Lethal Midline Granuloma; A Case Report

Abstract

A 54-year-old man was referred to the Self-Defense Forces Central Hospital for evaluation of nasal obstruction, a nonhealing ulcer of the hard palate, severe headache and spiking fever. A necrotic and hemorrhagic tumor was found in the nasal cavity. The original biopsy revealed a dense cellular infiltrate to the submucosa in which mononuclear cells predominated. Chest roentgengram, ECG, clinical examination, serology and urinalysis were all within normal limits. The diagnosis was lethal midline granuloma. Radiotherepy was administered in a dose of 5000 rads to the involved areas, and predonisolone, cyclophosphamide, 6-MP and vinclistine were given. The treatment was effective, localized lesion was controlled, and the necrosis disappeared. Two months later, painful soft tissue swellings appeared in his thigh, scapula and hip. A biopsy of the hip lesion showed atypical mononuclear cells infiltrating the subcutaneous area and muscle layers. Serum CPK rose markedly and rapidly. Three weeks later the patient died of respiratory failure. This lethal midline granuloma was a systemic and progressive type, and the nasal involvement was the first manifestation of the disease.