Leiomyosarcoma Of Pulmonary Artery Research Articles

Outcomes after surgical resection of primary cardiac sarcomas

Aim. To assess the immediate and long-term results of surgical treatment of patients with primary cardiac sarcomas.Methods. In the period from 2013 to 2020, five patients (two men and three women) of different age groups (from 10 to 54 years old) with primary cardiac sarcomas various localisations had been operated at our hospital. In all the patients, the operation was performed from the median sternotomy with cardiopulmonary bypass, normothermia and crystalloid cardioplegia.Results. The average age of the patients was 32 ± 12 years. Two patients had leiomyosarcoma (of right atrium and pulmonary artery), two had angiosarcoma of the right atrium and one was diagnosed with pleomorphic left atrial sarcoma. Only in two patients, the lesion was limited to one chamber; in other cases, the tumour exhibited aggressive infiltrative growth and invasion into the inferior vena cava, myocardium of the left ventricle, contralateral cardiac chambers, pericardium and parietal pleura. Three patients underwent radical surgery with R0 resection, two others underwent cytoreductive surgery with R2 resection owing to the prevalence of the process and the technical impossibility of radical resection. We did not record any in-hospital mortality. Postoperative chemotherapy was administered to four patients. At follow up, (median 18.8 ± 11.7 month), one patient was alive (follow-up 35 months) and was undergoing chemotherapy owing to local recurrence of the disease. One patient with pulmonary leiomyosarcoma underwent re-operation because of local recurrence 1 year after the primary surgery. All the patients died because of disease progression. The average postoperative survival duration in those who died was 14.8 ± 8.1 month.Conclusion. Despite effective early results, long-term survival and tumour-free course were disappointing owing to the initial prevalence of the disease course, the complexity of surgical procedures and the impossibility of radical resection in two cases. However, a multimodality treatment of this pathology, including radical surgical resection of the tumour (R0) along with the modern neoadjuvant and adjuvant chemotherapy, and in some cases, radiotherapy, can improve the survival duration. Received 18 December 2020. Revised 4 March 2021. Accepted 10 March 2021. Funding: The study did not have sponsorship. Conflict of interest: Authors declare no conflict of interest.

An unusually pulmonary embolism

Case report Pulmonary embolism is usually caused by blood clots that travel to the lungs from the legs or other parts and me life-threatening. More rarely, pulmonary vascular obstruction may be due to a tumor. We report the case of a 68-year old man who presented with symptoms and signs suggesting high-risk acute pulmonary embolism. The computed tomography pulmonary angiography initially confirmed the diagnosis showing filling defects within the pulmonary vasculature. Fibrinolysis treatment allowed hemodynamic restoration. Unfortunately, one month later, the patient presented with cardiogenic shock due to acute pulmonary obstruction. A rescue embolectomy was decided and the surgeon discovered a tumor instead of a clot. Anatomopathology confirmed an unusual pulmonary artery leiomyosarcoma not initially suspected.

Pulmonary artery leiomyosarcoma: A clinical dilemma

Leiomyosarcomas are rare neoplasms of the smooth muscles. Primary pulmonary leiomyosarcomas, which constitute approximately 0.2%–0.5% of all primary lung malignancies, are extremely rare and highly lethal. They may originate from the smooth muscle cells of the bronchial wall, the blood vessels, or the pulmonary interstitium, and their rare occurrence, localization, and nonspecific clinical symptoms mean that correct diagnosis and proper management are often delayed. Here, we report a rapidly growing primary pulmonary leiomyosarcoma, which invaded the right atrium, vena cava superior, mediastinum, right hilar area, and left pulmonary artery within 4 months. On histopathology, a transthoracic needle biopsy of the mass confirmed leiomyosarcoma, and delayed presentation meant that there was a local spread to the neighboring structures at the time of diagnosis.

Treatment of a recurrent pulmonary artery leiomyosarcoma with a combination of surgery, chemotherapy, and radiotherapy: A case report and literature review.

Pulmonary artery (PA) leiomyosarcoma (PAL) is a rare but extremely malignant tumor of the cardiovascular system, which is often misdiagnosed as pulmonary thromboembolism. Although the early detection and surgical resection improves patient survival, the benefits of adjuvant therapy are not well understood. The current study presents the case of a patient with primary PAL who underwent surgical resection along with three courses of chemotherapy. Despite these interventions, the patient experienced recurrence of the PAL within four months following treatment. The patient received localized radiotherapy and subsequently achieved a stable disease state. This case indicates that radiotherapy may offer a benefit to PAL patients, particularly those who do not respond to chemotherapy.

