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Presentation and management of pulmonary artery sarcoma.

Wong HH, Gounaris I, McCormack A, Berman M, Davidson D, Horan G, Pepke-Zaba J, Jenkins D, Earl HM, Hatcher HM - Clin Sarcoma Res (2015)

Bottom Line: 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.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).Although outcome appears to be better when PEA is combined with post-operative chemo- and radio-therapy, further studies are warranted.

View Article: PubMed Central - PubMed

Affiliation: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.

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.

No MeSH data available.


Related in: MedlinePlus

Pulmonary artery intimal sarcoma mimicking pulmonary embolism. CT images of two patients who presented with dyspnoea and dependent oedema.
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Fig1: Pulmonary artery intimal sarcoma mimicking pulmonary embolism. CT images of two patients who presented with dyspnoea and dependent oedema.

Mentions: A total of 20 patients were treated at our centres between 2000 and 2014. The patient characteristics are summarised in Table 1. Eleven (55%) patients were male and the age at presentation ranged from 27 to 77 years, with a median of 57 years. The vast majority of patients presented with symptoms consistent with acute or chronic pulmonary hypertension such as dyspnoea (New York Heart Association (NYHA) functional class III and IV in 60% of cases) and chest pain. Some experienced constitutional symptoms of fever, night sweats, anorexia and weight loss. Intimal sarcoma was the histological diagnosis for the majority of the patients (65%), while high-grade sarcoma NOS was found in 35%. We were unable to obtain tumour tissue from an elderly patient with significant co-morbidities despite radiologically-guided biopsy, although his CT scan was unequivocal of PAS. In seven (35%) of the total cases, initial CT images were suggestive of pulmonary emboli (Figure 1).Table 1


Presentation and management of pulmonary artery sarcoma.

Wong HH, Gounaris I, McCormack A, Berman M, Davidson D, Horan G, Pepke-Zaba J, Jenkins D, Earl HM, Hatcher HM - Clin Sarcoma Res (2015)

Pulmonary artery intimal sarcoma mimicking pulmonary embolism. CT images of two patients who presented with dyspnoea and dependent oedema.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4307142&req=5

Fig1: Pulmonary artery intimal sarcoma mimicking pulmonary embolism. CT images of two patients who presented with dyspnoea and dependent oedema.
Mentions: A total of 20 patients were treated at our centres between 2000 and 2014. The patient characteristics are summarised in Table 1. Eleven (55%) patients were male and the age at presentation ranged from 27 to 77 years, with a median of 57 years. The vast majority of patients presented with symptoms consistent with acute or chronic pulmonary hypertension such as dyspnoea (New York Heart Association (NYHA) functional class III and IV in 60% of cases) and chest pain. Some experienced constitutional symptoms of fever, night sweats, anorexia and weight loss. Intimal sarcoma was the histological diagnosis for the majority of the patients (65%), while high-grade sarcoma NOS was found in 35%. We were unable to obtain tumour tissue from an elderly patient with significant co-morbidities despite radiologically-guided biopsy, although his CT scan was unequivocal of PAS. In seven (35%) of the total cases, initial CT images were suggestive of pulmonary emboli (Figure 1).Table 1

Bottom Line: 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.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).Although outcome appears to be better when PEA is combined with post-operative chemo- and radio-therapy, further studies are warranted.

View Article: PubMed Central - PubMed

Affiliation: Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0QQ UK.

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.

No MeSH data available.


Related in: MedlinePlus