Yongxin Han1, Yanan Zhen2, Xiaopeng Liu2, Xia Zheng2, Jianbin Zhang2, Zhenguo Zhai3, Jun Duan4, Yajun Zhang5, Peng Liu6,7. 1. Peking University China-Japan Friendship School of Clinical Medicine, Beijing, 100029, China. 2. Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China. 3. Respiratory Department, China-Japan Friendship Hospital, Beijing, China. 4. Surgical Intensive Care Unit, China-Japan Friendship Hospital, Beijing, China. 5. Surgical Anesthesia Department, China-Japan Friendship Hospital, Beijing, China. 6. Peking University China-Japan Friendship School of Clinical Medicine, Beijing, 100029, China. sdbzhyx@163.com. 7. Department of Cardiovascular Surgery, China-Japan Friendship Hospital, Cherry Park East Street, Chaoyang District, Beijing, 100029, China. sdbzhyx@163.com.
Abstract
OBJECTIVE: Primary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS. METHODS: From September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data. RESULTS: All of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7-28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve. CONCLUSIONS: PAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.
OBJECTIVE:Primary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS. METHODS: From September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data. RESULTS: All of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7-28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve. CONCLUSIONS:PAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.
Authors: Hong Kwan Kim; Yong Soo Choi; Kwhanmien Kim; Young Mog Shim; Kiick Sung; Young Tak Lee; Pyo Won Park; Jhingook Kim Journal: Eur J Cardiothorac Surg Date: 2008-03-04 Impact factor: 4.191