Langerhans' cell granulomatosis in an adult: a 22-year follow up

A 57-year-old patient with Langerhans' cell granulomatosis (LCG) is present ed. During 22 years of treatment, there were five relapsing infiltrations a t different sites of the skull bones, which were treated by surgery, local radiotherapy, and chemotherapy. During the last relapse, the right temporal bone was infiltrated by granuloma and the petrous bone was destroyed with an intimate spread to the internal carotid artery. After two palliative sur gical resections and ineffective radiotherapy, 12 cycles of chemotherapy (v inblastine plus prednisolone) were applied and a clinical remission of the disease was achieved. Special attention is paid to the complexity of diagno sis and choice of therapy. It is concluded that the behavior of LCG may cha nge with time, and assume an aggressive form of the disease. Chemotherapy i s the treatment of choice for this type of multifocal malignant form of LCG . Prognosis of the disease is unpredictable.