The Journal of Bone and Joint Surgery American Volume Soft-Tissue Neoplasms Associated with Congenital

Many reports have helped to clarify and to classify the numerous clinical manifesta-tions which may occur in the bizarre disease known as congenital neurofibromatosis. While the skeletal manifestations are perhaps better known to the orthopaedic surgeon, soft-tissue neoplasms are also commonly encountered. Of these, the caf#{233}au lait spot, naevus lateralis, verrucous-type skin hypertrophy, and the neurofibromatous nodule are of little clinical significance. The diffuse soft-tissue hypertrophy, plexiform type of neuro-fibromatosis, and primary tumors arising in the major nerve components, however, are of major clinical importance. Experience derived from the present series of 136 consecutive cases indicates that soft-tissue neoplasms occurring in this disease may be classified separately under two major headings: primary neurogenic tumors and tumors of other soft-tissue structures. Obviously, the two may occur simultaneously in the same patient; yet, in some patients, tumefaction of major nerve components may exist independently with minimal involvement of adjacent soft-tissue structures. NEOPLASMS OF SOFT TISSUES OTHER THAN NERVE TISSUE A classification of neoplasms of soft tissues other than nerve tissue is misleading i