Division of Thoracic Surgery, Cancer Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, People's Republic of China.
Med Oncol. 2010 Mar;27(1):49-54. doi: 10.1007/s12032-009-9169-0. Epub 2009 Mar 11.
This report describes a case of pulmonary epithelioid haemangioendothelioma presented as multi-organ metastases including lung, liver and bone in a 56-year-old woman with an initial diagnosis made with thoracoscopic wedge biopsy. The diagnosis is confirmed through immunohistochemistry. This is a rare disease, with approximately 90 cases described in the English literature and approximately 10 cases reported in China. The case of PEH presented as multiple pulmonary nodules and metastasing to liver and bone is rare in the English literature. The rarity of this condition, the lack of clear standards for treatment, and the partial-to-complete spontaneous regression of EHE seen in some patients up to 15 years from initial detection makes it difficult to decide on the most appropriate treatment. This report may contribute to the data on clinical findings and natural history of this rare tumor.
本报告描述了一例肺上皮样血管内皮细胞瘤病例,该患者为 56 岁女性,最初诊断为胸腔镜楔形活检,表现为多器官转移,包括肺、肝和骨。通过免疫组织化学检查确诊。这是一种罕见疾病,在英文文献中约有 90 例描述,在中国约有 10 例报道。英语文献中罕见肺上皮样血管内皮细胞瘤表现为多发性肺结节并转移至肝和骨。这种疾病较为罕见,治疗标准不明确,部分患者至初始发现后 15 年完全自发消退,这使得很难确定最合适的治疗方法。本报告可能有助于提供该罕见肿瘤的临床发现和自然病史数据。