EXTRAOSSEOUS EXTENSION OF GAUCHER CELL DEPOSITS MIMICKING MALIGNANCY

Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell d eposits. In one instance the mass destroyed the posterior cortex of th e left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended in to the soft tissues. While the incidence of neoplastic disorder such a s lymphoproliferative disease appears to be more common in Gaucher dis ease patients than in the general population, lesions of benign etiolo gy that mimic these aggressive processes should be considered in the d ifferential diagnosis when cortical destruction with coexisting soft t issue most is found in these patients.