Morioka Hideo, Kamata Yasuhiro, Nishimoto Kazumasa, Susa Michiro, Kikuta Kazutaka, Horiuchi Keisuke, Sasaki Aya, Kameyama Kaori, Nakamura Masaya, Matsumoto Morio
Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan.
Department of Orthopaedic Surgery, National Defense Medical College, Tokorozawa, Japan.
Case Rep Oncol. 2016 Feb 19;9(1):126-33. doi: 10.1159/000443476. eCollection 2016 Jan-Apr.
Fibrous dysplasia (FD) is a monostotic or polyostotic benign bone lesion with spindle-cell proliferation in woven bone and stroma. Rarely, cartilaginous differentiation can be seen in the lesions of FD. FD with massive cartilaginous differentiation is called fibrocartilaginous dysplasia (FCD) and is considered a rare variant of FD. Although pathological findings of FD show irregular immature bone formation without osteoblastic rimming in fibrous tissue, and rarely show very small amounts of cartilage, histological images of FCD are said to show that cartilage with a relatively high cell density is present in the majority and that FD-like findings are seen in parts of it. The most characteristic feature of FCD on images is calcification in the lesions reflecting cartilaginous tissue. On the other hand, typical radiographic findings of FD include shadows with a ground-glass appearance and thinning and bulging of the cortical bone, the observation if calcification is not usual. Therefore, in the diagnosis of FCD, differentiation from multiple enchondromatosis, Ollier disease, chondrosarcoma, and chondrosarcoma secondary to FD is necessary, and it seems important to make a careful diagnosis based not only on the pathological findings but also on imaging and clinical findings. Herein, we report on a case of FD of the proximal femur associated with intralesional extensive carti laginous differentiation in which a pathological fracture occurred during follow-up, with a review of the literature.
骨纤维异常增殖症(FD)是一种单骨型或多骨型良性骨病变,在编织骨和间质中存在梭形细胞增殖。FD病变中罕见软骨分化。伴有大量软骨分化的FD称为纤维软骨发育异常(FCD),被认为是FD的一种罕见变异型。尽管FD的病理表现显示纤维组织中存在不规则的不成熟骨形成,且无成骨细胞边缘,且很少显示极少量软骨,但据说FCD的组织学图像显示大部分存在细胞密度相对较高的软骨,且部分可见FD样表现。FCD在影像学上最具特征性的表现是病变内反映软骨组织的钙化。另一方面,FD的典型影像学表现包括磨玻璃样阴影以及皮质骨变薄和膨出,通常观察不到钙化。因此,在FCD的诊断中,有必要与多发性内生软骨瘤、Ollier病、软骨肉瘤以及继发于FD的软骨肉瘤相鉴别,似乎不仅基于病理表现,而且基于影像学和临床发现进行仔细诊断很重要。在此,我们报告一例股骨近端FD伴有病灶内广泛软骨分化,随访期间发生病理性骨折,并对文献进行复习。