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

Published on May 9, 2019in Childs Nervous System 1.33
· DOI :10.1007/s00381-019-04187-z
Aruna Nambirajan4
Estimated H-index: 4
M. Chand Sharma + 3 AuthorsVivek Suri1
Estimated H-index: 1
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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Published on Apr 1, 2017in Current Treatment Options in Neurology 3.57
Bernardo Baptista1
Estimated H-index: 1
Alina Casian6
Estimated H-index: 6
(Guy's and St Thomas' NHS Foundation Trust)
+ 2 AuthorsClaire M Rice2
Estimated H-index: 2
(Southmead Hospital)
IgG4-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 IgG...
Published on Dec 1, 2016in Journal of Neurosurgery
Bishan Dass Radotra19
Estimated H-index: 19
Ashish Aggarwal20
Estimated H-index: 20
+ 2 AuthorsDebajyoti Chatterjee4
Estimated H-index: 4
IgG4-related disease is relatively new disease entity and a rare one, and our knowledge of this entity continues to evolve. It was first described in the pancreas and since then has been described in virtually every organ. Spinal involvement resulting in pachymeningitis is rare, and there are only 8 reported cases of the same to date, with the cervicothoracic spine being the most commonly affected region. The authors describe 2 cases in which the patients presented with spinal compression result...
Published on Oct 1, 2016in Joint Bone Spine 3.28
Gyuhwan Hwang1
Estimated H-index: 1
(SCH: Soonchunhyang University),
Sy Jin12
Estimated H-index: 12
(SCH: Soonchunhyang University),
Hyun-Sook Kim4
Estimated H-index: 4
(SCH: Soonchunhyang University)
Joint Bone Spine - In Press.Proof corrected by the author Available online since vendredi 8 avril 2016
Published on Jan 1, 2016in Journal of Korean Neurosurgical Society 1.19
Ming Zhu1
Estimated H-index: 1
Bing-bing Yu1
Estimated H-index: 1
+ 1 AuthorsGang Sun1
Estimated H-index: 1
Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on under...
Published on Jul 1, 2014in Brain Pathology 6.16
Aseem Lal4
Estimated H-index: 4
(UCSF: University of California, San Francisco),
Sonika Dahiya22
Estimated H-index: 22
(WashU: Washington University in St. Louis)
+ 4 AuthorsArie Perry76
Estimated H-index: 76
(UCSF: University of California, San Francisco)
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...
Published on Mar 1, 2014in Medicine 1.87
Jose D. Sandoval-Sus4
Estimated H-index: 4
(UM: University of Miami),
Ana Sandoval-Leon2
Estimated H-index: 2
(UM: University of Miami)
+ 5 AuthorsIzidore S. Lossos48
Estimated H-index: 48
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is an uncommon benign idiopathic lymphoproliferative disorder. The histologic hallmark of RDD is the finding of emperipolesis displayed by lesional histiocytes. While RDD most commonly affects lymph nodes, extranodal involvement of multiple organs has been reported, including the central nervous system (CNS). However, CNS involvement in RDD is rare and is not well characterized. As a result, thera...
Published on Feb 1, 2014in Histopathology 3.29
Madhu P Menon5
Estimated H-index: 5
(NIH: National Institutes of Health),
Moses O. Evbuomwan7
Estimated H-index: 7
(NIH: National Institutes of Health)
+ 2 AuthorsStefania Pittaluga73
Estimated H-index: 73
(NIH: National Institutes of Health)
Published on Jan 1, 2014in Journal of Neuroimmunology 2.83
Emanuel Della-Torre13
Estimated H-index: 13
(UniSR: Vita-Salute San Raffaele University),
Laura Galli1
Estimated H-index: 1
+ 7 AuthorsMaria Grazia Sabbadini35
Estimated H-index: 35
(UniSR: Vita-Salute San Raffaele University)
Abstract Diagnosis of IgG4-Related Hypertrophic Pachymeningitis (IgG4-HP) relies on meningeal biopsies, because cerebrospinal fluid (CSF) diagnostic biomarkers are lacking. Here, we determined whether IgG4 intrathecal production could distinguish IgG4-HP from other disorders presenting with HP (OHP). In patients with IgG4-HP, the median CSF IgG4 concentration, IgG4 Index and IgG4 Loc were significantly higher than in both controls and OHP. CSF IgG4 levels higher than 2.27 mg/dL identified 100% o...
Published on Dec 1, 2013in BMC Surgery 1.77
Che-Kuang Lin1
Estimated H-index: 1
(Memorial Hospital of South Bend),
Dar-Ming Lai1
Estimated H-index: 1
(NTU: National Taiwan University)
Background Immunoglobulin G4 (IgG4)-related disease is a systemic syndrome, characterized by sclerosing lesions and usually associated with a raised serum IgG4 level; the pancreas, salivary glands, and lacrimal glands are typically affected. Recently, it has been suggested that IgG4-related sclerosing disease represents a subset of cases previously diagnosed as idiopathic hypertrophic pachymeningitis. This rare inflammatory disorder causes localized or diffused thickening of intracranial dura ma...
Published on Jul 1, 2013in Medicine 1.87
Zachary S. Wallace17
Estimated H-index: 17
Mollie N. Carruthers15
Estimated H-index: 15
+ 5 AuthorsJohn H. Stone67
Estimated H-index: 67
Hypertrophic pachymeningitis (HP) is an inflammatory condition in which the dura mater of the cranium or spine becomes thickened, leading to symptoms that result from mass effect, nerve compression, or vascular compromise. The differential diagnosis of HP includes immune-mediated conditions such as rheumatoid arthritis and vasculitis, malignancies, and infections. Many times, no diagnosis is reached; in such cases, the disease has been described as idiopathic HP. IgG4-related disease (IgG4-RD) i...
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