Primary low-grade diffuse small lymphocytic lymphoma of the central nervous system

• Published in: BMJ case reports Vol. 2014; p. bcr2013202051
• Main Authors: Aziz, Mehar, Chaurasia, Jai Kumar, Khan, Roobina, Afroz, Nishat
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 12.04.2014; BMJ Publishing Group
• Series: Case Report
• Subjects: Antigens; Asia; Brain Neoplasms - diagnosis; Brain Neoplasms - therapy; Chemotherapy; Edema; Female; Gangrene; Histology; Humans; Indian Sub-Continent; Leukemia, Lymphocytic, Chronic, B-Cell - diagnosis; Leukemia, Lymphocytic, Chronic, B-Cell - therapy; Lymphocytes; Lymphoma; Metastasis; Middle Aged; Nervous system; Neurotoxicity; Rare Disease; Tumors
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2013-202051

A 45-year-old Indian woman presented in neurosurgery outpatient with seizures, headache and vomiting for the past 1 month. MRI of the brain was suggestive of a malignant central nervous system (CNS) tumour. Histological and immunohistochemical examinations of stereotactic biopsy of the tumour were diagnostic of a low-grade diffuse small lymphocytic lymphoma of the CNS. No evidence of any occult systemic lymphoma was observed, confirming its ‘primary’ origin in the CNS. The diagnosis of a low-grade primary CNS lymphoma (PCNSL) is difficult as clinical and neuroradiological features are wide and variable. The clinical course is more indolent than a high-grade PCNSL and thus, a less aggressive and localised targeted treatment could be sufficient rather than the high dose, neurotoxic methotrexate-based chemotherapeutic treatment, recommended for high-grade PCNSL. Histological and immunohistological confirmation is therefore mandatory for early, appropriate treatment and prognostic implications.