Primary Peripheral Gamma Delta T-Cell Lymphoma of the Central Nervous System: Report of a Case Involving the Intramedullary Spinal Cord and Presenting with Myelopathy

• Published in: Journal of pathology and translational medicine Vol. 53; no. 1; pp. 57 - 61
• Main Authors: Yim, Jeemin, Song, Seung Geun, Kim, Sehui, Choi, Jae Won, Lee, Kyu-Chong, Bae, Jeong Mo, Jeon, Yoon Kyung
• Format: Journal Article
• Language: English
• Published: Korea (South) The Korean Society of Pathologists and the Korean Society for Cytopathology 01.01.2019; Korean Society of Pathologists & the Korean Society for Cytopathology; 대한병리학회
• Subjects: Case Study; peripheral T-cell lymphoma; primary central nervous system lymphoma; primary intramedullary spinal cord lymphoma; γδ T-cell lymphoma; 병리학; peripheral T-cell lymphoma; γδ T-cell lymphoma; primary intramedullary spinal cord lymphoma; primary central nervous system lymphoma
• ISSN: 2383-7837; 2383-7845
• DOI: 10.4132/jptm.2018.08.21

Primary central nervous system lymphoma of T-cell origin (T-PCNSL) is rare, and its clinicopathological features remain unclear. Peripheral T-cell lymphoma of γδ T-cell origin is an aggressive lymphoma mainly involving extranodal sites. Here, we report a case of γδ T-PCNSL involving the intramedullary spinal cord and presenting with paraplegia. A 75-year-old Korean woman visited the hospital complaining of back pain and lower extremity weakness. Magnetic resonance imaging revealed multifocal enhancing intramedullary nodular lesions in the thoracic and lumbar spinal cord. An enhancing nodular lesion was observed in the periventricular white matter of the lateral ventricle in the brain. There were no other abnormalities in systemic organs or skin. Laminectomy and tumor removal were performed. The tumor consisted of monomorphic, medium-to-large atypical lymphocytes with pale-to-eosinophilic cytoplasm. Immunohistochemically, the tumor cells were CD3(+), TCRβF1(-), TCRγ(+), CD30(-), CD4(-), CD8(-), CD56(+), TIA1(+), granzyme B(+), and CD103(+). Epstein-Barr virus in situ was negative. This case represents a unique T-PCNSL of γδ T-cell origin involving the spinal cord.