Article (Scientific journals)
Inflammatory pseudotumors of the central nervous system: Report of 3 cases and a literature review
Häusler, M.; Schaade, L.; Ramaekers, Vincent et al.
2003In Human Pathology, 34 (3), p. 253-262
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Keywords :
Brain neoplasm; Inflammatory disease; Inflammatory pseudotumor; Adolescent; Antibodies, Viral; Brain Diseases; Brain Neoplasms; Cell Transformation, Neoplastic; Child, Preschool; Dura Mater; Female; Frontal Lobe; Granuloma, Plasma Cell; Humans; Immunoglobulin G; Magnetic Resonance Imaging; Male; Occipital Lobe; Recurrence; Simplexvirus; Temporal Lobe
Abstract :
[en] Inflammatory pseudotumors (IPs), mostly benign lesions characterized by fibrotic ground tissue and polyclonal mononuclear infiltrate, may affect all organ systems. IPs originating in the central nervous system (IP-CNS) are very rare, and their distinct histopathologic features are poorly characterized. Three otherwise healthy patients (age 8, 15, and 17 years) presented with focal neurologic symptoms (seizures, n = 2; headaches, n = 1), corresponding to a left temporal, left occipital, and left frontal IP, respectively, extending from meningeal structures into brain tissue. After resection, no recurrence was observed in patient 1 during 5 years of follow-up, whereas patient 2 developed a rapidly progressive local recurrence and a second intracerebral lesion despite antiviral, immunosuppressive, antibiotic, and radiation therapy. In patient 3, who also showed local recurrences, sequential histopathologic investigations revealed transformation to a semimalignant fibrohistiocytic tumor. In this patient, anaplastic lymphoma kinase (ALK) expression was also positive, whereas it was negative in patient 1. A detailed literature analysis confirmed that most IP-CNS arise from dural/meningeal structures (n = 34). Intraparenchymatous (n = 7), mixed intraparenchymatous/meningeal (n = 4), and intraventricular lesions (n = 7) or IP extending per continuitatem from intracerebral to extracerebral sites (n = 5) were rare. The recurrence rate was 40% within 2 years in general. It was increased after incomplete resection and in female patients (multivariate Cox regression model, P < 0.02). Although rare, IP-CNS are important differential diagnoses among tumor-like intracranial lesions. Their potential risk of malignant transformation and high risk of recurrence necessitate close follow-up, especially when resection is incomplete. Prospective multicenter trials are needed to optimize classification and treatment of this rare inflammatory lesion. Copyright 2003, Elsevier Inc. All rights reserved.
Disciplines :
Neurology
Pediatrics
Author, co-author :
Häusler, M.;  Department of Pediatrics, University Hospital, RWTH Aachen, Pauwelsstr. 30, 52074 Aachen, Germany
Schaade, L.;  Rheinisch - Westfälische Technische Hochschule Aachen - RWTH
Ramaekers, Vincent ;  Rheinisch - Westfälische Technische Hochschule Aachen - RWTH > Pediatrics
Doenges, M.;  Rheinisch - Westfälische Technische Hochschule Aachen - RWTH
Heimann, G.;  Rheinisch - Westfälische Technische Hochschule Aachen - RWTH
Sellhaus, B.;  Rheinisch - Westfälische Technische Hochschule Aachen - RWTH
Language :
English
Title :
Inflammatory pseudotumors of the central nervous system: Report of 3 cases and a literature review
Publication date :
March 2003
Journal title :
Human Pathology
ISSN :
0046-8177
eISSN :
1532-8392
Publisher :
W. B. Saunders Co., United Kingdom
Volume :
34
Issue :
3
Pages :
253-262
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 07 June 2014

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