Malignant Transformation in Monostotic Fibrous Dysplasia Clinical Features, Imaging Features, Outcomes in 10 Patients, and Review
Malignant Transformation in Monostotic Fibrous Dysplasia Clinical Features, Imaging Features, Outcomes in 10 Patients, and Review
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DOI：
10.1097/md.0000000000000369
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发表时间：
2015-01-01
期刊：
影响因子：
1.6
通讯作者：
Zhang, Huizhen
中科院分区：
文献类型：
--
作者：
Qu, Nan;Yao, Weiwu;Zhang, Huizhen
纤维结构不良（FD）的恶性转化是罕见的。本研究的目的是探讨单发性FD的临床和影像学特征以及恶变的结局。回顾性分析了2005年1月至2013年12月10例经病理证实的单发性FD恶变的资料。记录患者数据，并由2名肌肉骨骼放射科医生评价X线片（n = 10）、计算机断层扫描（CT）（n = 5）、磁共振（MR）（n = 4）和骨密度图（n = 10）的病变位置、边缘、皮质破坏、骨髓受累、骨膜反应和软组织肿块，并达成一致意见。我们分析了10例患者的临床特征、治疗和预后，其中8例男性，2例女性（平均年龄46.5 ± 15.9岁）。受累部位为股骨（n = 4）、肱骨（n = 2）、胫骨（n = 3）和髂骨（n = 1）。5例有手术史，5例无手术史。没有患者接受过既往放射治疗。手术治疗5例，X线片及CT显示：单纯溶骨性病灶5例，边缘不清，骨皮质破坏5例，明显软组织肿块1例，矿化2例。对于5例未手术的病例，X线片和CT确定了边缘不佳的溶骨性病变，位于磨玻璃样阴影区域内或附近（n = 4），皮质侵蚀（n = 4）和矿化（n = 2）。磁共振成像（MRI）还发现了信号强度不均匀且明显增强的病变。骨显像显示单发病灶放射性核素偏心性摄取增高（n = 10）。病理报告显示骨肉瘤（n = 7）、纤维肉瘤（n = 2）和恶性纤维组织细胞瘤（MFH）（n = 1）。在研究结束时，1名患者死于肿瘤，1名患者因肺转移而存活，1名患者经历复发，7名患者存活而无复发。有手术史的FD患者应随访，因为手术部位的溶骨性病变强烈提示恶性转化。FD相关恶性肿瘤的影像学特征是边缘差、矿化和溶骨性病变伴皮质破坏。需要进一步的研究来探索FD中恶性肿瘤的发病机制，并建立最佳的治疗策略。
Malignant transformation in fibrous dysplasia (FD) is uncommon. The purpose of this study was to investigate clinical and imaging features, and outcomes of malignant transformation in monostotic FD.Data for 10 pathologically confirmed malignant transformations in monostotic FD from January 2005 to December 2013 were retrospectively reviewed. Patient data were recorded, and radiographs (n = 10), computed tomography (CT) (n = 5), magnetic resonance (MR) (n = 4), and bone scintigrams (n = 10) were evaluated for lesion location, margin, cortical destruction, marrow involvement, periosteal reaction, and soft tissue mass by 2 musculoskeletal radiologists with agreement by consensus. Clinical features, management, and prognosis were also analyzed for each of the 10 cases.There were 8 male and 2 female patients (mean age 46.5 +/- 15.9 years). The affected sites were the femur (n = 4), humerus (n = 2), tibia (n = 3), and ilium (n = 1). Five cases had received previous surgery and 5 cases had no history of surgery. No patients had been given prior irradiation treatment. For the 5 cases with surgery, radiographs and CT showed purely osteolytic lesions with poor margination in the curettage area (n = 5), cortical destruction (n = 5), obvious soft tissue mass (n = 1), and mineralization (n = 2). For the 5 cases without surgery, radiographs and CT identified poorly marginated, osteolytic lesions within or near the area with "ground-glass'' opacity (n = 4), cortical erosion (n = 4), and mineralization (n = 2). Magnetic resonance imaging (MRI) also identified lesions with heterogeneous signal intensity and pronounced enhancement. Bone scintigraphy revealed eccentric increased uptake of radionuclide in monostotic lesion (n = 10). Pathology reports revealed osteosarcoma (n = 7), fibrosarcoma (n = 2), and malignant fibrous histiocytoma (MFH) (n = 1). At the end of the study, 1 patient died from tumors, 1 patient was alive with lung metastasis, 1 patient experienced recurrence, and 7 patients were alive without recurrence. Patients with FD and a history of surgery should be followed up, for the osteolytic lesions in the operative areas strongly indicate the malignant transformation. The radiographic feature of FD-related malignancies is poorly marginated, mineralized, and osteolytic lesions with cortical destruction. Further investigations are needed to explore the pathogenesis of malignancies in FD and to establish optimal therapeutic strategies.