Division of Hematology/Oncology, Northwestern University, Feinberg School of Medicine, 676 North Saint Clair, Suite 850, Chicago, IL, 60611, USA.
Curr Treat Options Oncol. 2018 Mar 15;19(4):19. doi: 10.1007/s11864-018-0536-y.
Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma, as such it can pose a clinical dilemma based solely on its rarity. Also, the spectrum of disease varies greatly between an indolent disease and aggressive disease with widespread metastases. In our clinical practice, the primary focus has been to get a handle on the aggressive nature of the disease, which will then dictate how urgently one needs to treat the patient. Pathological review with immunohistochemistry and molecular characterization is paramount. Our treatment strategy is watch-and-wait versus active therapy on clinical trial or based on results of prior clinical trials. There is evidence to support the use of chemotherapeutics and targeted therapies specifically focusing on anti-angiogenesis. The current landscape of oncology with the emergence and excitement of immunotherapy could also translate in a role for immunotherapy in this disease. While rare, there is certainly no reason that research and trials for patients with EHE should not remain on utmost importance for those of us who specialize in the treatment of sarcomas.
上皮样血管内皮细胞瘤（EHE）是一种极为罕见的肉瘤，因此仅基于其罕见性，就可能会带来临床难题。此外，疾病的谱在惰性疾病和广泛转移的侵袭性疾病之间差异很大。在我们的临床实践中，主要重点是掌握疾病的侵袭性，然后根据疾病的侵袭性来决定患者的治疗紧迫性。病理学检查包括免疫组化和分子特征分析至关重要。我们的治疗策略是观察和等待，或者根据临床试验或先前临床试验的结果进行积极治疗。有证据支持使用化疗药物和针对抗血管生成的靶向治疗。随着免疫疗法的出现和发展，肿瘤学领域的现状也可能为这种疾病的免疫治疗提供一个机会。虽然罕见，但对于我们这些专门治疗肉瘤的人来说，为 EHE 患者进行研究和临床试验当然应该是最重要的。
