Paral Kristen, Krausz Thomas
Section of Dermatopathology, Consolidated Pathology Consultants, Libertyville, IL, USA.
Department of Pathology, University of Chicago, Chicago, IL, USA.
Mediastinum. 2020 Sep 30;4:25. doi: 10.21037/med-20-40. eCollection 2020.
Vascular tumors represent only a sliver of all tumors affecting the mediastinum, but they pose diagnostic challenges due to significant overlap among entities, ever-evolving classification schemes, and the exquisite rarity of some of the entities not only in the mediastinum but in pathology practice as a whole. Most of the vascular tumors are better known to the practice of soft tissue pathology, from which some of the knowledge of clinical behavior can be extrapolated. For example, the stratification of epithelioid hemangioendothelioma (EHE) into two biologically separate categories has effectively translated from the somatic soft tissues to the thorax. For other entities, the effective validation of soft tissue parameters is hindered by the small numbers of reported mediastinal cases. Many of the treatment modalities have also transferred over, with the key differences resting on the difficulty in achieving complete surgical resection for mediastinal tumors as opposed to those in the somatic soft tissues. Accordingly, systemic drug therapies have emerged as attractive options for some of the mediastinal vascular tumors, such as kaposiform hemangioendothelioma (KHE) and Kaposi sarcoma (KS). The categories presented herein mirror the classification scheme set forth by the 5th Edition WHO Classification of Soft Tissue and Bone Tumors. This review focuses on the biologically aggressive vascular neoplasms while limiting discussion of the benign entities to relevant comparisons in the differential diagnoses. While distinguishing among the benign entities has academic importance, in practice, the stakes are far higher for diagnosing the biologically aggressive tumors given their marked heterogeneity in clinical outcomes. Practical advice for problem areas in pathology will be reviewed alongside tumor pathobiology, including the latest in molecular diagnostics.
血管肿瘤仅占所有影响纵隔肿瘤的一小部分，但由于各实体之间存在显著重叠、分类方案不断演变，以及某些实体不仅在纵隔而且在整个病理学实践中都极为罕见，它们给诊断带来了挑战。大多数血管肿瘤在软组织病理学实践中更为人熟知，从中可以推断出一些临床行为的知识。例如，上皮样血管内皮瘤（EHE）分为两个生物学上不同的类别，这一分类已有效地从躯体软组织应用到了胸部。对于其他实体，由于报道的纵隔病例数量较少，软组织参数的有效验证受到了阻碍。许多治疗方式也已转移应用，关键区别在于与躯体软组织肿瘤相比，纵隔肿瘤实现完全手术切除存在困难。因此，全身药物治疗已成为一些纵隔血管肿瘤的有吸引力的选择，如卡波西样血管内皮瘤（KHE）和卡波西肉瘤（KS）。本文介绍的类别反映了《世界卫生组织软组织和骨肿瘤分类》第5版提出的分类方案。本综述重点关注具有生物学侵袭性的血管肿瘤，同时将良性实体的讨论限于鉴别诊断中的相关比较。虽然区分良性实体具有学术重要性，但在实践中，鉴于具有生物学侵袭性的肿瘤在临床结果上存在显著异质性，诊断这些肿瘤的风险要高得多。本文将结合肿瘤病理生物学，包括最新的分子诊断技术，对病理学中存在问题的领域给出实用建议。
