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Large dural-based mass with bony hyperostosis in a 16-year-old male: IgG4-related disease mimicking lymphoplasmacyte-rich meningioma

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Background: IgG4-related disease is an autoimmune process that presents with tumefactive lesions characterized by storiform fibrosis, a dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, obliterative phlebitis, and often elevated serum IgG4 levels. Central nervous system IgG4-related disease is very rare and usually occurs in the form of hypertrophic pachymeningitis or hypophysitis. Presentation as a large solitary meningioma-like mass with overlying hyperostosis in a young adult has not been reported before. Case summary: A 16-year-old male presented with focal seizures for 5 months. Imaging showed a large, extra-axial, and contrast-enhancing mass lesion in the left frontoparietal region with focal calvarial thickening. Histopathology revealed a fibrosclerotic lesion involving dura with a polymorphic infiltrate of plasma cells, mature lymphocytes, histiocytes, and occasional eosinophils. Immunohistochemical workup excluded the possibilities of meningioma, lymphoproliferative neoplasms, and histiocytic lesions. Majority of plasma cells were IgG4+ rendering a diagnosis of IgG4-related disease. Further serological and imaging workup did not reveal any evidence of systemic involvement. His serum IgG4 levels were normal. Considering a gross total resection of the lesion, no further treatment was given and the patient has been asymptomatic since. Conclusion: IgG4-related lesions of the CNS are under-recognized and accurate diagnosis, especially in those with isolated CNS disease and normal serum IgG4 levels, necessitates robust histopathological and laboratory workup to exclude mimics. They may occur as large dural masses with hyperostosis and differentiation from lymphoplasmacyte-rich meningiomas, in particular, can be challenging. While steroids are the mainstay of treatment in IgG4-related disease, surgical resection may be curative in solitary lesions presenting with compressive symptoms.

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  1. Baptista B, Casian A, Gunawardena H, D’Cruz D, Rice CM (2017) Neurological manifestations of IgG4-related disease. Curr Treat Options Neurol 19:14

    Article  PubMed Central  PubMed  Google Scholar 

  2. Lin CK, Lai DM (2013) IgG4-related intracranial hypertrophic pachymeningitis with skull hyperostosis: a case report. BMC Surg 13:37

    Article  PubMed Central  PubMed  Google Scholar 

  3. Wallace ZS, Carruthers MN, Khosroshahi A, Carruthers R, Shinagare S, Stemmer-Rachamimov A, Deshpande V, Stone JH (2013) IgG4-related disease and hypertrophic pachymeningitis. Medicine (Baltimore) 92:206–216

    Article  CAS  Google Scholar 

  4. Deshpande V, Zen Y, Chan JK et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192

    Article  Google Scholar 

  5. Radotra BD, Aggarwal A, Kapoor A, Singla N, Chatterjee D (2016) An orphan disease: IgG4-related spinal pachymeningitis: report of 2 cases. J Neurosurg Spine 25:790–794

    Article  PubMed  Google Scholar 

  6. Hwang G, Jin SY, Kim HS (2016) IgG4-related disease presenting as hypertrophic pachymeningitis and compressive optic neuropathy. Joint Bone Spine 83:601–602

    Article  PubMed  Google Scholar 

  7. Della-Torre E, Galli L, Franciotta D, Bozzolo EP, Briani C, Furlan R, Roveri L, Sessa M, Passerini G, Sabbadini MG (2014) Diagnostic value of IgG4 indices in IgG4-related hypertrophic pachymeningitis. J Neuroimmunol 266:82–86

    Article  CAS  PubMed  Google Scholar 

  8. Menon MP, Evbuomwan MO, Rosai J, Jaffe ES, Pittaluga S (2014) A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease? Histopathology 64:455–459

    Article  PubMed  Google Scholar 

  9. Zhu HD, Xie Q, Gong Y, Mao Y, Zhong P, Hang FP, Chen H, Zheng MZ, Tang HL, Wang DJ, Chen XC, Zhou LF (2013) Lymphoplasmacyte-rich meningioma: our experience with 19 cases and a systematic literature review. Int J Clin Exp Med 6:504–515

    PubMed Central  PubMed  Google Scholar 

  10. Lal A, Dahiya S, Gonzales M, Hiniker A, Prayson R, Kleinschmidt-DeMasters BK, Perry A (2014) IgG4 overexpression is rare in meningiomas with a prominent inflammatory component: a review of 16 cases. Brain Pathol 24:352–359

    Article  CAS  PubMed  Google Scholar 

  11. Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, Velazquez-Vega J, Borja MJ, Rosenberg S, Lossos A, Lossos IS (2014) Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore) 93:165–175

    Article  Google Scholar 

  12. Denis DJ, Elayoubi K, Weil AG, Berthelet F, Bojanowski MW (2013) Inflammatory myofibroblastic tumors of the central nervous system that express anaplastic lymphoma kinase have a high recurrence rate. Surg Neurol Int 4:70

    Article  PubMed Central  PubMed  Google Scholar 

  13. Yamamoto H, Yamaguchi H, Aishima S, Oda Y, Kohashi K, Oshiro Y, Tsuneyoshi M (2009) Inflammatory myofibroblastic tumor versus IgG4-related sclerosing disease and inflammatory pseudotumor: a comparative clinicopathologic study. Am J Surg Pathol 33:1330–1340

    Article  Google Scholar 

  14. Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ (2004) MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 25:880–891

    PubMed  Google Scholar 

  15. Zhu M, Yu BB, Zhai JL, Sun G (2016) Case of Langerhans cell histiocytosis that mimics meningioma in CT and MRI. J Korean Neurosurg Soc 59:165–167

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  16. Lachenal F, Cotton F, Desmurs-Clavel H, Haroche J, Taillia H, Magy N, Hamidou M, Salvatierra J, Piette JC, Vital-Durand D, Rousset H (2006) Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol 253:1267–1277

    Article  Google Scholar 

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Nambirajan, A., Sharma, M.C., Garg, K. et al. Large dural-based mass with bony hyperostosis in a 16-year-old male: IgG4-related disease mimicking lymphoplasmacyte-rich meningioma. Childs Nerv Syst 35, 1423–1427 (2019).

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