title: An aggressive primary orbital natural killer/T-cell lymphoma case: poor response to chemotherapy.
abstract: Natural killer/T-cell lymphoma (NKTCL) and its presentation with extranodal orbital involvement as a single lesion are extremely rare. The aim of this article was to describe the presentation, diagnosis, and systemic treatment of a primary orbital NKTCL. A 67-year-old Caucasian woman presented with left exophthalmos, pain, periorbital swelling, and limited extrinsic ocular motility. Orbital cellulitis was suspected, but finally orbital biopsy was performed due to no response to initial antibiotic and anti-inflammatory standard treatment. The pathologic diagnosis was NKTCL. Systemic evaluations were negative. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy was initiated, but after 2 cycles of treatment, tumoral progression was observed. SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) rescue chemotherapy was then administered. Lymphoma progression was inevitable. She died 10 months later. Although more nasal NKTCL cases have been described, the nonnasal primary orbital NKTCL is an uncommon neoplasm with high mortality rate, despite the recent use of more potent chemotherapy regimens.
authors: *Department of Ophthalmology, Santa Creu i Sant Pau Hospital; †Department of Orbital and Ophthalmic Plastic Surgery, Institut Català de Retina; ‡Department of Hematology and §Department of Pathology, Santa Creu i Sant Pau Hospital, Barcelona, Spain.
authors: Tizana
authors: T
authors: Marchino
authors: Núria
authors: N
authors: Ibáñez
authors: Sebastián
authors: S
authors: Prieto
authors: Silvana
authors: S
authors: Novelli
authors: Justyna
authors: J
authors: Szafranska
authors: Anna
authors: A
authors: Mozos
authors: Xavier
authors: X
authors: Graell
authors: José A
authors: JA
authors: Buil
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D005094:Exophthalmos / Q000175:diagnosis; D058447:Eye Pain / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D054391:Lymphoma, Extranodal NK-T-Cell / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D014057:Tomography, X-Ray Computed