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Presentation and management of pulmonary artery sarcoma

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 Cambridg...

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Autores principales: Wong, Han Hsi, Gounaris, Ioannis, McCormack, Ann, Berman, Marius, Davidson, Dochka, Horan, Gail, Pepke-Zaba, Joanna, Jenkins, David, Earl, Helena M, Hatcher, Helen M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4307142/
https://www.ncbi.nlm.nih.gov/pubmed/25628857
http://dx.doi.org/10.1186/s13569-014-0019-2
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author Wong, Han Hsi
Gounaris, Ioannis
McCormack, Ann
Berman, Marius
Davidson, Dochka
Horan, Gail
Pepke-Zaba, Joanna
Jenkins, David
Earl, Helena M
Hatcher, Helen M
author_facet Wong, Han Hsi
Gounaris, Ioannis
McCormack, Ann
Berman, Marius
Davidson, Dochka
Horan, Gail
Pepke-Zaba, Joanna
Jenkins, David
Earl, Helena M
Hatcher, Helen M
author_sort Wong, Han Hsi
collection PubMed
description 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.
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spelling pubmed-43071422015-01-28 Presentation and management of pulmonary artery sarcoma Wong, Han Hsi Gounaris, Ioannis McCormack, Ann Berman, Marius Davidson, Dochka Horan, Gail Pepke-Zaba, Joanna Jenkins, David Earl, Helena M Hatcher, Helen M Clin Sarcoma Res Research 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. BioMed Central 2015-01-21 /pmc/articles/PMC4307142/ /pubmed/25628857 http://dx.doi.org/10.1186/s13569-014-0019-2 Text en © Wong et al.; licensee BioMed Central. 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
Wong, Han Hsi
Gounaris, Ioannis
McCormack, Ann
Berman, Marius
Davidson, Dochka
Horan, Gail
Pepke-Zaba, Joanna
Jenkins, David
Earl, Helena M
Hatcher, Helen M
Presentation and management of pulmonary artery sarcoma
title Presentation and management of pulmonary artery sarcoma
title_full Presentation and management of pulmonary artery sarcoma
title_fullStr Presentation and management of pulmonary artery sarcoma
title_full_unstemmed Presentation and management of pulmonary artery sarcoma
title_short Presentation and management of pulmonary artery sarcoma
title_sort presentation and management of pulmonary artery sarcoma
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4307142/
https://www.ncbi.nlm.nih.gov/pubmed/25628857
http://dx.doi.org/10.1186/s13569-014-0019-2
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