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


CONCLUSIONS
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.
Department of Ophthalmology and.
Karolien
K
Termote
Daan
D
Dierickx
Gregory
G
Verhoef
Mark
M
Jorissen
Thomas
T
Tousseyn
Ilse
I
Mombaerts
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D003131:Combined Modality Therapy; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000628:therapy / Q000821:virology; D005142:Eyelid Neoplasms / Q000175:diagnosis* / Q000628:therapy / Q000821:virology; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification; D006801:Humans; D017403:In Situ Hybridization; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis* / Q000628:therapy / Q000821:virology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010255:Paranasal Sinus Neoplasms / Q000175:diagnosis* / Q000628:therapy / Q000821:virology; D012367:RNA, Viral / Q000235:genetics; D011878:Radiotherapy; D014057:Tomography, X-Ray Computed; D055815:Young Adult
D014408:Biomarkers, Tumor; D012367:RNA, Viral
Extranodal lymphoma; nasal lymphoma; natural killer/T-cell lymphoma; orbital lymphoma