Series of Extranodal Natural Killer/T-cell Lymphoma, Nasal Type, with Periorbital Involvement

Abstract

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.

Nasal Type Extranodal Natural Killer/T (NK/T) Cell Lymphoma Presenting as Periorbital Cellulitis: A Case Report

“…Very few cases with periorbital cellulitis as the initial presentation of NKTCL have been reported in the literature, as we report in our case [8–10]. Termote et al described 3 similar cases, all treated by chemo-radiotherapy, but they died within 5–35 months of diagnosis [8]. The other similar presentation was described by Kim JW et al and was treated as sinusitis, but biopsies taken after relapses confirmed NKTCL diagnosis [9].…”

“…Clinically, it can be divided into nasal and extranasal types [4]; the nasal variety commonly presents with nasal obstruction, but also it can cause epistaxis, extensive involvement of the midfacial structure, involvement of the orbit causing proptosis, and, occasionally, the hard palate [4,5,7]. Very few cases with periorbital cellulitis as the initial presentation of NKTCL have been reported in the literature, as we report in our case [8–10]. Termote et al described 3 similar cases, all treated by chemo-radiotherapy, but they died within 5–35 months of diagnosis [8].…”

Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa

“…It was only after repeated recurrences and multiple biopsies that a diagnosis of NKTL involving the lacrimal sac, orbit and the nasal cavity was made. Termote et al 19 reported a case series comprising three patients with periorbital involvement from NKTL who all developed painless eyelid swelling, which, along with a history of chronic sinusitis with nasal congestion, masqueraded as periorbital cellulitis. The diagnosis was established from biopsies of the orbital mass, and in one case of the cervical lymphadenopathy 17 .…”

Lymphoproliferative Disease of the Orbit

“…Cases could present with painless periorbital cellulitis with chronic sinusitis. 59 Only 8 cases of isolated orbital involvement without nasal mucosal involvement have been reported in the literature. Majority of the patients were middle-aged males.…”

“…Epstein-Barr virusencoded RNA in situ hybridization would assist in diagnosing the disease. 59 Natural killer/T-cell lymphoma involving the orbit typically runs an aggressive course. In the largest series reported by Woog et al, 60 6 of 8 patients died within 1 year after presentation, and the 5-year mortality rate was up to 87.5% despite radiation and/or chemotherapy.…”