Osseous destruction by neurofibroma diagnosed in infancy as "desmoplastic fibroma"

A 2-month-old male infant presented with a lytic lesion of the distal radius which was diagnosed as desmoplastic fibroma. Tumor recurred following en bloc resection of the distal radius and forearm, necessitating an elbow disarticulation. Subsequently, the child developed multiple café-au-lait spots, and histologic sections have been reinterpreted as neurofibromatosis. This case illustrates the potential difficulty in diagnosing neurofibromatosis when few or no classic stigmata of the disease are present.