"Slow and Steady" Infiltrates the Brain: An Autopsy Report of Lymphomatosis Cerebri

• Published in: Neurology India Vol. 67; no. 6; pp. 1504 - 1508
• Main Authors: Gupta, Kirti, Gupta, Vivek, Radotra, Bishan, Tewari, Manoj
• Format: Journal Article
• Language: English
• Published: India Wolters Kluwer India Pvt. Ltd 01.11.2019; Medknow Publications & Media Pvt. Ltd
• Subjects: Edema; Encephalitis; Histology; Lymphoma; Metabolism; the term which has been deleted from the recent WHO classification; primary CNS lymphomaKey Message: Lymphomatosis cerebri is a rare form of primary CNS lymphoma characterized by neoplastic cells which diffusely percolate through the white matter instead of forming cohesive masses. Such a pattern of infiltration is seen with Gliomatosis cerebri; lymphomatosis cerebri; Autopsy; primary CNS lymphoma
• ISSN: 0028-3886; 1998-4022
• DOI: 10.4103/0028-3886.273648

Primary central nervous system lymphomas (PCNSL) usually present as single or multiple lesions with mass effect involving the cerebral hemispheres or basal ganglia. An extremely rare pattern of involvement termed "Lymphomatosis cerebri" (LC) presents as diffuse, non-enhancing infiltrative lesions without mass effect. We describe the clinical, radiological, and autopsy findings of one such rare example with a patient presenting with a short history of fever, memory loss, and progressive cognitive decline. Because of subtle yet rapidly progressive clinical symptoms and overlapping neuroimaging features, the diagnosis of LC is challenging with wide ranging differential diagnoses. The dilemma in diagnosing such lesions can lead to delay in diagnosis and institution of appropriate management. Thus, knowledge about its imaging and morphological features is very critical for correct categorization and to avoid potential misdiagnosis of this often-missed disease.