Extranodal Natural KillerT-cell lymphoma, nasal type ‘midline lethal granuloma.’ A case report.docVIP

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Extranodal Natural KillerT-cell lymphoma, nasal type ‘midline lethal granuloma.’ A case report.doc

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Extranodal Natural KillerT-cell lymphoma, nasal type ‘midline lethal granuloma.’ A case report

Tlholoeetal.HeadFaceMedicine2013,9:4 /content/9/1/4 HEAD FACE MEDICINE CASE REPORT OpenAccess ExtranodalNaturalKiller/T-celllymphoma,nasal type:‘midlinelethalgranuloma.’Acasereport MarthaMTlholoe1,MonicaKotu2,RaziaAGKhammissa3,MeschackBida4,JohanLemmer3andLiviuFeller3* Abstract Extranodalnaturalkiller/Tcelllymphoma,nasaltype,isanon-Hodgkinlymphoma,mostcommonlyaffectingthe nasalcavity,paranasalsinusesandnasopharynx.Clinicallyitischaracterisedbydestructionoffacialtissues, commencinginthemidline.Inmostcasesitarisesfrommalignanttransformationofnaturalkillercells(NK); sometimesfrommalignanttransformationofcytotoxicTcells. ExtranodalNK/Tcelllymphoma,nasaltype,israre,butevenmorerareinblackpersons.Thepurposeofthisarticle istoreportaseverecaseofextranodalNK/Tcelllymphoma,nasaltype,inanelderlyblackmale. Keywords:Midlinelethalgranuloma,ExtranodalNK/Tcelllymphoma Introduction granulomatosisandfungalinfections,thedefinitivediagno- Extranodal natural killer/T cell (NK/T cell) lymphoma, sismustbebasedonhistopathological,immunologicaland nasaltype,isararenon-Hodgkinlymphomaoriginating molecularstudies[5]. in the nasal cavity or in the paranasal sinuses. It is Localised extranodal NK/Tcell lymphoma, nasal type strongly associated with Epstein-Barr virus (EBV) infec- usually responds favourably to radiotherapy. As in any tion. Its prevalence is higher in countries in South-East neoplasm the best clinical outcome is achieved when Asia and in Central and South America than in Europe treatment is started early in the course of the disease; andinNorthAmerica;itoccursinmiddle-agedpersons but oncethetumour hasinvaded,radiotherapy mustbe and affects males more frequently than females [1-5]. supplemented with chemotherapy. Nevertheless, local Most cases arise from natural killer cells, only a few recurrenceoccursinabout50%ofcases.Extensivelocal fromcytotoxicT-cells[6,7]. invasion, regional lymph node involvement, elevated Clinically,extranodalNK/Tcelllymphoma,nasaltype,