J Pathol Transl Med.  2019 Jan;53(1):57-61. 10.4132/jptm.2018.08.21.

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 J
• Song SG
• Kim S
• Choi JW
• Lee KC
• Bae JM
• Jeon YK

• KMID: 2437579
• DOI: http://doi.org/10.4132/jptm.2018.08.21

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.

Keyword

Primary central nervous system lymphoma; Primary intramedullary spinal cord lymphoma; Peripheral T-cell lymphoma; γδ T-cell lymphoma

MeSH Terms

Aged
Back Pain
Brain
Central Nervous System*
Cytoplasm
Female
Granzymes
Herpesvirus 4, Human
Humans
Laminectomy
Lateral Ventricles
Lower Extremity
Lymphocytes
Lymphoma
Lymphoma, T-Cell*
Lymphoma, T-Cell, Peripheral
Magnetic Resonance Imaging
Paraplegia
Skin
Spinal Cord Diseases*
Spinal Cord*
T-Lymphocytes*
White Matter
Granzymes

Figure

• 

  Fig. 1. Spine and brain magnetic resonance imaging (MRI) features at presentation (A, C–E) and 6 weeks later (B, F–K). (A) Spine MRI sagittal view revealed enhancing intramedullary nodular lesions at T9/10 and T11 levels (arrows). (B) Six weeks later, enhancing intramedullary nodular lesions (arrows) at T7/8, T9/10, and T11 levels were enlarged, and an enhancing nodule appeared at L5 level with leptomeningeal enhancement. (C–E) A nodular lesion (arrows) was observed in the periventricular white matter of the left lateral ventricle, which showed enhancement in T1 enhanced image (D) compared to T1 weighted image (C) and heterogeneous high signal intensity in T2 weighted images (E). (F–H) After 6 weeks, the lesion (arrows) increased in size with surrounding edema in T1 weighted (F), T1 enhanced (G), and T2 weighted images (H). (I, J ) Diffusion weighted images revealed diffusion restriction within the tumor with high signal intensity (arrow) (I) and corresponding low signal intensity (arrow) on the apparent diffuse coefficient map (J). (K) There were also multiple newly developed enhancing nodules in the lateral subependymal lining, left frontal lobe, and right temporal lobe (arrow).

• 

  Fig. 2. Histologic features of γδ T-cell lymphoma involving the spinal cord. (A) Monomorphic medium-to-large atypical lymphoid cells diffusely infiltrating the spinal cord parenchyma with occasional perivascular arrangement. (B) Atypical lymphoid cells showed clear to eosinophilic cytoplasm with distinct cell borders and hyperchromatic nuclei with small indistinct nucleoli.

• 

  Fig. 3. Immunohistochemical and genetic features of γδ T-cell lymphoma involving the spinal cord. Tumor cells were CD3(+) (A), TCRβF1(–) (B), TCRγ(+) (C), CD4(–) (D), CD8(–) (E), CD56(+) (F), TIA1(+) (G), and CD103(+) (H). (I) Monoclonal peak was observed in TCRγ gene rearrangement study.

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