Primary Pulmonary Artery Sarcoma

Redmond, M.; Shepard, Jr Jw; Gaffey, TA; Payne, W. Spencer

doi:10.1378/chest.98.3.752

Cited by 23 publications

(5 citation statements)

References 3 publications

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“…There are several reports of PAS that were initially diagnosed as thromboembolism and treated with anticoagulants [63] or IVC filters [65]. Such patients have subsequently developed respiratory failure and been referred for emergency 'pulmonary embolectomy'.…”

Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

“…Historically, the surgical approach was pneumonectomy through a thoracotomy or sternotomy. Redmond et al [65] described pneumonectomy with suction extraction of the tumour fragments from the proximal PA. Although combined pneumonectomy for tumour propagation beyond the first hilar branch or for recognized distal tumour emboli has been described, the higher operative risk should mandate careful consideration in such cases.…”

Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

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Primary cardiac sarcoma

Shanmugam¹

2006

European Journal of Cardio-Thoracic Surgery

132

157

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Primary malignant lesions of the heart are rare. Although myxomas have been extensively described, there is a paucity of large studies on non-myxomatous cardiac tumours. On the other hand, there are several case reports on specific histopathological variants, in small numbers. Consequently there exists no consensus on therapeutic modalities for cardiac sarcomas. The prognosis for these lesions remains dismal, despite the enhanced diagnostic ability of newer technology. The reasons for the dismal prognosis are (1) the advanced tumour stage at presentation, (2) non-specific symptomatolgy, (3) insufficient awareness of these lesions, due to their rarity, (4) delayed diagnosis and/or misdiagnosis, which leads to (5) advanced tumour stage at presentation. Thus a vicious cycle is created. This article addresses these issues, deals with the surgically relevant modes of presentation, rather than the histopathology, and reviews the diagnosis and management options for the various sarcomas, categorized by the site and extent of cardiac involvement. Clinicians should be familiar with the presentation of these tumours and have a high index of suspicion, since the potential for long-term survival following resection does exist. Wide surgical resection remains the cornerstone of sarcoma therapy. Complete characterization of tumour extent using echocardiography and CT/MRI is mandatory to achieve this goal. Radical resections such as 'bench surgery' and transplantation may reduce local recurrence, but the risk of metastatic disease remains. The clinical experience with such approaches is limited. The role of adjuvant therapy is not yet established. In no other field of cardiac surgery would a multidisciplinary approach be more useful, in achieving cure or long-term palliation.

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Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

Section: Pa Sarcoma (Pas)mentioning

confidence: 99%

Primary cardiac sarcoma

Shanmugam¹

2006

European Journal of Cardio-Thoracic Surgery

132

157

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“…Endarterectomy has been widely used in operations since 1990’s ( 19 ), despite as a palliative resection, because it may be suitable to the neoplasm located in the pulmonary trunk, pulmonary artery and proximal part of pulmonary lobar artery. Due to misdiagnosis as pulmonary embolism, PEA is frequently taken in emergency surgery ( 20 ), and the technical details of PEA are similar to chronic thromboembolism pulmonary hypertension (CTEPH) ( 21 ).…”

Section: Discussionmentioning

confidence: 99%

Additional treatment prolonged survival of pulmonary artery sarcoma after surgical resection

Xu¹,

Lu²,

Li³

et al. 2020

Transl Cancer Res TCR

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Background Pulmonary artery sarcoma is too rare for a randomized controlled trial or cohort study. We aimed to perform a retrospective analysis on the therapeutic effect of adjuvant therapy on postoperative survival time. Methods Medline and EMBASE database were searched for articles on pulmonary artery sarcoma published between 1923 and 2018. Age, gender, postoperative overall survival, therapeutic approach, tumor extension, tumor localization, status of resection margins, metastasis, surgical method were extracted as parameters to analyze postoperative overall survival. Results A total of 162 articles and 275 cases are included in analysis. Median postoperative overall survival time was 31 months. Patients who received adjuvant and/or neo-adjuvant therapy were associated with improved survival [hazard ratio (HR) =0.64, P=0.017, 95% confidence interval (CI): 0.45–0.92]. Patients with complete resection or without metastasis had longer postoperative overall survival compared with incomplete resection (HR =0.55, P=0.002, 95% CI: 0.37–0.79) or with metastasis (HR =6.01, P=0.000, 95% CI: 3.33–10.67). Subgroup analysis suggested chemotherapy was related with longer postoperative overall survival (HR =0.63, P=0.015, 95% CI: 0.43–0.91), especially for patients with incomplete resection (HR =0.53, P=0.025, 95% CI: 0.31–0.92) and metastasis (HR =0.44, P=0.000, 95% CI: 0.28–0.68). Conclusions Radical surgery offers the only chance to cure pulmonary artery sarcoma. Palliative and aggressive surgery can relieve the symptoms and extend the life expectance. Patients can benefit from adjuvant and/or neo-adjuvant chemotherapy, especially those who have metastasis or undergo incomplete resection.

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“…Treatment of PPAS has included endarterectomy and pneumonectomy 10 and segmental arterial resection with grafting. 7 The role of adjuvant radiotherapy or chemotherapy has not been adequately evaluated.…”

Section: Discussionmentioning

confidence: 99%