Vignon-Pennamen M D, Rybojad M, Verola O, Puissant A, Morel P
Service de Dermatologie, Hôpital Saint-Louis, Vellefaux, Paris.
Ann Dermatol Venereol. 1997;124(2):165-6.
Epithelioid hemangioendothelioma is an uncommon vascular neoplasm, one of intermediate-grade malignancy. Cutaneous epithelioid hemangioendothelioma is rare and often associated with multiple site involvement.
Three patients with cutaneous EHE without systemic involvement in one case, with skin, liver and lung tumoral lesions in two others cases are reported. One patient is in complete remission after one year. The second patient is stable without treatment after ten years. In the third patient, alpha interferon given for one year don't produce effective results and the lesions do not progress without treatment after eight years of follow-up.
Cutaneous presentation of EHE is quite variable. After the diagnosis is done, systemic involvement must be detected specially in bone, liver or lung. Metastatic spread or mulitcentric origin of the tumor are a matter of controversy. The pathobiologic behavior of EHE is not clearly recognized. Little data are available regarding the results of treatment with alpha interferon.
上皮样血管内皮瘤是一种罕见的血管肿瘤，属于中度恶性肿瘤之一。皮肤上皮样血管内皮瘤罕见，且常伴有多部位受累。
报告了3例皮肤上皮样血管内皮瘤患者，1例无全身受累，另外2例有皮肤、肝脏和肺部肿瘤病变。1例患者1年后完全缓解。第2例患者10年后未经治疗病情稳定。第3例患者接受α干扰素治疗1年无效，随访8年后未经治疗病变未进展。
皮肤上皮样血管内皮瘤的表现差异很大。诊断明确后，必须特别检查是否有全身受累，尤其是骨骼、肝脏或肺部。肿瘤的转移扩散或多中心起源存在争议。上皮样血管内皮瘤的病理生物学行为尚未明确。关于α干扰素治疗结果的数据很少。
