title: An extranodal NK/T cell lymphoma, nasal type, with specific immunophenotypic and genotypic features.
abstract: Extranodal NK/T cell lymphoma, 'nasal type,' is a rare clinicopathological entity in Europe. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. We report the case of a patient who presented with fever, dyspnea, nasal congestion, headache, distention of right nasal turbinates and exophytic lower leg ulcerating lesions. A CT scan of visceral scull demonstrated a filling mass of right frontal, ethmoidal and maxillary sinuses with erosion of the wall of right maxillary sinus and ventral portion of the diaphragm. A biopsy was performed in the skin lesion and showed an angioinvasive NK/T cell lymphoma CD56 negative with clonal rearrangement of the T-cell-receptor gamma gene. Up to our knowledge, this is a rare immunophenotype for NK/T-cell, 'nasal type,' lymphomas. However, the lymphoma may be classified as extranodal NK/T cell lymphoma, 'nasal type,' due to typical clinical presentation, radiologic findings and pathological characteristics of polymorphism, angioinvasion, angiodestruction and coagulative necrosis.
authors.affiliation: 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Katsaounis
authors.affiliation: 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece. alexopou@ath.forthnet.gr.|, 40 Konstantinoupoleos St., 16342, Hilioupolis, Athens, Greece. alexopou@ath.forthnet.gr.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Alexopoulou
authors.affiliation: 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.
authors.forename: Spyros P
authors.initials: SP
authors.lastname: Dourakis
authors.affiliation: 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.
authors.forename: Alexandros
authors.initials: A
authors.lastname: Smyrnidis
authors.affiliation: Pathology of Blood Department, Evangelismos Hospital, Athens, Greece.
authors.forename: Leonidas
authors.initials: L
authors.lastname: Marinos
authors.affiliation: 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.
authors.forename: Anna
authors.initials: A
authors.lastname: Filiotou
authors.affiliation: 2nd Department of Medicine, University of Athens Medical School, Hippokration General Hospital, Athens, Greece.
authors.forename: Athanasios J
authors.initials: AJ
authors.lastname: Archimandritis
mesh_terms: D000369:Aged, 80 and over; D001706:Biopsy; D019002:CD56 Antigen / Q000378:metabolism; D017809:Fatal Outcome; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology*; D009669:Nose Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D019002:CD56 Antigen