- Physician, Intensive Care Unit, Fundación Valle del Lili, Cali, Colombia
- Physician, CIDEIM, International Biomedical Research Center, Cali, Colombia
- Pathologist, Fundación Valle del Lili, Professor of Pathology, Universidad ICESI, Fundación Valle del Lili, Cali, Colombia
- Neurosurgeon, Fundación Valle del Lili, Professor of Neuroanatomy and Neurosurgery, Universidad ICESI, Cali, Colombia
Neurosurgeon, Fundación Valle del Lili, Professor of Neuroanatomy and Neurosurgery, Universidad ICESI, Cali, Colombia
DOI:10.4103/2152-7806.189732Copyright: © 2016 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Quinones E, Potes LI, Silva N, Lobato-Polo J. Lymphomatoid granulomatosis of the brain: A case report. Surg Neurol Int 01-Sep-2016;7:
How to cite this URL: Quinones E, Potes LI, Silva N, Lobato-Polo J. Lymphomatoid granulomatosis of the brain: A case report. Surg Neurol Int 01-Sep-2016;7:. Available from: http://surgicalneurologyint.com/surgicalint_articles/lymphomatoid-granulomatosis-brain-case-report/
Background:Lymphomatoid granulomatosis is a rare disorder of the central nervous system (CNS) with few cases being reported in literature. We present the case of an adult with an unusual lesion of the CNS who presented with motor seizures and was diagnosed with lymphomatoid granulomatosis, followed by a discussion of the process of evaluation and management.
Case Description:A 42-year-old male presented with motor seizures and loss of consciousness for 10 minutes along with dysarthria and left hemiplegia. Neurological examination and imaging with magnetic resonance imaging (MRI) of the brain revealed a mass in the right striatum. The patient was hospitalized and underwent an image-guided right frontal craniotomy using the Leksell Stereotactic G-Frame. Pathology reported a lymphomatoid granulomatosis. Being immunocompetent, the patient received medical treatment with prednisone and rituximab. Two years after his diagnosis, the patient had no active disease and his brain MRI did not show contrast enhancement. After almost 3 years of follow-up, the patient has a mild weakness in the left-side of his body (4/5), is seizure-free, and can walk and perform daily activities.
Conclusions:This rare lesion in an adult, immunocompetent patient, debuting with motor seizures represents a challenge in terms of diagnosis and treatment. After surgical and medical treatment, the patient had a satisfactory recovery. Clinical features, imaging, differential diagnosis, and pathology are discussed.
Keywords: Brain tumor, lymphomatoid granulomatosis, stereotactic neurosurgery, symptomatic epilepsy
Lymphomatoid granulomatosis (LG) is a disease that was first described in 1972 by Liebow et al.,[
Because there is still much to be learned about this disease, we present the following case along with a detailed description of its radiological, pathological, and clinical findings.
A 42-year-old man, previously healthy, was living with his step father and other members of an extended family in a small farm. He presented with a first episode of a motor seizure that started on the left side of his body before becoming generalized. Shortly afterwards, he noticed left hemiparesis and dysarthria; he was admitted to our institution through the emergency department.
After a physical examination, magnetic resonance imaging (MRI) of the patient revealed a well-defined, spherical lesion, located in the superior aspect of the anterior limb of the internal capsule and right striatum, with surrounding edema [
Magnetic resonance T1 image. A round-shaped, well-limited lesion, with contrast enhancement, located in the right striatum besides the the lateral ventricle with compressive effect on the brain parenchyma and adjacent structures deviation midline approximately 8.0 mm. Surrounding edema is observed
In the pathological analysis, there was an evident atypical T and B infiltrate; morphological and phenotypical characteristics of Grade 1 lymphomatoid granulomatosis. The patient was subjected to thoracic and abdominal screening, which revealed paratracheal, jugular, and inguinal adenopathies, but no other masses.
After consulting with the hematology group, the patient received a four-cycle medical treatment with rituximab and prednisone. Clinically, he recovered almost completely with strength of 4/5 and complete reintegration to his daily activities, which involved bimanual work. Six months after his diagnosis, a new MRI showed the absence of new or residual lesions.
Two years after the surgery the patient continued to be free of seizures, and his MRI showed no evidence of new lesions, areas of restriction of diffusion, or anomalous enhancements that could indicate residual or recurrent tumor [
LG is a lymphoproliferative disorder, in which tumorous B cells transform after infection with the Epstein-Barr virus.[
The pathogenesis of LG is unknown. Studies based on clinicopathological, immunophenotypic, and clonal evidence have shown that this is a unique type of extranodal, malignant lymphoma.[
LG can be graded based on the relative amount of tumor reactive cells and accompanying tumor cells.[
Radiological, lymphomatoid granulomatosis (LG) appearance in a brain computed tomography (CT) varies. It is common to find intraparenchymal lesions, which may be unilateral or multifocal, supra or infratentorial. These lesions may appear as low-density areas within the cerebral white matter; they may be solid or ring-like, sometimes with accompanying edema.[
Because of its angiocentric and angiodestructive character, LG can debut with intraparenchymal hemorrhage, stroke, or aneurysms.[
LG and CNS lymphoma share similar radiological features that may be difficult to distinguish on an MRI or CT image [
Our patient, unlike what literature reports [
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