Missed pulmonary artery sarcoma requiring radical excision and pneumonectomy.
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ABSTRACT:
SUBMITTER: Malik MI
PROVIDER: S-EPMC8691900 | biostudies-literature | 2021 Dec
REPOSITORIES: biostudies-literature
Publications
Missed pulmonary artery sarcoma requiring radical excision and pneumonectomy.
Malik Mohsyn Imran MI Rabbani Mohamad M Hage Fadi F Inculet Richard R Chu Michael W A MWA
JTCVS techniques 20211012
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Project description:Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.
| S-EPMC4189355 | biostudies-literature
Project description:Introductionand importance: Pulmonary Function Tests (PFTS) is an important tool in the assessment of pulmonary pathologies and preoperative evaluation. Case presentation: A 54-year-old man with history of massive pleurisy in the left thorax, treated by placing chest tube and drainage of bloody effusion, was readmitted for epithelioid mesothelioma. He was then presented with pneumothorax due by a refractory bronchial fistula while having a plural catheter. Based on the consultation, the whole-body bone scan was conducted, and findings demonstrated epithelioid mesothelioma (stage 1) with the refractory fistula for which the patient was candidate for thoracic surgery. Decreased lung capacity was seen by Pulmonary Function Testing - PFTS.Clinical discussionThe novel PFTS Evaluation Technique was designed to measure the true pulmonary capacities in order to evaluate the pulmonary post-operative tolerance. In this technique the chest tube was placed for 4 weeks until the patient reaches mediastinal fixation then the measurements by PFTS were carried out in two steps. First, using an open chest tube and second, using a clamped chest tube. In both steps, the pulmonary capacities were measured and provided to the pulmonologist for consultation.ConclusionIn this case, after acquiring the approval of the specialist depended on PFTS after PFTS Evaluation Technique, the radical extra pleural pneumonectomy surgery was conducted, and the patient was discharged with a good general appearance and treated fistula.
| S-EPMC8605380 | biostudies-literature
Project description: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.
| S-EPMC11250646 | biostudies-literature
Project description:BackgroundUnilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature. The occasional necessity for pneumonectomy is still supported by limited evidence.MethodsA systematic review of the literature was conducted using the PubMed search engine, focusing on UPIPA cases that received pneumonectomy. Thirty-one pertinent articles were selected and included in the analysis. A case reported from our institution was included in the analysis.ResultsWe found 25 adults and seven children affected by UPIPA who received an indication for pneumonectomy, plus an additional case that was reported by our institution. Among adult patients, the predominant indication was hemoptysis (57%), followed by suspected or confirmed lung cancer (23%). Approximately 46% of surgical procedures were classified as urgent or emergent. Postoperative complications were observed in 36% of cases, with no recorded mortality. In pediatric cases, pneumonectomy was primarily a life-saving intervention, performed urgently or emergently in 75% of instances. A possible late complication in pediatric patients involves a mediastinal shift leading to respiratory distress, which may be mitigated using an inflatable prosthesis.ConclusionsPneumonectomy achieves complete resolution of UPIPA symptoms. In the adult population, its primary indication is hemoptysis, with procedures conducted in both elective and urgent/emergent settings. Despite a mortality rate of zero, a notable proportion of patients may experience postoperative complications. In pediatric cases, the clinical presentation varies more extensively, and pneumonectomy is typically reserved for life-threatening situations, emphasizing the need for careful patient selection.
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Project description:Pulmonary artery intimal sarcoma (PAIS) is a very rare tumour. The prevalence of PAIS is estimated to be between 0.001% and 0.003%, but this may be an underestimation because of potential misdiagnosis due to its similar presentation to that of pulmonary thromboembolism. The prognosis is very poor, with median overall survival between 11 and 18 months. We report a case of a 36-year-old man who presented to our cardiac surgery clinic reporting nonspecific symptoms and was found to have PAIS requiring surgical resection and adjuvant chemotherapy. We outline the radiologic features, pathologic characteristics, surgical approach, and chemotherapy treatment utilized.
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Project description:Chest digital dynamic radiography (DDR) is a novel method for evaluating pulmonary perfusion and ventilation. It could depict ventilation-perfusion mismatch in a pulmonary artery sarcoma with severe stenosis in the right pulmonary artery. This report is the first demonstration of ventilation-perfusion mismatch in a malignant neoplasm using DDR.
| S-EPMC7943749 | biostudies-literature
Project description:The present case study reported on a patient initially diagnosed with pulmonary embolism at the First Affiliated Hospital of Fujian Medical University (Fuzhou, China) in May 2021. Furthermore, a relevant literature review was performed. The patient was a 57-year-old Chinese male who presented with dyspnea and wheezing following exercise. Physical examination revealed pulmonary valve second heart sound > aortic valve second heart sound but lack of swelling on both lower limbs, while the imaging results suggested pulmonary artery filling defects. Initially, the patient was diagnosed with pulmonary embolism and was administered anticoagulation treatment, which lasted for 3 months but proved to be ineffective. Subsequent re-examination via chest computed tomography further indicated multiple nodules in the left hilum and lung. Therefore, the patient was hospitalized for lung aspiration biopsy, which led to the final diagnosis of pulmonary artery intimal sarcoma based on the pathological review.
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Project description:Pulmonary artery sarcoma is a rare malignancy with poor prognosis which can be misdiagnosed as pulmonary thromboembolism. We present a case of a middle age woman who initially diagnosed with presumptive pulmonary embolism that was later found to have pulmonary artery sarcoma. Symptoms, pathology, imaging characteristics and available treatments are discussed.
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