Presentation and management of pulmonary artery sarcoma

Clinical Sarcoma Research

Helen M Hatcher³  Helena M Earl¹  David Jenkins²  Joanna Pepke-Zaba²  Gail Horan³  Dochka Davidson³  Marius Berman²  Ann McCormack²  Ioannis Gounaris³  Han Hsi Wong³ 

[1] University of Cambridge Department of Oncology and NIHR Cambridge Biomedical Research Centre, Hills Road, Cambridge CB2 0QQ, UK;Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge CB23 3RE, UK;Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust, Box 193, Cambridge Biomedical Campus, Hills Road, Cambridge CB2 0QQ, UK

关键词: Survival;    Treatment;    Pulmonary endarterectomy;    Symptoms;    Signs;    Intimal sarcoma;    Pulmonary artery sarcoma;   

Others  :  1092794
DOI  :  10.1186/s13569-014-0019-2

 received in 2014-10-20, accepted in 2014-12-18,  发布年份 2015

【 摘 要 】

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.

【 授权许可】

   
2015 Wong et al.; licensee BioMed Central.

【 图 表 】

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【 参考文献 】

• [1]Mandelstamm M: Über primäre Neubildungen des Herzens. Virchows Arch 1923, 245:43-54.
• [2]Blackmon SH, Rice DC, Correa AM, Mehran R, Putnam JB, Smythe WR, et al.: Management of primary pulmonary artery sarcomas. Ann Thorac Surg 2009, 87:977-984.
• [3]Mussot S, Ghigna MR, Mercier O, Fabre D, Fadel E, Le Cesne A, et al.: Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg 2013, 43:787-793.
• [4]Gan HL, Zhang JQ, Zhou QW, Xiao W, Gao YM, Liu S, et al.: Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg 2011, 142:1469-1472.
• [5]Grazioli V, Vistarini N, Morsolini M, Klersy C, Orlandoni G, Dore R, D’Armini AM: Surgical treatment of primary pulmonary artery sarcoma. J Thorac Cardiovasc Surg 2014, 148:113-118.
• [6]Bacha EA, Wright CD, Grillo HC, Wain JC, Moncure A, Keel SB, et al.: Surgical treatment of primary pulmonary sarcomas. Eur J Cardiothorac Surg 1999, 15:456-460.
• [7]Huo L, Moran CA, Fuller GN, Gladish G, Suster S: Pulmonary artery sarcoma: a clinicopathologic and immunohistochemical study of 12 cases. Am J Clin Pathol 2006, 125:419-424.
• [8]Kim HK, Choi YS, Kim K, Shim YM, Sung K, Lee YT, et al.: Surgical treatment for pulmonary artery sarcoma. Eur J Cardiothorac Surg 2008, 33:712-716.
• [9]Chhaya NC, Goodwin AT, Jenkins DP, Pepke-Zaba J, Dunning JJ: Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg 2006, 131:1410-1411.
• [10]Mayer F, Aebert H, Rudert M, Königsrainer A, Horger M, Kanz L, et al.: Primary malignant sarcomas of the heart and great vessels in adult patients–a single-center experience. Oncologist 2007, 12:1134-1142.
• [11]Tavora F, Miettinen M, Fanburg-Smith J, Franks TJ, Burke A: Pulmonary artery sarcoma: a histologic and follow-up study with emphasis on a subset of low-grade myofibroblastic sarcomas with a good long-term follow-up. Am J Surg Pathol 2008, 32:1751-1761.
• [12]Xue H, Wu QY: Surgical treatment of primary pulmonary artery sarcoma. Chin Med J 2011, 124:461-463.
• [13]Yi CA, Lee KS, Choe YH, Han D, Kwon OJ, Kim S: Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease. J Comput Assist Tomogr 2004, 28:34-39.
• [14]Gan HL, Zhang JQ, Huang XY, Yu W: The wall eclipsing sign on pulmonary artery computed tomography angiography is pathognomonic for pulmonary artery sarcoma. PLoS One 2013, 8:e83200.
• [15]Lee EJ, Moon SH, Choi JY, Lee KS, Choi YS, Choe YS, et al.: Usefulness of fluorodeoxyglucose positron emission tomography in malignancy of pulmonary artery mimicking pulmonary embolism. ANZ J Surg 2013, 83:342-347.
