Huo Lei, Moran Cesar A, Fuller Gregory N, Gladish Gregory, Suster Saul
Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston 77030-4009, USA.
Am J Clin Pathol. 2006 Mar;125(3):419-24.
We report 12 cases of pulmonary artery sarcoma. The mean age at diagnosis was 48.4 years. Based on histomorphologic features and immunohistochemical findings, 2 tumors were classified as rhabdomyosarcoma, 4 as leiomyosarcoma, 1 as osteogenic sarcoma, 1 as angiosarcoma, and 4 as high-grade sarcoma. All patients underwent surgery. In addition, 7 patients received neoadjuvant or adjuvant therapy. Five patients died 3 to 23 months after surgery. Three patients were still alive at 8, 27, and 68 months at last follow-up. Another 3 patients were alive at 2, 15, and 40 months and then lost to follow-up. The 2 patients with the longest survival (40 months and 68 months) had a diagnosis of leiomyosarcoma. Both patients with rhabdomyosarcoma died at 3 months after surgery. Pulmonary artery sarcoma is an uncommon entity with a poor prognosis. The role of early diagnosis, histologic classification, surgical treatment, and adjuvant therapy in patient outcome is discussed.
我们报告了12例肺动脉肉瘤病例。诊断时的平均年龄为48.4岁。根据组织形态学特征和免疫组化结果，2例肿瘤被分类为横纹肌肉瘤，4例为平滑肌肉瘤，1例为骨肉瘤，1例为血管肉瘤，4例为高级别肉瘤。所有患者均接受了手术。此外，7例患者接受了新辅助或辅助治疗。5例患者在术后3至23个月死亡。在最后一次随访时，3例患者分别在8个月、27个月和68个月时仍存活。另外3例患者分别在2个月、15个月和40个月时存活，随后失访。存活时间最长的2例患者（40个月和68个月）诊断为平滑肌肉瘤。2例横纹肌肉瘤患者均在术后3个月死亡。肺动脉肉瘤是一种罕见的疾病，预后较差。本文讨论了早期诊断、组织学分类、手术治疗和辅助治疗在患者预后中的作用。
