Lymphomatoid granulomatosis of the brain: A case report

Abstract

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.

Isolated lymphomatoid granulomatosis of the central nervous system: A case report and literature review

“…The last search through PubMed was conducted on December 1, 2018. The publication times of the literature on iCNS‐LYG cases ranged from 1984 to 2018, including 37 articles and 48 patients . The total number of cases was 49, including the one case reported by us in this study.…”

“…Some researchers suggested that steroid therapy alone could be administered to patients with grade I LYG, steroids and other chemotherapeutic drugs should be used in combination for patients with grade II LYG, and radiotherapy and chemotherapy should be used for patients with grade III LYG, as used in the treatment for lymphoma. In addition, there have been reports of successful treatment using rituximab . Although interferon‐α‐2b could be used to regulate the host's immune system in cases of grades 1–2 systemic LYG and the specific dosage and course of treatment were also provided in the literature, it was not used in any of the iCNS‐LYG cases reviewed.…”

Systematic Review of Spinal Lymphomatoid Granulomatosis Cases

“…There is no standard treatment protocol for systemic LYG and isolated CNS LYG, 1 , 2 , 23 and drug treatments, such as systemic steroids, rituximab, and several chemotherapies, vary widely. 23 There are reports on the usefulness of rituximab for LYG including CNS LYG, 9 , 24 , 25 although it is not always effective. 24 Interferon-α was useful for low-grade LYG treatment including cases involving the CNS, and cyclophosphamide and prednisone were expected for high-grade LYG treatment.…”

Primary Central Nervous System Involvement in Peripheral T-Cell Lymphoma: A Case Report

“…Meanwhile, of all PCNSL, intravascular lymphoma and lymphomatoid granulomatosis of the CNS may have similar radiological features; pathological findings are needed to distinguish these diseases ( 8 ). Intravascular lymphoma is a subtype of extranodal diffuse large B-cell lymphoma and lymphomatoid granulomatosis is a lymphoproliferative disorder, in which tumorous B cells transform after infection with the EBV ( 9 ). In our case, based on the expression of the T-cell specific marker and negative results of molecular analysis for the EBV, two diseases can be ruled out.…”