Hermann G, Shapiro R, Abdelwahab I F, Klein M J, Pastores G, Grabowski G
Department of Radiology, Mount Sinai Medical Center of the City University of New York, New York 10029-6574.
Skeletal Radiol. 1994 May;23(4):253-6. doi: 10.1007/BF02412357.
Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell deposits. In one instance the mass destroyed the posterior cortex of the left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended into the soft tissues. While the incidence of neoplastic disorder such as lymphoproliferative disease appears to be more common in Gaucher disease patients than in the general population, lesions of benign etiology that mimic these aggressive processes should be considered in the differential diagnosis when cortical destruction with coexisting soft tissue most is found in these patients.
本文描述了两例I型戈谢病患者出现由戈谢细胞沉积形成的骨外软组织肿块的病例。其中一例肿块破坏了左股骨远端的后皮质并突入软组织。在第二例中，病变累及胫骨近端并逐渐延伸至软组织。虽然诸如淋巴增殖性疾病等肿瘤性疾病在戈谢病患者中的发病率似乎比普通人群更高，但当在这些患者中发现皮质破坏并伴有软组织肿块时，鉴别诊断中应考虑到具有类似侵袭性过程的良性病因病变。
