Fibrocartilaginous Dysplasia - A Report of Five Cases with Review of Literature

Abstract

Introduction: Fibrocartilaginous dysplasia (FCD) is a variant of fibrous dysplasia (FD) with extensive cartilaginous differentiation. This has been reported in both monostotic and polyostotic types of FD, the proximal femur being the most common site involved.

Case report: We report five cases of fibrocartilaginous dysplasia with varying degrees of cartilaginous differentiation. The age of the patients ranged from 7 to 30 years, and there was a female predominance (M:F ratio of 1:4). The proximal femur was the site of involvement in all the cases. Imaging showed well demarcated radiolucent lesions with stippled calcifications. Histologically, cartilaginous areas were noted juxtaposed to typical areas of fibrous dysplasia. Four of the patients were treated with curettage and one with a marginal resection. None of the five cases had recurrences at the past follow-up.

Conclusion: FCD is a rare variant of fibrous dysplasia which needs to diagnosed and treated early, as there is a high risk of pathological fracture.

Keywords: Fibrous dysplasia; cartilaginous tumors; chondrosarcoma; enchondroma; fibrocartilaginous.

Figure 1

(a) Radiograph showing classical shepherd crook deformity with associated fracture (b) gross specimen showing pale white lesion with a cystic area with associated fracture.

Figure 2

(a) (H and E, 40×) fibrous dysplasia with cartilaginous areas, (b) (H and E, 40×) fibrous dysplasia areas with irregular trabeculae within spindle cell stroma, (c) (H and E, 100×) cartilaginous areas, (d) (H and E, 40×) Epiphyseal growth plate like area.