Tomassen Tess, Versleijen-Jonkers Yvonne M H, Hillebrandt-Roeffen Melissa H S, Van Cleef Patricia H J, van Dalen Thijs, Weidema Marije E, Desar Ingrid M E, Flucke Uta, van Gorp Joost M
Department of Pathology, Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The Netherlands.
Department of Medical Oncology, Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The Netherlands.
Cancers (Basel). 2023 Jun 23;15(13):3304. doi: 10.3390/cancers15133304.
Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma with variable aggressive clinical behavior. In this retrospective study, we aimed to investigate prognostic factors based on clinicopathologic findings in a molecularly/immunohistochemically confirmed nationwide multicenter cohort of 57 EHE cases. Patients had unifocal disease ( = 29), multifocal disease ( = 5), lymph node metastasis ( = 8) and/or distant metastasis ( = 15) at the time of diagnosis. The overall survival rate was 71.4% at 1 year and 50.7% at 5 years. Survival did not correlate with sex, age or histopathological parameters. No survival differences were observed between multifocal and metastatic disease, suggesting that multifocality represents early metastases and treatment options are limited in comparison to unifocal disease. In unifocal tumors, survival could be predicted using the risk stratification model of Shibayama et al., dividing the cases into low- ( = 4), intermediate- ( = 15) and high- ( = 3) risk groups. No clinical or histopathological parameters were associated with progressive unifocal disease course. Lymph node metastases at the time of diagnosis occurred in 14.0% of the cases and were mainly associated with tumor localization in the head and neck area, proposing lymph node dissection. In conclusion, our results demonstrate the aggressive behavior of EHE, emphasize the prognostic value of a previously described risk stratification model and may provide new insights regarding tumor focality, therapeutic strategies and prognosis.
上皮样血管内皮瘤（EHE）是一种极其罕见的血管肉瘤，临床侵袭性行为各异。在这项回顾性研究中，我们旨在基于临床病理结果，对一个经分子/免疫组化确诊的全国多中心队列中的57例EHE病例的预后因素进行调查。诊断时，患者有单发病灶（n = 29）、多发病灶（n = 5）、淋巴结转移（n = 8）和/或远处转移（n = 15）。1年总生存率为71.4%，5年为50.7%。生存率与性别、年龄或组织病理学参数无关。多发病灶和转移性疾病之间未观察到生存差异，这表明多发病灶代表早期转移，与单发病灶相比，治疗选择有限。在单发病灶肿瘤中，可使用Shibayama等人的风险分层模型预测生存率，将病例分为低风险（n = 4）、中风险（n = 15）和高风险（n = 3）组。没有临床或组织病理学参数与单发病灶疾病的进展过程相关。诊断时14.0%的病例发生淋巴结转移，主要与头颈部肿瘤定位有关，建议进行淋巴结清扫。总之，我们的结果证明了EHE的侵袭性行为，强调了先前描述风险分层模型的预后价值，并可能为肿瘤灶性、治疗策略和预后提供新的见解。
