Intracranial Inflammatory Pseudotumors Associated with Immunoglobulin G4-Related Disease Mimicking Multiple Meningiomas: A Case Report and Review of the Literature

Okano, Atsushi; Nakatomi, Hirofumi; Shibahara, Junji; Tsuchiya, Tsukasa; Saito, Nobuhito

doi:10.1016/j.wneu.2015.02.011

Cited by 22 publications

(17 citation statements)

References 26 publications

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“…Hypertrophic pachymeningitis usually exhibits a diffusely invasive pattern, but rarely demonstrates a focal mass as in the present case or in a few other case reports [2][3][4][5]. When hypertrophic pachymeningitis reveals a focal mass, it is difficult to distinguish from a tumor, such as meningioma and/or solitary fibrous tumor, preoperatively [2,4,5]. Tang et al reported a case of clivus massive IgG4-related disease similar to the present case [5].…”

supporting

confidence: 58%

“…Glucocorticoid treatment was not performed in their case since the mass was soft and totally removed via surgery; and the follow-up period was not described [5]. The effectiveness of glucocorticoid treatment for IgG4-related hypertrophic pachymeningitis has been noted in previous case reports [3,4], and we also obtained a favorable outcome with remarkable shrinkage of the mass lesion and improvement of the palsies of the bilateral abducens nerves following glucocorticoid treatment. It is important therefore to perform surgery with the primary purpose of diagnosis to avoid complications, when we suspect IgG4-related disease for a skull base mass since it displays a good reaction for glucocorticoid treatment and in some cases, like the present one, can be completely cured.…”

mentioning

confidence: 61%

“…IgG4-related hypertrophic pachymeningitis is being increasingly recognized, but a consensus concerning the optimal diagnostic approach and therapeutic approach remains lacking [1]. Hypertrophic pachymeningitis usually exhibits a diffusely invasive pattern, but rarely demonstrates a focal mass as in the present case or in a few other case reports [2][3][4][5]. When hypertrophic pachymeningitis reveals a focal mass, it is difficult to distinguish from a tumor, such as meningioma and/or solitary fibrous tumor, preoperatively [2,4,5].…”

mentioning

confidence: 89%

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Treatment-responsive case of focal clivus IgG4-related hypertrophic pachymeningitis mimicking meningioma; case report

et al. 2021

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confidence: 58%

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confidence: 61%

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confidence: 89%

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Treatment-responsive case of focal clivus IgG4-related hypertrophic pachymeningitis mimicking meningioma; case report

et al. 2021

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“…Although involvement of the nervous system appears to be relatively rare, a range of sites have been noted including involvement of the meninges (mimicking meningioma), ventricles, parietotemporal parenchyma, pituitary gland, cranial nerves and spinal cord [87–94]. Symptoms typically arise due to the compressive nature of these lesions and therefore depend on their location.…”

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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Opinion statementIgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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“…Intracranial lesions can result from IgG4-RD, and generally present as IgG4-related hypophysitis or IgG4-related pachymeningitis, although a small number of case reports have also described intracranial inflammatory pseudotumors. 31 Since 2004, there have been 39 reported cases of IgG4-related hypophysitis: 29 men and 10 women, with a mean age of 68 years. Patients with IgG4-related hypophysitis tend to be older men unlike patients with IgG4-related ophthalmic disease (IgG4-OD; as described below).…”

Section: Type 1 Autoimmune Pancreatitismentioning

confidence: 99%

The Geoepidemiology and Clinical Aspects of IgG4-Related Disease

et al. 2016

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Immunoglobulin G4-related disease (IgG4-RD) is a recently described systemic inflammatory disease characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis. However, although IgG4-RD has become increasingly recognized, the number of patients with IgG4-RD remains unclear. Data from several studies indicate that patients who have a T-helper type 2 (Th2-) dominant immune response, which leads to the hyperproduction of Th2 cytokines, then progress to IgG4-RD. Glucocorticoids are the most common treatment for IgG4-RD and generally, patients have a good response-a characteristic of IgG4-RD. However, relapses during the tapering of glucocorticoid therapy are common. Second-line therapy after glucocorticoids includes immunosuppressant agents. Although the long-term outcome still remains unclear, there is increased interest in the relationships between IgG-RD and malignancies. In this review, the authors provide a detailed overview of the geoepidemiology, pathogenesis, diagnostic features, treatment, and prognosis of IgG4-RD.

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Intracranial Inflammatory Pseudotumors Associated with Immunoglobulin G4-Related Disease Mimicking Multiple Meningiomas: A Case Report and Review of the Literature

Cited by 22 publications

References 26 publications

Treatment-responsive case of focal clivus IgG4-related hypertrophic pachymeningitis mimicking meningioma; case report

Treatment-responsive case of focal clivus IgG4-related hypertrophic pachymeningitis mimicking meningioma; case report

Neurological Manifestations of IgG4-Related Disease

The Geoepidemiology and Clinical Aspects of IgG4-Related Disease

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