Series of Extranodal Natural Killer/T-cell Lymphoma, Nasal Type, with Periorbital Involvement. (August 2014)

• Record Type: Journal Article
• Authors: Termote, Karolien; Dierickx, Daan; Verhoef, Gregory; Jorissen, Mark; Tousseyn, Thomas; Mombaerts, Ilse
• Is Part Of: Orbit. Volume 33:Number 4(2014)
• Page Start: 245
• Page End: 251
• Publication Date: 2014-08
• Subjects: Eye-sockets -- Surgery -- Periodicals; Eye-sockets -- Diseases -- Periodicals; Orbit -- surgery -- Periodicals; Orbital Diseases -- Periodicals; 617.7
• DOI: 10.3109/01676830.2014.902478
• Languages: English
• ISSNs: 0167-6830

Abstract

<abstract> <title>Abstract</title> <p> <italic>Purpose</italic>: To describe the clinical presentation, diagnosis and treatment of periorbital extranodal natural killer/T-cell lymphoma, nasal type.</p> <p> <italic>Methods</italic> <sc>:</sc> 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.</p> <p> <italic>Results</italic> <sc>:</sc> 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<italic>. </italic>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.</p> <p> <italic>Conclusion</italic> <sc>:</sc> 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.</p> </abstract>