TY - JOUR TI - An extranodal NK/T cell lymphoma, nasal type, with specific immunophenotypic and genotypic features AU - Katsaounis, Panagiotis AU - Alexopoulou, Alexandra AU - Dourakis, Spyros AU - P. AU - Smyrnidis, Alexandros AU - Marinos, Leonidas AU - Filiotou, Anna AU - and Archimandritis, Athanasios J. JO - International Journal of Hematologic Oncology PY - 2008 VL - 88 TODO - 2 SP - 202-205 PB - Springer Tokyo SN - 2045-1393, 2045-1407 TODO - 10.1007/s12185-008-0137-9 TODO - midline granuloma; destructive facial mass; angioivasion; clonal rearrangement of the T-cell receptor; CD56-negative TODO - 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. ER -