Bally Olivia, Tassy Louis, Richioud Bertrand, Decouvelaere Anne-Valérie, Blay Jean-Yves, Derbel Olfa
Sarcoma Unit, Centre Léon Bérard, 28, Laennec street, 69008 Lyon, France.
Clin Sarcoma Res. 2015 Apr 23;5:12. doi: 10.1186/s13569-014-0018-3. eCollection 2015.
Epithelioid hemangioendothelioma is a rare connective tissue tumor of vascular origin. It is most commonly found in young to middle aged women, and its clinical behavior is remakably variable from an indolent metastatic tumor to an aggressive rapidly growing neoplasm. Most tumors are diagnosed in an advanced unresectable phase and when clinically aggressive, require systemic cytotoxic treatment of sarcoma. Then, the 5-year survival rate after chemotherapy does not exceed 30%. Antiangiogenics are active in selected sarcoma subtypes: pazopanib, the only anti angiogenic registered agent for sarcoma provides a median PFS of 4.5 months only in the pivotal study. Their activity in EHE has been reported but long term outcome of these patients remain unreported. We report a case of a female patient with HEH who was treated with pazopanib for almost 8 years. Pazopanib therapy resulted in clinical improvement of symptoms and durable stabilization of liver tumors and lung lesions.
Pazopanib is a promising therapeutic option in patients with HEH.
上皮样血管内皮瘤是一种罕见的血管源性结缔组织肿瘤。它最常见于年轻至中年女性，其临床行为差异很大，从惰性转移性肿瘤到侵袭性快速生长的肿瘤。大多数肿瘤在晚期无法切除阶段被诊断出来，当临床上具有侵袭性时，需要进行肉瘤的全身细胞毒性治疗。然后，化疗后的5年生存率不超过30%。抗血管生成药物在某些肉瘤亚型中具有活性：帕唑帕尼是唯一一种被批准用于肉瘤的抗血管生成药物，在关键研究中仅提供4.5个月的中位无进展生存期。其在上皮样血管内皮瘤中的活性已有报道，但这些患者的长期预后仍未报道。我们报告一例上皮样血管内皮瘤女性患者，接受帕唑帕尼治疗近8年。帕唑帕尼治疗导致症状临床改善，肝脏肿瘤和肺部病变持久稳定。
帕唑帕尼是上皮样血管内皮瘤患者有前景的治疗选择。
