Intracranial mass‐forming lesion associated with dural thickening and hypophysitis

Kanno, Hiromi; Tanino, Mishie; Watanabe, Kentaro; Ozaki, Yoshimaru; Itoh, Tamio; Kimura, Tohru; Nishihara, Hiroshi; Itoh, Tomoo; Narita, Takuhito; Nagashima, Kazuo; Tanaka, Shinya

doi:10.1111/j.1440-1789.2012.01342.x

Cited by 3 publications

(3 citation statements)

References 9 publications

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“…15,16,23 Our first case contributes to the few published cases in the literature of intracranial IgG4-related inflammatory pseudotumors that mimic meningioma on imaging. Nishino et al 7 and Kanno et al 25 were the first to describe supratentorial meningioma-like lesions. Lal et al report a case series of 16 inflammatory meningiomas in which two cases demonstrated tumors with >10 IgG4-positive plasma cells/hpf and IgG4/IgG ratio was focally increased in two cases up to 31%.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related intracranial disease

et al. 2018

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IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel's caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.

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Section: Discussionmentioning

confidence: 99%

IgG4-related intracranial disease

et al. 2018

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“…Remarkably, in all instances, there were no other previous clinical manifestations of IgG4-related disease. Only few cases with tumor-like intracranial lesions as a manifestation of IgG4-related hypertrophic pachymeningitis have been published previously [ 7 , 13 , 15 – 17 , 25 , 26 ]. It is important to obtain a definitive histopathological diagnosis in such patients in order to avoid delayed and unnecessary treatment.…”

Section: Discussionmentioning

confidence: 99%

IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions

et al. 2022

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Objective IgG4-related hypertrophic pachymeningitis is a rare fibroinflammatory disorder that may cause localized or diffused thickening of the dura mater. Misinterpretations of the clinical and imaging findings are common. Clinical manifestations depend on the location of the inflammatory lesion and on compression of neural structures leading to functional deficits. A dural biopsy is commonly needed for a definitive diagnosis. Immunomodulatory therapy is considered the therapy of choice. Methods Four patients with IgG4-related hypertrophic pachymeningitis were identified over a 5-year period. Patient-related characteristics including age, preoperative workup, signs and symptoms of patients, and diagnostic procedures were evaluated. Furthermore, the surgical treatment and 5-year follow-up outcomes were analyzed. Results There were two adults and two adolescents (mean age 32 years; range 15 to 67 years). Two patients were male, and two were female. No history of disease was known in any of the patients. Clinical symptoms were epilepsy (n = 2), ataxia and nausea (n = 1), and facial nerve palsy (n = 1). MR imaging studies showed contrast enhancing lesions in the temporal region in two patients, and in the cerebellar region in the other two patients. Subtotal resection was performed in two instances and a biopsy via a suboccipital retrosigmoid approach was obtained in the other two patients. Histochemical and immunohistochemical investigations revealed an IgG 4 disease in all of these patients. Immunomodulatorry therapy led to clinical stability during follow-up of 5 years in all four cases. Conclusion The diagnosis of IgG4-related hypertrophic pachymeningitis is challenging, but is of great relevance as treatment differs significantly from other forms of pachymeningitis and a specific therapeutic approach may avoid long-term neurological complications. Our series contributes to a better clinical characterization of this rare disease.

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“…They therefore concluded that a subset of dural‐based chronic inflammatory lesions previously considered idiopathic might, in fact, represent IgG4‐related meningeal disease. A review of the literature on CNS involvement by this disease also reveals isolated case reports of IgG4 disease presenting as hypophysitis , pachymeningitis with or without dural mass , spinal cord involvement , or an orbital mass .…”

Section: Introductionmentioning

confidence: 99%

IgG4 Overexpression Is Rare in Meningiomas with a Prominent Inflammatory Component: A Review of 16 Cases

et al. 2014

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Meningiomas with prominent inflammation are traditionally classified as "lymphoplasmacyte-rich meningioma" (LPM). Both inflammatory and neoplastic meningeal proliferations have recently been linked to IgG4 disease, although a potential association with LPM has not been previously explored. Sixteen meningiomas with inflammatory cells outnumbering tumor cells were further characterized by CD3, CD20, CD68 and/or CD163, CD138, kappa, lambda, IgG and IgG4 immunostains. There were 11 female and 4 male patients, ranging from 22 to 78 (median 59) years of age. Tumors consisted of 10 World Health Organization (WHO) grade I, 5 grade II and 1 grade III LPMs. Immunohistochemically, the most numerous cell type was the macrophage in all cases followed by CD3-positive T cells and fewer CD20-positive B cells. Plasma cells ranged from moderate-marked (N = 5) to rare (N = 7), or absent (N = 4). Maximal numbers of IgG4 plasma cells per high power field (HPF) ranged from 0 to 32, with only two cases having counts exceeding 10/HPF. The IgG4/IgG ratio was increased focally in only two cases (30% and 31%). Additionally, plasma cells represented only a minor component in most examples, whereas macrophages predominated, suggesting that "inflammation-rich meningioma" may be a more accurate term. The inflammatory stimulus for most cases remains to be elucidated.

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Intracranial mass‐forming lesion associated with dural thickening and hypophysitis

Cited by 3 publications

References 9 publications

IgG4-related intracranial disease

IgG4-related intracranial disease

IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions

IgG4 Overexpression Is Rare in Meningiomas with a Prominent Inflammatory Component: A Review of 16 Cases

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