Yang X D, Xu G, Song L H, Zhu H, Liu X, Li F H, Peng S K, Ma Z Y, Zhou H
Department of Pathology, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu 610072, China.
Department of Radiology, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu 610072, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Feb 8;49(2):122-128. doi: 10.3760/cma.j.issn.0529-5807.2020.02.004.
To investigate the clinicopathological and radiological features of benign fibro-osseous lesion (BFOL). Sixty-five cases of craniofacial BFOL, eight cases of peripheral ossifying fibroma (POF) and one case of low-grade central osteosarcoma diagnosed at Sichuan Provincial People's Hospital between January 2010 and March 2019 were collected. The clinicopathologic features, hematoxylin-eosin and immunohistochemical (IHC) staining and radiographic features were analyzed. MDM2 gene amplification was detected by FISH in difficult borderline cases. This cohort of BFOLs included 50 cases of fibrous dysplasia (FD), 12 cases of ossifying fibroma (OF), and three cases of juvenile psammomatoid ossifying fibroma (JPOF). The average ages of patients with FD,OF and JPOF were 31.7, 39.2 and 26.0 years respectively. The male to female ratio was 1.0∶1.8.The average age of POF was 47.0 years, with male to female ratio of 1∶7. Patient of low-grade central osteosarcoma was a 48-year-old man. Twenty-seven cases of FD were located in the jaw, and 23 cases were in other craniofacial bones. Nine cases of OF were located in the jaw, and three cases were in the nasal cavity. Two cases of JPOF were in the nasal sinus, and one was in the jaw. All POF were located in the gingiva, and low-grade central osteosarcoma was located in the mandible. The imaging features of FD were luffa-like or ground-glass like signal shadows with poorly defined borders with expansion. OF had clear borders or sclerosing margins. Both JOF and low-grade central osteosarcoma were expansile intraosseously and with focally invasive nodular masses with ground-glass like signal shadows; and POF showed soft tissue mass with bone formation. Histological features of BFOLs showed mixed fibrous and irregular osteoid lesions. FD had no clear relationship with the host bone and no osteoblasts surrounded the bone trabeculae. Osteoblasts rimming was found in OF, and the boundaries of the host bone were clear. JPOF and low-grade central osteosarcoma infiltrated the host bone focally, and the latter showed mild cellular atypia. MDM2 amplification was detected in low-grade central osteosarcoma. BFOLs are a group of fibro-osseous lesions with similar morphology in the head and neck and face, but their clinical features and prognosis are different; and their imaging and histological characteristics are also slightly different. Attentions should be given to the combination of clinical, imaging and pathologic features of BFOLs, especially the differential diagnosis between BFOLs and low-grade central osteosarcoma. Molecular detection could be used to assist the diagnosis in difficult cases.
探讨骨纤维异常增殖症（BFOL）的临床病理及影像学特征。收集2010年1月至2019年3月在四川省人民医院确诊的65例颅面部BFOL、8例骨化性纤维瘤（POF）及1例低级别中央型骨肉瘤患者资料。分析其临床病理特征、苏木精-伊红及免疫组化（IHC）染色结果和影像学特征。对诊断困难的临界病例采用荧光原位杂交（FISH）检测MDM2基因扩增情况。该组BFOL包括50例骨纤维结构不良（FD）、12例骨化性纤维瘤（OF）及3例青少年砂粒样骨化性纤维瘤（JPOF）。FD、OF及JPOF患者的平均年龄分别为31.7岁、39.2岁和26.0岁。男女比例为1.0∶1.8。POF患者平均年龄47.0岁，男女比例为1∶7。低级别中央型骨肉瘤患者为1名48岁男性。27例FD位于颌骨，23例位于其他颅面骨。9例OF位于颌骨，3例位于鼻腔。2例JPOF位于鼻窦，1例位于颌骨。所有POF均位于牙龈，低级别中央型骨肉瘤位于下颌骨。FD的影像学特征为丝瓜样或磨玻璃样信号影，边界不清，有膨胀。OF边界清晰或有硬化边缘。JOF和低级别中央型骨肉瘤均呈骨内膨胀性生长，有局灶浸润性结节状肿块，呈磨玻璃样信号影；POF表现为有骨形成的软组织肿块。BFOL的组织学特征为纤维组织与不规则骨样病变混合存在。FD与宿主骨无明确关系，骨小梁周围无成骨细胞。OF可见成骨细胞围绕，宿主骨边界清晰。JPOF和低级别中央型骨肉瘤局灶浸润宿主骨，后者有轻度细胞异型性。低级别中央型骨肉瘤检测到MDM2扩增。BFOL是一组头颈部及面部形态相似的纤维-骨病变，但临床特征及预后不同；其影像学和组织学特征也略有差异。应重视BFOL临床、影像学及病理特征的综合判断，尤其是BFOL与低级别中央型骨肉瘤的鉴别诊断。分子检测可用于疑难病例的辅助诊断。
