PURPOSE: To describe the clinical presentation, diagnosis and treatment of periorbital extranodal natural killer/T-cell lymphoma, nasal type.
METHODS: Case series of three patients with periorbital involvement of extranodal natural killer/T-cell lymphoma, nasal type, of whom clinical data, orbital imaging and immunohistochemical analysis were collected. For the purpose of this study, all histopathological and immunohistochemical slides were re-examined.
RESULTS: All patients presented with painless eyelid swelling and a history of sinonasal disease, of whom one with bilateral panuveitis, not responding to systemic antibiotics. Extraocular muscle involvement was present in 2 cases upon presentation and in 1 case later on. Initial paranasal and orbital biopsies were negative in 2 patients, with only the second orbital biopsy leading to the diagnosis. Natural killer/T-cell and cytotoxic markers were present in all cases, as well as Epstein-Barr virus encoded RNA in situ hybridization. The patients died respectively 5, 9 and 35 months from diagnosis despite treatment with chemotherapy and radiotherapy.
CONCLUSION: Extranodal natural killer/T-cell lymphoma, nasal type, should be suspected in a painless periorbital cellulitis with chronic sinusitis, not responding to conventional therapy. A high index of suspicion is necessary in biopsies showing angiodestruction and necrosis. Epstein-Barr virus encoded RNA in situ hybridization and expert hematopathologist consultation is necessary to decrease the delay in diagnosis.
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Biomarkers, Tumor/analysis
- Combined Modality Therapy
- Epstein-Barr Virus Infections/diagnosis
- Eyelid Neoplasms/diagnosis
- Herpesvirus 4, Human/genetics
- In Situ Hybridization
- Lymphoma, Extranodal NK-T-Cell/diagnosis
- Magnetic Resonance Imaging
- Middle Aged
- Paranasal Sinus Neoplasms/diagnosis
- RNA, Viral/genetics
- Tomography, X-Ray Computed
- Young Adult