title: Presentation and management of pulmonary artery sarcoma.
abstract: BACKGROUND
  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.
  
  
  METHODS
  Details of PAS patients treated at Papworth and Addenbrooke's Hospitals between 2000 and 2014 were reviewed.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.
authors: Han Hsi
authors: HH
authors: Wong
authors: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.
authors: Ioannis
authors: I
authors: Gounaris
authors: Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, CB23 3RE UK.
authors: Ann
authors: A
authors: McCormack
authors: Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, CB23 3RE UK.
authors: Marius
authors: M
authors: Berman
authors: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.
authors: Dochka
authors: D
authors: Davidson
authors: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.
authors: Gail
authors: G
authors: Horan
authors: Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, CB23 3RE UK.
authors: Joanna
authors: J
authors: Pepke-Zaba
authors: Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, CB23 3RE UK.
authors: David
authors: D
authors: Jenkins
authors: University of Cambridge Department of Oncology and NIHR Cambridge Biomedical Research Centre, Hills Road, Cambridge, CB2 0QQ UK.
authors: Helena M
authors: HM
authors: Earl
authors: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.
authors: Helen M
authors: HM
authors: Hatcher
keywords: Intimal sarcoma; Pulmonary artery sarcoma; Pulmonary endarterectomy; Signs; Survival; Symptoms; Treatment
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