Sbaraglia Marta, Righi Alberto, Gambarotti Marco, Vanel Daniel, Picci Piero, Dei Tos Angelo P
Department of Pathology, Azienda ULSS2 Marca Trevigiana, Treviso, Italy.
Department of Pathology, Istituto Ortopedico Rizzoli, Bologna, Italy.
Surg Pathol Clin. 2017 Sep;10(3):705-730. doi: 10.1016/j.path.2017.04.013. Epub 2017 Jun 29.
Primary bone sarcomas represent extremely rare entities. The use of now abolished labels, such as malignant fibrous histiocytoma and hemangiopericytoma, has significantly hampered the chance of identifying specific entities. It is now accepted that a broad variety of mesenchymal malignancies most often arising on the soft tissue may actually present as primary bone lesions. A more accurate morphologic partition is justified based on availability of distinct therapeutic options. An integrated diagnostic approach represents the only way to achieve a correct classification. In consideration of the significant complexity, primary bone sarcomas should ideally be handled in the context of expert centers.
原发性骨肉瘤是极为罕见的疾病。过去使用现已废除的名称，如恶性纤维组织细胞瘤和血管外皮细胞瘤，极大地妨碍了识别特定疾病的可能性。目前已公认，多种最常发生于软组织的间叶性恶性肿瘤实际上可能表现为原发性骨病变。基于不同治疗方案的可用性，进行更准确的形态学分类是合理的。综合诊断方法是实现正确分类的唯一途径。鉴于其显著的复杂性，原发性骨肉瘤理想情况下应由专家中心进行处理。
