Additional treatment prolonged survival of pulmonary artery sarcoma after surgical resection

Xu, Li; Lu, Weishan; Li, Jianqiang; Wang, Changchun

doi:10.21037/tcr.2020.02.80

Cited by 3 publications

(2 citation statements)

References 51 publications

(58 reference statements)

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“…In clinical practice and published literature, PAS primarily refers to pulmonary artery intimal sarcoma. Due to its rarity and complexity, the optimal treatment approach for PAS remains uncertain ( 8 ). Complete surgical resection is the preferred treatment, but achieving complete resection is challenging due to PAS often involving bilateral pulmonary arteries and spreading along the pulmonary intima to the entire pulmonary artery system ( 9 ).…”

Section: Discussionmentioning

confidence: 99%

Outcomes of pulmonary endarterectomy for patients with pulmonary artery sarcoma

Zhang,

Zhen,

Liu

et al. 2024

Front. Cardiovasc. Med.

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ObjectivePulmonary artery sarcoma (PAS) is an exceedingly rare and insufficiently investigated disease, leading to uncertain in its optimal management. This study aims to present our institutional experience and the outcomes of pulmonary endarterectomy for PAS.MethodsWe gathered clinical characteristics, intraoperative data, postoperative outcomes, and prognosis information from PAS patients who underwent surgical treatment at our institution between December 2016 and September 2023.ResultsA total of 20 patients with PAS underwent pulmonary endarterectomy. The median age of the patients was 52 (IQR 45, 57) years, with 12 patients (60%) being female. Intimal sarcoma was confirmed in 19 patients, while the remaining one was diagnosed with large cell neuroendocrine carcinoma. The perioperative mortality rate was three cases (15%). Follow-up was conducted for a median duration of 14 months (range: 1–61). During the follow-up period, 11 patients experienced recurrence or metastasis, and 5 patients succumbed to the disease. The estimated cumulative survival rates at 1 and 2 years for all 20 patients were 66.4% and 55.3%, respectively.ConclusionPulmonary endarterectomy emerges as a palliative but effective approach for managing PAS, particularly when complemented with postoperative therapies such as chemotherapy and targeted therapy, which collectively contribute to achieving favorable long-term survival outcomes.

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Section: Discussionmentioning

confidence: 99%

Outcomes of pulmonary endarterectomy for patients with pulmonary artery sarcoma

Zhang,

Zhen,

Liu

et al. 2024

Front. Cardiovasc. Med.

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“…As in lung cancer, if lung resection is planed surgical approach, neoadjuvant treatment with radiotherapy and/or chemotherapy may be considered to decrease tumor size and improve resectability. Xu et al [21] reported a meta-analysis including 275 cases. Although surgical resections were different, patients who received adjuvant and/or neo-adjuvant therapy had longer survival rates.…”

Section: Discussionmentioning

confidence: 99%

Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival?

Başar,

Ermerak,

Olgun Yıldızeli

et al. 2023

Turk Gogus Kalp Dama

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Background: In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease. Methods: Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery. Results: Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median followup was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas. Conclusion: Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.

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