Wong Han Hsi, Gounaris Ioannis, McCormack Ann, Berman Marius, Davidson Dochka, Horan Gail, Pepke-Zaba Joanna, Jenkins David, Earl Helena M, Hatcher Helen M
Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.
Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, CB23 3RE UK.
Clin Sarcoma Res. 2015 Jan 21;5(1):3. doi: 10.1186/s13569-014-0019-2. eCollection 2015.
Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy that leads to heart failure and death without treatment. Here we reviewed the presentation and management of patients treated at a national centre for pulmonary endarterectomy (PEA) and its associated hospital in Cambridge, UK.
Details of PAS patients treated at Papworth and Addenbrooke's Hospitals between 2000 and 2014 were reviewed.
Twenty patients were diagnosed with PAS (11 males, 9 females), with a median age of presentation of 57 years (range 27-77). Presenting symptoms include dyspnoea (20), chest pain/tightness (7), oedema (5), constitutional symptoms (5), cough (3) and haemoptysis (3). Twelve patients were in group III/IV of the NYHA functional classification of symptoms. Initial CT scans were suggestive of thromboembolism in seven patients. Histological findings were of intimal sarcoma (13) and high grade sarcoma NOS (6). Median overall survival (OS) was 17 months. Fourteen patients underwent PEA to relieve vascular obstruction, while six had inoperable and/or metastatic disease. There were three peri-operative deaths. Although there was no difference in median OS between patients who had PEA and those who did not (20 vs 17 months, P = 0.2488), surgery provided significant symptomatic improvement and some with long-term survival. Five patients received post-surgical chemotherapy (anthracycline +/- ifosfamide), and after completion four also had radiotherapy. Patients who received post-operative chemo- and radio-therapy showed a trend towards better survival compared to those who had surgery alone (24 vs 8 months, P = 0.3417). For palliative chemotherapy, partial responses were observed with the VID regimen and pegylated liposomal doxorubicin. Stable disease was achieved in a patient with intimal sarcoma with rhabdomyosarcomatous differentiation on third-line cisplatin and topotecan. The longest surviving patient (102 months) has had PEA, adjuvant epirubicin and radiotherapy. She developed lung metastases 7 years later, which were treated with radiofrequency ablation.
PAS often presents with symptoms mimicking pulmonary hypertension, heart failure or thromboembolic disease. PEA provides good symptomatic relief and in some cases, offers a chance of long-term survival. Although outcome appears to be better when PEA is combined with post-operative chemo- and radio-therapy, further studies are warranted.
肺动脉肉瘤（PAS）是一种罕见但侵袭性强的恶性肿瘤，若不治疗会导致心力衰竭和死亡。在此，我们回顾了在英国剑桥一家国家肺内膜剥脱术（PEA）中心及其相关医院接受治疗的患者的临床表现及治疗情况。
回顾了2000年至2014年间在帕普沃思医院和阿登布鲁克医院接受治疗的PAS患者的详细信息。
20例患者被诊断为PAS（11例男性，9例女性），中位发病年龄为57岁（范围27 - 77岁）。主要症状包括呼吸困难（20例）、胸痛/胸闷（7例）、水肿（5例）、全身症状（5例）、咳嗽（3例）和咯血（3例）。12例患者属于纽约心脏协会（NYHA）症状功能分级的III/IV级。最初的CT扫描显示7例患者提示血栓栓塞。组织学检查结果为内膜肉瘤（13例）和高级别肉瘤，未特指（NOS）（6例）。中位总生存期（OS）为17个月。14例患者接受了PEA以缓解血管阻塞，而6例患有无法手术和/或转移性疾病。有3例围手术期死亡。尽管接受PEA的患者与未接受PEA的患者的中位OS无差异（20个月对17个月，P = 0.2488），但手术显著改善了症状，一些患者获得了长期生存。5例患者接受了术后化疗（蒽环类药物+/-异环磷酰胺），4例完成化疗后还接受了放疗。与仅接受手术的患者相比，接受术后化疗和放疗的患者有生存改善的趋势（24个月对8个月，P = 0.3417）。对于姑息化疗，采用VID方案和聚乙二醇脂质体阿霉素观察到部分缓解。一名内膜肉瘤伴横纹肌肉瘤分化的患者在接受三线顺铂和拓扑替康治疗后病情稳定。存活时间最长的患者（102个月）接受了PEA、辅助表柔比星和放疗。7年后她出现了肺转移，接受了射频消融治疗。
PAS常表现出类似肺动脉高压、心力衰竭或血栓栓塞性疾病的症状。PEA能有效缓解症状，在某些情况下提供长期生存的机会。尽管PEA联合术后化疗和放疗似乎能取得更好的结果，但仍需进一步研究。
