Kounami Shinji, Tsujimoto Hiroshi, Ichikawa Takayuki, Yoshiyama Megumi, Yuzaki Mitsuru, Nishimura Yoshiharu, Suzuki Hiroyuki
Department of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Division of Pediatrics, Hidaka General Hospital, Gobo, Japan.
Case Rep Oncol. 2017 Jun 23;10(2):564-570. doi: 10.1159/000477657. eCollection 2017 May-Aug.
Pulmonary vein leiomyosarcoma is extremely rare and has a poor prognosis. Surgical resection with a wide margin seems to offer the only chance of cure. The role of adjuvant therapy is controversial, and the exact efficacy of chemotherapy has not been observed. In this report, we present an 18-year-old male patient with pulmonary vein leiomyosarcoma in whom the use of paclitaxel (PAX) proved to be effective. Because the tumor originated from the left superior pulmonary vein and diffused into the left atrial wall and the junction of the right superior pulmonary vein and left atrium, the en bloc excision of the tumor was impossible. The first-line chemotherapy, including ifosfamide, doxorubicin, and dacarbazine, in conjugation with radiation therapy could not demonstrate any effect on the tumor size. However, the following PAX-containing regimen provided complete regression of the tumor. After PAX-based high-dose chemotherapy with autologous peripheral blood stem cell transplantation, the patient showed complete remission for 2 years. Although he suffered metastatic recurrences and died 4 years after the onset of symptoms, our patient's clinical course clearly reveals the efficacy of PAX.
肺静脉平滑肌肉瘤极为罕见，预后较差。广泛切缘的手术切除似乎是唯一的治愈机会。辅助治疗的作用存在争议，化疗的确切疗效尚未明确。在本报告中，我们介绍了一名18岁的男性肺静脉平滑肌肉瘤患者，在该患者中使用紫杉醇（PAX）被证明是有效的。由于肿瘤起源于左上肺静脉并扩散至左心房壁以及右上肺静脉与左心房的交界处，无法进行肿瘤整块切除。一线化疗，包括异环磷酰胺、多柔比星和达卡巴嗪，并联合放射治疗，对肿瘤大小未显示出任何效果。然而，随后含PAX的方案使肿瘤完全消退。在基于PAX的高剂量化疗联合自体外周血干细胞移植后，患者实现了2年的完全缓解。尽管他出现了转移性复发并在症状出现4年后死亡，但我们患者的临床病程清楚地显示了PAX的疗效。
