Patel Eshan, Kancharla P, Surapaneni B K, Hennrick K, Goldfinger M, Kalavar M
New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, USA.
JSS Medical College, Mysuru, India.
Case Rep Oncol. 2018 Aug 10;11(2):534-540. doi: 10.1159/000491529. eCollection 2018 May-Aug.
Epithelioid hemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that is characterized as intermediate between benign hemangioma and high-grade angiosarcoma affecting 1 in 1,000,000 people worldwide. It has been described throughout the body with lung, liver, skin, and bone being the most frequent sites. Primary EHE of the spine has been reported in 56 cases so far with no correlation of age and sex. Our case highlights a rare clinical presentation, etiopathogenesis, diagnosis, and treatment of EHE of the spine with metastasis to the right atrium. This is the first documented case of EHE of the spine with metastatic spread to the heart treated with bevacizumab leading to resolution of the heart metastatic mass. Further studies are warranted to develop a treatment formula for this rare tumor, to consider combination chemotherapy and new adjuvant targeted immunotherapies to prevent progression of disease.
上皮样血管内皮瘤（EHE）是一种罕见的低级别血管肿瘤，其特征介于良性血管瘤和高级别血管肉瘤之间，全球每100万人中约有1人患病。它可发生于全身各处，其中肺、肝、皮肤和骨骼是最常见的发病部位。迄今为止，已有56例脊柱原发性EHE的报道，且与年龄和性别无关。我们的病例突出了脊柱EHE伴右心房转移的罕见临床表现、病因发病机制、诊断和治疗。这是首例记录在案的脊柱EHE伴心脏转移并接受贝伐单抗治疗后心脏转移瘤消退的病例。有必要进一步开展研究，以制定针对这种罕见肿瘤的治疗方案，考虑联合化疗和新的辅助靶向免疫疗法以预防疾病进展。
