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Nasal-type NK/T cell lymphoma of the orbit with distant metastases
Hiroko Kuwabara1, Motomu Tsuji, Yasuyoshi Yoshii
1Department of Pathology, Osaka Medical College, Takatsuki, Osaka, Japan.
Abstract:
A case of a nasal-type NK/T cell lymphoma of the orbit with distant metastases is reported. The orbital lymphoma was angiodestructive with prominent necrosis and frequent apoptosis. Lymphoma cells were positive for CD56, cytotoxic molecules (granzyme B and TIA-1), and EBV. Despite local radiotherapy, the patient had a highly aggressive clinical course. A postmortem examination revealed distant metastases to the heart and lungs exhibiting angiocentric growth. TCR and Ig genes of a cardiac lesion were in germ line configuration. This case demonstrated that nasal-type NK/T cell lymphomas with a poor prognosis can occur in the orbit.
Insights
Nasal-type NK/T cell lymphoma can occur in the orbit, presenting with aggressive angiodestructive features and distant metastases. This rare orbital lymphoma indicates a poor prognosis despite radiotherapy.
Area of Science:
- Oncology
- Ophthalmology
- Pathology
Background:
- Nasal-type Natural Killer/T cell lymphoma (NKTL) is an aggressive extranodal lymphoma.
- Orbital involvement by NKTL is rare, with limited data on its clinical presentation and behavior.
Observation:
- A case of orbital NKTL is described, characterized by angiodestructive growth, necrosis, and apoptosis.
- Tumor cells expressed CD56, cytotoxic molecules (granzyme B, TIA-1), and Epstein-Barr virus (EBV).
Findings:
- The patient experienced a rapid clinical decline despite radiotherapy.
- Postmortem examination revealed widespread metastases to the heart and lungs with angiocentric patterns.
- T-cell receptor (TCR) and immunoglobulin (Ig) genes in a cardiac lesion were found in germline configuration, suggesting a non-B/non-T lineage or a very early stage of rearrangement.
Implications:
- This case highlights that nasal-type NK/T cell lymphomas can manifest in the orbit.
- Orbital NKTL may follow a highly aggressive course with a propensity for distant metastases.
- The findings underscore the importance of considering NKTL in the differential diagnosis of orbital lymphomas, especially those with aggressive features.