Department of Pathology.
Department of Pathology, Hospital of Padua, Padova.
Am J Surg Pathol. 2020 Sep;44(9):1192-1203. doi: 10.1097/PAS.0000000000001487.
Recent molecular discoveries have refined vascular bone tumor classification. To investigate the clinical relevance of these refinements, we reviewed all cases of primary vascular bone tumors treated at our Institute. On the basis of morphology, cases were assessed immunohistochemically and molecularly. A total of 427 cases of primary vascular tumor of bone with available follow-up and histologic material were retrieved and reclassified according to the most recent diagnostic criteria as follows: 289 hemangiomas, 38 epithelioid hemangiomas, 21 epithelioid hemangioendotheliomas, 2 retiform hemangioendotheliomas, 1 intraosseous papillary intralymphatic angioendothelioma, 24 pseudomyogenic hemangioendotheliomas, and 52 angiosarcomas (of these, 45 were epithelioid angiosarcomas and 7 spindle cell secondary angiosarcoma). Both epithelioid and classic hemangiomas behave as benign tumors with excellent prognosis. The distinction between cellular and conventional type of epithelioid hemangioma was not associated with a different clinical course. Conversely, epithelioid hemangioendothelioma exhibited a more aggressive clinical behavior than hemangioma, with higher rates of multifocality and distant spread. Immunohistochemical positivity for CAMTA1 or TFE3 did not have a prognostic implication. In epithelioid hemangioendothelioma, the presence of morphologic malignant features was associated with reduced disease-free (P=0.064) and overall survival (P=0.055). Pseudomyogenic hemangioendothelioma featured local aggressiveness in 5/24 patients exhibiting a clinical behavior closer to epithelioid hemangioma than epithelioid hemangioendothelioma. Last, 32/45 patients with epithelioid angiosarcoma died of disease with a median survival time of 10 months from diagnosis. In conclusion, the integration of morphologic, immunohistochemical, and molecular features allows a better stratification of primary vascular tumors of bone with significant prognostic and therapeutic implications.
最近的分子发现已经完善了血管性骨肿瘤的分类。为了研究这些细化的临床相关性，我们回顾了我们研究所治疗的所有原发性血管性骨肿瘤病例。根据形态学，对病例进行了免疫组织化学和分子评估。共检索到 427 例具有可获得随访和组织学材料的原发性骨血管肿瘤病例，并根据最新的诊断标准重新分类如下：289 例血管瘤、38 例上皮样血管瘤、21 例上皮样血管内皮细胞瘤、2 例网状血管内皮细胞瘤、1 例骨内乳头状淋巴管内血管内皮瘤、24 例假肌源性血管内皮细胞瘤和 52 例血管肉瘤（其中 45 例为上皮样血管肉瘤，7 例为梭形细胞继发性血管肉瘤）。上皮样和经典血管瘤均表现为良性肿瘤，预后良好。细胞型和经典型上皮样血管瘤之间的区别与不同的临床病程无关。相反，上皮样血管内皮细胞瘤的临床行为比血管瘤更具侵袭性，其多灶性和远处转移的发生率更高。CAMTA1 或 TFE3 的免疫组织化学阳性结果没有预后意义。在上皮样血管内皮细胞瘤中，形态学恶性特征的存在与无病生存（P=0.064）和总生存（P=0.055）降低相关。24 例假肌源性血管内皮细胞瘤中有 5 例患者局部侵袭性较大，其临床行为更接近上皮样血管瘤，而非上皮样血管内皮细胞瘤。最后，32 例上皮样血管肉瘤患者中有 32 例死于疾病，从诊断到中位生存时间为 10 个月。总之，形态学、免疫组织化学和分子特征的综合分析可以更好地对原发性血管性骨肿瘤进行分层，具有重要的预后和治疗意义。
