Series of extranodal natural killer/T-cell lymphoma, nasal type, with periorbital involvement

Karolien Termote, Daan Dierickx, Gregory Verhoef, Mark Jorissen, Thomas Tousseyn, Ilse Mombaerts

Research output: Contribution to journalArticlepeer-review

24 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)245-251
Number of pages7
JournalOrbit (Amsterdam, Netherlands)
Issue number4
Publication statusPublished - Aug 2014


  • Antineoplastic Combined Chemotherapy Protocols/therapeutic use
  • Biomarkers, Tumor/analysis
  • Combined Modality Therapy
  • Epstein-Barr Virus Infections/diagnosis
  • Eyelid Neoplasms/diagnosis
  • Female
  • Herpesvirus 4, Human/genetics
  • Humans
  • In Situ Hybridization
  • Lymphoma, Extranodal NK-T-Cell/diagnosis
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Paranasal Sinus Neoplasms/diagnosis
  • RNA, Viral/genetics
  • Radiotherapy
  • Tomography, X-Ray Computed
  • Young Adult


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