• [16]Lee DH, Jung TE, Lee JH, Shin DG, Park WJ, Choi JH: Pulmonary artery intimal sarcoma: poor 18F-fluorodeoxyglucose uptake in positron emission computed tomography. J Cardiothorac Surg 2013, 8:40. BioMed Central Full Text
• [17]Fernández-Golfín C, Escribano P, Cortina J, Tello R, Hernández F, López-Rios F, et al.: Management of primary pulmonary artery sarcoma: experience of a single center. Angiology 2008, 59:636-639.
• [18]Tanaka A, Shirasaka T, Okada K, Okita Y. Aggressive multiple surgical interventions to pulmonary artery sarcoma. Eur J Cardiothorac Surg. 2015;47:384-5.
• [19]Linden PA, Morgan JA, Couper GS: Seven-year disease-free survival after radical pneumonectomy for a pulmonary artery sarcoma. J Thorac Cardiovasc Surg 2013, 146:e17-e18.
• [20]Nakahira A, Ogino H, Sasaki H, Katakami N: Long-term survival of a pulmonary artery sarcoma produced by aggressive surgical resection and adjuvant chemoradiotherapy. Eur J Cardiothorac Surg 2007, 32:388-390.
• [21]Chaubey S, Benson C, Khan H, Monaghan M, Shah AM, Judson I, et al.: Six-year survival of a patient with pulmonary artery angiosarcoma. Asian Cardiovasc Thorac Ann 2012, 20:728-730.
• [22]Uchida A, Tabata M, Kiura K, Tanimoto Y, Kanehiro A, Aoe M, et al.: Successful treatment of pulmonary artery sarcoma by a two-drug combination chemotherapy consisting of ifosfamide and epirubicin. Jpn J Clin Oncol 2005, 35:417-419.
• [23]Chen X, Ren S, Li A, Zhou C: A case report of chemo-sensitive intimal pulmonary artery sarcoma. Cell Biochem Biophys 2014, 68:153-157.
• [24]Xu Y, Wang K, Geng Y, Shao Y, Yin Y: A case of intimal sarcoma of the pulmonary artery successfully treated with chemotherapy. Int J Clin Oncol 2012, 17:522-527.
• [25]Said SM, Sundt TM, Garces YI, Wigle DA: 5-year survival after multiple repeat metastasectomy for pulmonary artery angiosarcoma. Ann Thorac Surg 2011, 91:e49-e51.
• [26]Choi YM, Jang EK, Ahn SH, Jeon MJ, Han JM, Kim SC, et al.: Long-term survival of a patient with pulmonary artery intimal sarcoma after sequential metastasectomies of the thyroid and adrenal glands. Endocrinol Metab (Seoul) 2013, 28:46-49.
• [27]Van der Graaf WTA, Blay JY, Chawla SP, Kim DW, Bui-Nguyen B, Casali PG, et al.: Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 2012, 379:1879-1886.
• [28]Bode-Lesniewska B, Zhao J, Speel EJ, Biraima AM, Turina M, Komminoth P, et al.: Gains of 12q13-14 and overexpression of mdm2 are frequent findings in intimal sarcomas of the pulmonary artery. Virchows Arch 2001, 438:57-65.
• [29]Neuville A, Collin F, Bruneval P, Parrens M, Thivolet F, Gomez-Brouchet A, et al.: Intimal sarcoma is the most frequent primary cardiac sarcoma: clinicopathologic and molecular retrospective analysis of 100 primary cardiac sarcomas. Am J Surg Pathol 2014, 38:461-469.
• [30]Dewaele B, Floris G, Finalet-Ferreiro J, Fletcher CD, Coindre J-M, Guillou L, et al.: Coactivated platelet-derived growth factor receptor {alpha} and epidermal growth factor receptor are potential therapeutic targets in intimal sarcoma. Cancer Res 2010, 70:7304-7314.
• [31]Zhao J, Roth J, Bode-Lesniewska B, Pfaltz M, Heitz PU, Komminoth P: Combined comparative genomic hybridization and genomic microarray for detection of gene amplifications in pulmonary artery intimal sarcomas and adrenocortical tumors. Genes Chromosomes Cancer 2002, 34:48-57.