Nasal T-cell lymphoma as a differential diagnosis of the midline granulomasyndrome

Background: The lethal midline granuloma is a heterogeneous disorder and th e pathogenesis is likely to be complex. Advances in immunocytochemical phen otyping and molecular genetics suggest that in several cases the origin hav e been proven to be malignant lymphomas, especially of T-cell lineage. Pati ent: The present clinical and pathological case of a 35-year-old woman show s how difficult it is to establish the precise diagnosis of lethal midline granuloma (LMG). The patient presented with recurrent infections of the par anasal sinuses. Later she developed infiltrates and ulcerous lesions of the nose, paranasal sinuses, and the base of the skull clinically consistent w ith a LMG. Immunhistologic studies using monoclonal antibodies led to the p ositive diagnosis of peripheral lymphoma of T-cells. Results: Several surgi cal interventions and explorations revealed a diffuse infiltration and part ly necrotic lesions of the tissue. After identification of a T-cell lymphom a by histological examination, the patient received megavoltage irradiation of 45 Gy. Additional chemotherapy with alkylating agents was given. Two ye ars after treatment the patient achieved and remained in remission. Conclus ion: The midline granuloma syndrome is a mutilating process produced by a n umber of diseases that progressively destroy the nose, paranasal sinuses an d other regions of the midface. Infectious, neoplastic and idiopathic forms have been described. The specific diagnosis must be accertained, as the tr eatment is different depending of the etiology of the disease. Sinonasal ly mphomas constitute a important distinct clinicopathologic entity which may present as lethal midline granuloma. They seems to be strongly associated w ith Epstein-Barr virus. Aggressive therapy with radiotherapy and chemothera py can stop the progress of the midfacial destruction. The correct treatmen t is still discussed controversially.