G Tigges1, K W Delank, S Blasius, W Stoll. 1. Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde, Westfälischen Wilhelms-Universität Münster.
Abstract
BACKGROUND: The lethal midline granuloma is a heterogeneous disorder and the pathogenesis is likely to be complex. Advances in immunocytochemical phenotyping and molecular genetics suggest that in several cases the origin have been proven to be malignant lymphomas, especially of T-cell lineage. PATIENT: The present clinical and pathological cases of a 35-year-old woman shows how difficult it is to establish the precise diagnosis of lethal midline granuloma (LMG). The patient presented with recurrent infections of the paranasal sinuses. Later she developed infiltrates and ulcerous lesions of the nose, paranasal sinuses, and the base of the skull clinically consistent with a LMG. Immunohistologic studies using monoclonal antibodies led to the positive diagnosis of peripheral lymphoma of T-cells. RESULTS: Several surgical interventions and explorations revealed a diffuse infiltration and partly necrotic lesions of the tissue. After identification of a T-cell lymphoma by histological examination, the patient received megavoltage irradiation of 45 Gy. Additional chemotherapy with alkylating agents was given. Two years after treatment the patient achieved and remained in remission. CONCLUSION: The midline granuloma syndrome is a mutilating process produced by a number of diseases that progressively destroy the nose, paranasal sinuses and other regions of the midface. Infectious, neoplastic and idiopathic forms have been described. The specific diagnosis must be ascertained, as the treatment is different depending of the etiology of the disease. Sinonasal lymphomas constitute a important distinct clinicopathologic entity which may present as lethal midline granuloma. They seems to be strongly associated with Epstein-Barr virus. Aggressive therapy with radiotherapy and chemotherapy can stop the progress of the midfacial destruction. The correct treatment is still discussed controversially.
BACKGROUND: The lethal midline granuloma is a heterogeneous disorder and the pathogenesis is likely to be complex. Advances in immunocytochemical phenotyping and molecular genetics suggest that in several cases the origin have been proven to be malignant lymphomas, especially of T-cell lineage. PATIENT: The present clinical and pathological cases of a 35-year-old woman shows how difficult it is to establish the precise diagnosis of lethal midline granuloma (LMG). The patient presented with recurrent infections of the paranasal sinuses. Later she developed infiltrates and ulcerous lesions of the nose, paranasal sinuses, and the base of the skull clinically consistent with a LMG. Immunohistologic studies using monoclonal antibodies led to the positive diagnosis of peripheral lymphoma of T-cells. RESULTS: Several surgical interventions and explorations revealed a diffuse infiltration and partly necrotic lesions of the tissue. After identification of a T-cell lymphoma by histological examination, the patient received megavoltage irradiation of 45 Gy. Additional chemotherapy with alkylating agents was given. Two years after treatment the patient achieved and remained in remission. CONCLUSION: The midline granuloma syndrome is a mutilating process produced by a number of diseases that progressively destroy the nose, paranasal sinuses and other regions of the midface. Infectious, neoplastic and idiopathic forms have been described. The specific diagnosis must be ascertained, as the treatment is different depending of the etiology of the disease. Sinonasal lymphomas constitute a important distinct clinicopathologic entity which may present as lethal midline granuloma. They seems to be strongly associated with Epstein-Barr virus. Aggressive therapy with radiotherapy and chemotherapy can stop the progress of the midfacial destruction. The correct treatment is still discussed controversially.