Missed pulmonary artery sarcoma requiring radical excision and pneumonectomy.
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ABSTRACT:
SUBMITTER: Malik MI
PROVIDER: S-EPMC8691900 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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.
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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.
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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.
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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: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:Primary pulmonary artery sarcoma is a rare tumor that is highly fatal. It can be misdiagnosed as acute or chronic pulmonary thromboembolic disease. Herein, we report the case of a 22-year-old woman with a preoperative diagnosis of pulmonary embolism and superior vena caval thrombosis. Intraoperatively, an extensive sarcoma was seen to extend retrograde from the pulmonary artery, past the right ventricle and right atrium, and into the superior vena cava. Surgical resection of the tumor and reconstruction of the central pulmonary arteries, followed by adjuvant chemotherapy, relieved the clinical symptoms. The patient remained free of cancer at 14 months postoperatively. We believe that this is the 1st report of a primary pulmonary artery sarcoma that extended retrograde into the superior vena cava.
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Project description:Pulmonary artery sarcomas are exceptionally rare, and they are often misdiagnosed as chronic pulmonary thromboemboli. Early and accurate diagnosis is crucial to the prognosis of patients who have pulmonary artery sarcomas.Herein, we describe the case of a 74-year-old man who presented with dyspnea and was initially thought to have a pulmonary embolus. Anticoagulation with unfractionated heparin was ineffective. Rare angiographic findings during routine cardiac catheterization led to the diagnosis of a high-grade, nonmyogenic, primary pulmonary artery sarcoma. This case illustrates the usefulness of angiographic findings as an adjunct to conventional diagnostic methods in correctly identifying this rare, aggressive malignancy.
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