Hospital Nostra Senyora de Meritxell, Carrer dels Escalls, Escaldes-Engordany, Andorra.
Department of Otolaryngology-Head and Neck Surgery, Oxford University Hospitals, Oxford, UK.
J Oral Pathol Med. 2022 Nov;51(10):904-910. doi: 10.1111/jop.13354. Epub 2022 Sep 20.
Angiosarcoma is a rare but often fatal malignancy from blood and lymphatic vessels that can arise anywhere in the body and often affects the head and neck region. Although its dismal prognosis is predominantly explained by its aggressive biology, several secondary factors contribute to poor outcomes. These include a phenotypic resemblance to innocuous blood vessel lesions, which contributes to a significant degree of late diagnosis. Another important factor is the rarity of angiosarcoma, which has impaired scientific determination of its optimal treatment significantly. As a result, treatment of angiosarcomas has largely been guided by information derived from the study of sarcomas at large, themselves a highly heterogeneous group of mesenchymal cancers both from a diagnostic as well as therapeutical perspective. The Digital Revolution and resultant Information Age promise to transform the clinical management of rare cancers from a generic to a more customized approach. In this paper, we review the current understanding of head and neck angiosarcomas within the context of this process.
血管肉瘤是一种罕见但常致命的血液和淋巴管恶性肿瘤,可发生于身体任何部位,常累及头颈部。尽管其预后不良主要归因于其侵袭性生物学特性,但还有一些次要因素也导致了不良结局。其中包括与良性血管病变的表型相似,这在很大程度上导致了晚期诊断。另一个重要因素是血管肉瘤的罕见性,这严重影响了对其最佳治疗方法的科学确定。因此,血管肉瘤的治疗在很大程度上是基于从广义肉瘤研究中获得的信息,肉瘤本身从诊断和治疗的角度来看都是一组高度异质的间叶性癌症。数字革命和随之而来的信息时代有望将罕见癌症的临床管理从通用方法转变为更具针对性的方法。本文就在这一过程中,综述了头颈部血管肉瘤的当前认识。