Noguchi Takuro, Gomi Daisuke, Fukushima Toshirou, Ozawa Takesumi, Kobayashi Takashi, Sekiguchi Nodoka, Mamiya Keiko, Koizumi Tomonobu
Department of Comprehensive Cancer Therapy, Shinshu University School of Medicine, Matsumoto, Japan.
Case Rep Oncol. 2019 Feb 20;12(1):192-198. doi: 10.1159/000496334. eCollection 2019 Jan-Apr.
Pulmonary artery intimal sarcoma is a rare malignant tumor. Due to its low prevalence, little is known about efficacious systemic chemotherapies in cases where the tumors are unresectable or metastatic. In addition, the location of the disease can contribute to poor survival regardless of the response to therapy, as the tumor's position can cause pulmonary artery hypertension either rapidly or chronically. We encountered a case of unresectable pulmonary artery intimal sarcoma with lung metastases. Treatment with several cytotoxic agents resulted in prolonged survival of 14.2 months. Here, we report the clinical course of this case and present a review of the relevant literature.
肺动脉内膜肉瘤是一种罕见的恶性肿瘤。由于其发病率低，对于肿瘤无法切除或发生转移的病例，有效的全身化疗知之甚少。此外，无论治疗反应如何，疾病的位置都可能导致生存率低下，因为肿瘤的位置可迅速或慢性地引起肺动脉高压。我们遇到了一例伴有肺转移的无法切除的肺动脉内膜肉瘤病例。使用多种细胞毒性药物治疗使生存期延长至14.2个月。在此，我们报告该病例的临床病程并对相关文献进行综述。
