Elsevier

Human Pathology

Volume 34, Issue 3, March 2003, Pages 253-262
Human Pathology

Original Contributions
Inflammatory pseudotumors of the central nervous system: Report of 3 cases and a literature review

https://doi.org/10.1053/hupa.2003.35Get rights and content

Abstract

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. Hum Pathol 34:253-262. Copyright 2003, Elsevier Inc. All rights reserved.

Section snippets

Patients and methods

Detailed clinical records were available for the 3 patients with IP-CNS who had been treated at the University Hospital RWTH Aachen between 1996 and 2001. One patient was suffering from an additional pulmonary IP. To begin, 7-μm-thick sections from formalin-fixed and paraffin-embedded tissue specimens were stained for hematoxylin and eosin and Elastica-van Gieson according to routine methods. Immunohistochemistry was also performed according to routine methods with peroxidase/DAB or alkaline

Patient 1

In this boy, a left thoracic IP had necessitated left-sided pneumonectomy at age 2 years. Six years later he suffered a right-sided seizure due to a round, calcified non-contrast-enhanced lesion within the left temporal lobe. During surgery, a solid, not well-delineated tumor infiltrating the surrounding brain tissue was found and resected incompletely. After surgery, no radiation or chemotherapy was performed. Postsurgical magnetic resonance imaging (MRI) revealed a small residual left

Discussion

IP-CNS represent a diagnostic and clinical challenge. Due to their nonspecific neuroradiologic and clinical findings,4 the correct diagnosis depends on the postsurgical histopathologic examinations and on the pathologist's awareness of this rare disease. The possibility of IP should be considered in all intracranial tumor-like lesions composed of a lymphoplasmacellular infiltrate and a collagenous stroma of varying density, regardless of additional features such as giant cells, calcifications,

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