Narter Selin, Yalcinkaya Ulviye, Bilgen Muhammet S, Yazici Zeynep
Department of Surgical Pathology, Uludag University, Faculty of Medicine, Uludag University, Bursa, Turkey.
Department of Orthopaedics and Traumatology, Uludag University, Faculty of Medicine, Uludag University, Bursa, Turkey.
Indian J Pathol Microbiol. 2021 Apr-Jun;64(2):394-397. doi: 10.4103/IJPM.IJPM_897_20.
The majority of bone angiosarcomas are primary tumors while secondary angiosarcomas arise after radiation therapy or bone infarctus. This article presents a case of malignant transformation of monostotic fibrous dysplasia into angiosarcoma. An 80-year-old female presented with pain on right cruris. Radiological examination revealed a lesion with lytic areas and destruction of cortical bone on right tibia. Gross and histopathological examination showed two areas with an abrupt transition. The solid component was composed of curved, immature bony trabeculae in a fibroblastic stroma. The other component involved epitheloid cells forming slit-like vascular spaces. The diagnosis of angiosarcoma and fibrous dysplasia was given. Malignant transformation of fibrous dysplasia into angiosarcoma is extremely rare; as this is the sixth case in the existing literature. Prognosis of fibrous dysplasia is generally good and less than 1% of the patients develop a malignant tumor. Therefore, patients with fibrous dysplasia should be offered a life-long follow-up.
大多数骨血管肉瘤是原发性肿瘤，而继发性血管肉瘤则发生于放射治疗或骨梗死之后。本文介绍了一例单骨型骨纤维异常增殖症恶变成为血管肉瘤的病例。一名80岁女性因右小腿疼痛就诊。放射学检查显示右侧胫骨有溶骨性区域及皮质骨破坏的病变。大体及组织病理学检查显示两个区域有突然转变。实性成分由成纤维细胞基质中弯曲的、不成熟的骨小梁组成。另一成分则由形成裂隙样血管腔隙的上皮样细胞构成。诊断为血管肉瘤及骨纤维异常增殖症。骨纤维异常增殖症恶变为血管肉瘤极为罕见，这是现有文献报道的第六例。骨纤维异常增殖症的预后通常良好，不到1%的患者会发展为恶性肿瘤。因此，骨纤维异常增殖症患者应接受终身随访。
