal Radiol 49, 155–160 (2020). https://doi.org/10.1007/s00256-019-03250-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00256-019-03250-9

Keywords

Search

Navigation