DOI QR코드

DOI QR Code

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

  • Yim, Jeemin (Department of Pathology, Seoul National University Hospital) ;
  • Song, Seung Geun (Department of Pathology, Seoul National University Hospital) ;
  • Kim, Sehui (Department of Pathology, Seoul National University Hospital) ;
  • Choi, Jae Won (Department of Radiology, Seoul National University Hospital) ;
  • Lee, Kyu-Chong (Department of Radiology, Korea University Guro Hospital) ;
  • Bae, Jeong Mo (Department of Pathology, Seoul National University Hospital) ;
  • Jeon, Yoon Kyung (Department of Pathology, Seoul National University Hospital)
  • Received : 2018.07.23
  • Accepted : 2018.08.21
  • Published : 2019.01.15

Abstract

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.

References

  1. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haemotopoietic and lymphoid tissues. Revised 4th ed. Lyon: International Agency for Research on Cancer, 2017.
  2. Shenkier TN, Blay JY, O'Neill BP, et al. Primary CNS lymphoma of T-cell origin: a descriptive analysis from the international primary CNS lymphoma collaborative group. J Clin Oncol 2005; 23: 2233-9. https://doi.org/10.1200/JCO.2005.07.109
  3. Lim T, Kim SJ, Kim K, et al. Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes. Ann Hematol 2011; 90: 1391-8. https://doi.org/10.1007/s00277-011-1225-0
  4. Menon MP, Nicolae A, Meeker H, et al. Primary CNS T-cell Lymphomas: a clinical, morphologic, immunophenotypic, and molecular analysis. Am J Surg Pathol 2015; 39: 1719-29. https://doi.org/10.1097/PAS.0000000000000503
  5. Nael A, Walavalkar V, Wu W, et al. CD4-positive T-cell primary central nervous system lymphoma in an HIV positive patient. Am J Clin Pathol 2016; 145: 258-65. https://doi.org/10.1093/ajcp/aqv087
  6. Guzzetta M, Drexler S, Buonocore B, Donovan V. Primary CNS Tcell lymphoma of the spinal cord: case report and literature review. Lab Med 2015; 46: 159-63. https://doi.org/10.1309/LM85GPXDT8NJSDAB
  7. Flanagan EP, O'Neill BP, Porter AB, Lanzino G, Haberman TM, Keegan BM. Primary intramedullary spinal cord lymphoma. Neurology 2011; 77: 784-91. https://doi.org/10.1212/WNL.0b013e31822b00b9
  8. Yang W, Garzon-Muvdi T, Braileanu M, et al. Primary intramedullary spinal cord lymphoma: a population-based study. Neuro Oncol 2017; 19: 414-21.
  9. Tripodo C, Iannitto E, Florena AM, et al. Gamma-delta T-cell lymphomas. Nat Rev Clin Oncol 2009; 6: 707-17. https://doi.org/10.1038/nrclinonc.2009.169
  10. Mooney KL, Choy W, Woodard J, et al. Primary central nervous system gamma delta cytotoxic T-cell lymphoma. J Clin Neurosci 2016; 26: 138-40. https://doi.org/10.1016/j.jocn.2015.10.017
  11. Arnulf B, Copie-Bergman C, Delfau-Larue MH, et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood 1998; 91: 1723-31.
  12. Garcia-Herrera A, Song JY, Chuang SS, et al. Nonhepatosplenic gammadelta T-cell lymphomas represent a spectrum of aggressive cytotoxic T-cell lymphomas with a mainly extranodal presentation. Am J Surg Pathol 2011; 35: 1214-25. https://doi.org/10.1097/PAS.0b013e31822067d1
  13. Choe JY, Bisig B, de Leval L, Jeon YK. Primary gammadelta T cell lymphoma of the lung: report of a case with features suggesting derivation from intraepithelial gammadelta T lymphocytes. Virchows Arch 2014; 465: 731-6. https://doi.org/10.1007/s00428-014-1623-5
  14. Kucuk C, Jiang B, Hu X, et al. Activating mutations of STAT5B and STAT3 in lymphomas derived from gammadelta-T or NK cells. Nat Commun 2015; 6: 6025. https://doi.org/10.1038/ncomms7025
  15. Roberti A, Dobay MP, Bisig B, et al. Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations. Nat Commun 2016; 7: 12602. https://doi.org/10.1038/ncomms12602