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Peer-reviewedCC BY 4.0Frontiers in Oncology

Secondary intraocular extranodal natural killer/T-cell lymphoma with optic nerve involvement: a case report

  • Sinan Ersan1,2
  • Charles Zhang2,3,4
  • Sofia M. Miguez4
  • Maria A. Idarraga4,5
  • Lubomir Sokol6
  • Sander R. Dubovy4,5
  • Carlos E. Mendoza-Santiesteban4
  • Basil K. Williams4
6 affiliations
  1. 1Department of Ophthalmology, Storm Eye Institute, Medical University of South Carolina
  2. 2Department of Ophthalmology, Ross Eye Institute, University at Buffalo
  3. 3Retina Consultants of Texas
  4. 4Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine
  5. 5Florida Lions Ocular Pathology Laboratory, University of Miami Miller School of Medicine
  6. 6Department of Malignant Hematology, H. Lee Moffitt Cancer Center
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Abstract

A 61-year-old female with a history of extranodal natural killer/T-cell (NK/T-cell) lymphoma of the left thigh presented with a six-month history of presumed vitritis in the left eye managed by an outside provider. Diagnostic vitrectomy revealed atypical lymphocytes positive for CD3, CD7, CD56, and Epstein–Barr virus-encoded RNA, confirming the diagnosis of secondary extranodal intraocular NK/T-cell lymphoma. Initial systemic imaging showed no evidence of extraocular disease; nonetheless, systemic chemotherapy and external beam radiation therapy were recommended. The patient elected treatment with intravitreal methotrexate alone, with early improvement in visual acuity and intraocular inflammation. However, over the following three months, she developed retinal vasculitis and sub-retinal pigment epithelium infiltrates of the left eye, and abducens nerve palsy of the right eye. A week later, she developed acute worsening of symptoms in the left eye. MRI at the time demonstrated posterior optic nerve enhancement and nasal cavity involvement. Systemic and intrathecal chemotherapy was subsequently initiated. This represents a rare presentation of secondary intraocular extranodal NK/T-cell lymphoma with subsequent optic nerve involvement. The case highlights the aggressive clinical course and neurotropic potential of NK/T-cell lymphoma, underscoring the limitations of local therapy alone and the importance of early systemic evaluation and treatment.

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Cite

BibTeXRIS
BibTeX
@article{ersan2026secondary,
  title = {{Secondary intraocular extranodal natural killer/T-cell lymphoma with optic nerve involvement: a case report}},
  author = {Sinan Ersan and Charles Zhang and Sofia M. Miguez and Maria A. Idarraga and Lubomir Sokol and Sander R. Dubovy and Carlos E. Mendoza-Santiesteban and Basil K. Williams},
  journal = {Frontiers in Oncology},
  year = {2026},
  month = oct,
  doi = {10.3389/fonc.2026.1954795},
  url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1954795/full},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Secondary intraocular extranodal natural killer/T-cell lymphoma with optic nerve involvement: a case report
AU  - Sinan Ersan
AU  - Charles Zhang
AU  - Sofia M. Miguez
AU  - Maria A. Idarraga
AU  - Lubomir Sokol
AU  - Sander R. Dubovy
AU  - Carlos E. Mendoza-Santiesteban
AU  - Basil K. Williams
PY  - 2026
DA  - 2026/10/20
JO  - Frontiers in Oncology
DO  - 10.3389/fonc.2026.1954795
UR  - https://www.frontiersin.org/articles/10.3389/fonc.2026.1954795/full
AB  - A 61-year-old female with a history of extranodal natural killer/T-cell (NK/T-cell) lymphoma of the left thigh presented with a six-month history of presumed vitritis in the left eye managed by an outside provider. Diagnostic vitrectomy revealed atypical lymphocytes positive for CD3, CD7, CD56, and Epstein–Barr virus-encoded RNA, confirming the diagnosis of secondary extranodal intraocular NK/T-cell lymphoma. Initial systemic imaging showed no evidence of extraocular disease; nonetheless, systemic chemotherapy and external beam radiation therapy were recommended. The patient elected treatment with intravitreal methotrexate alone, with early improvement in visual acuity and intraocular inflammation. However, over the following three months, she developed retinal vasculitis and sub-retinal pigment epithelium infiltrates of the left eye, and abducens nerve palsy of the right eye. A week later, she developed acute worsening of symptoms in the left eye. MRI at the time demonstrated posterior optic nerve enhancement and nasal cavity involvement. Systemic and intrathecal chemotherapy was subsequently initiated. This represents a rare presentation of secondary intraocular extranodal NK/T-cell lymphoma with subsequent optic nerve involvement. The case highlights the aggressive clinical course and neurotropic potential of NK/T-cell lymphoma, underscoring the limitations of local therapy alone and the importance of early systemic evaluation and treatment.
SN  - 2234-943X
ER  -