[Lethal midline granuloma, An anatomoclinical study apropos of 3 cases]

In Midline malignant granuloma, a clinical entity characterized by necrotic ulcerations of the upper respiratory tract, two different histological features can be observed. The first one, called "idiopathic" granuloma, looks like simple granulation tissue and remains a strictly localized disease. In the second type (midline malignant reticulosis or histiocytosis), the granulation tissue contains atypical histiocytic cells. The 3 cases reported here belong to this last group. The early lesions were located in the nose (2 cases) or in the maxillary sinus (1 case). The histological analysis showed in all cases 20 to 40% of atypical histiocytes associated with other "inflammatory" cells. Radiotherapy was used in association with chemotherapy or surgery in the treatment. One patient is still alive at 3 years. We have no follow-up data for another patient. The third patient died after 18 months. An autopsy demonstrated multifocal visceral involvement by atypical histiocytes (malignant histiocytic lymphoma). The histogenesis of this disease, in light of histoenzymological and ultrastructural findings, and its possible relationship to lymphomatoid granulomatosis are discussed.