Multiple neurofibroma is a systemic disease easily diagnosed, little studied and seldom cured. In 13 per cent of the reported cases sarcomatous transformation has occurred. Skeletal changes have been demonstrated in a number of instances. Endocrine disturbances were noted in a series of cases. Hence, reporting a single case of neurofibromatosis with sarcomatous transformation and complete clinical investigation of the endocrine and skeletal system adds another link to the chain of events that may eventually lead to the complete understanding of the disease.
Fischer1 in 1927 collected from the literature 59 cases of Recklinghausen's disease with sarcomatous transformation (13 per cent). Hosoi2 reported 2 cases in 1931 and found the number in the literature increased to 65. There were 39 male and 24 female patients (in 2 cases the sex was not mentioned), a ratio of 1.6 to 1. At the time of the present writing the number
CHARACHE H. MULTIPLE NEUROFIBROMA WITH SARCOMATOUS TRANSFORMATION AND SKELETAL INVOLVEMENT. Arch Derm Syphilol. 1939;40(2):185–191. doi:10.1001/archderm.1939.01490020012002
Customize your JAMA Network experience by selecting one or more topics from the list below.