Pulmonary Hypertension due to a Pulmonary Artery Leiomyosarcoma: A Case Report

Background. Primary pulmonary artery sarcomas are very rare and their histologic type, called leiomyosarcoma, is even rarer. Case Report. A 64-year-old woman presented with progressive weakness, fatigue, malaise, and dyspnea, and a marked elevation of pulmonary artery pressure was admitted. She was initially diagnosed with chronic pulmonary thromboembolism and chest computed tomography (CT) scan revealed that lobulated heterogeneous left hilar mass extended to precarinal and subcarinal space. MRI demonstrated a polypoid lesion at trunk with extension to left main pulmonary artery and its first branch. She was operated, a yellowish-shiny solid mass in pulmonary trunk was seen intraoperatively, and pulmonary endarterectomy was performed. Her tumor was pathologically diagnosed as pulmonary artery leiomyosarcoma. She died 3 months later after one chemotherapy course. Conclusion. Initially, the patient underwent surgery due to pulmonary embolism but, during the operation, the observed mass increased the probability of pulmonary artery neoplasm. Clinicians must consider pulmonary artery sarcoma when making the differential diagnosis for patients with pulmonary arteries masses. In addition to clinical prediction scores and CT and MRI findings to identify the patients with pulmonary artery sarcoma, PET scanning is the diagnosis of choice in differentiating embolism and neoplasm and is strongly recommended in these patients.

Primary Pulmonary Artery Leiomyosarcoma Mimicking Pulmonary Embolism

SESSION TYPE: Cancer Case Report Posters I

The evaluation of combined CT pulmonary and bronchial arteriography for the diagnosis of hemoptysis

To establish the method of multi-slice spiral CT pulmonary angiography (CTPA)combined with bronchial angiography (CTBA) and to evaluate its value for diagnosis and treatment in hemoptysis. After contrast material was administered intravenously, a 16-detector row helical CT scanner (Light Speed Ultra 16; GE Medical Systems) was used to complete CTPA first with a scan delay time of 12 - 16 s. Then CTBA was carried out, with a scan delay time of 26 - 28 s. The images were reformatted to evaluate the pulmonary artery and bronchial artery (BA). In 36 cases of hemoptysis, CTPA showed 7 pulmonary arterial abnormalities (3 cases with pulmonary embolism, 4 cases with one of pulmonary artery abnormity, primary pulmonary artery leiomyosarcoma, loss of sharpness of pulmonary artery or occlusion of right-inferior lung artery). In the 36 cases, CTBA showed 37 right BAs (11 tortuosity and thickening), 40 left right BAs (10 tortuosity and thickening) and 3 non-bronchial systemic arteries (1 from abdominal aorta tortuosity and thickening). Abnormal vessels had a close relation with pulmonary diseases. With this method CTPA and CTBA can be completed in a single procedure and abnormal pulmonary arteries and bronchial arteries can be shown clearly. This procedure maybe of important value for the diagnosis and treatment of hemoptysis.

Surgical Treatment of Pulmonary Artery Leiomyosarcoma: A Good Survival Without Adjuvant Therapy

Primary pulmonary artery leiomyosarcoma is a rare tumor that can be misdiagnosed as acute or chronic pulmonary thromboembolic disease. In this report, we present a case of a 58-year-old woman initially diagnosed with chronic thromboembolic pulmonary disease, but who was later found to have pulmonary artery leiomyosarcoma. A complete mass resection was performed surgically. The definitive pathologic examination was consistent with pulmonary artery leiomyosarcoma. Although the patient did not receive adjuvant therapy after the surgery, she had a good outcome and was free of disease 8 months after surgery.

The first case of 15 years survival after radical surgical excision and right ventricular outflow tract reconstruction for primary leiomyosarcoma of pulmonary artery

Leiomyosarcoma of the pulmonary artery is a extremely rare and highly lethal malignancy arising from the multipotential mesenchymal cell of the intima of the pulmonary artery. Diagnosis is difficult due to their unspecific symptoms, which are frequently confused with other clinical conditions such as pulmonary embolism.

Pulmonary artery leiomyosarcoma: an unusual cause of shortness of breath

Primary leiomyosarcomas of the pulmonary artery are very rare tumours with 30 cases reported in the English literature. Herein, we report the first case with evidence of a renal metastasis. The tumour was discovered at autopsy in a 61-year-old woman who had presented with shortness of breath and chest pain. It was located in the pulmonary trunk with extension into the adjacent pericardial cavity. Separate nodules were found in the pulmonary valve, the left pulmonary artery and the left kidney. On microscopic examination, the tumour was composed of spindle cells with cigar-shaped nuclei immunoreactive for vimentin, desmin, CD68 and α-smooth muscle actin. Primary pulmonary artery leiomyosarcoma is easily confused clinically with pulmonary thromboembolism and most cases are diagnosed post mortem. Most tumours arise in the pulmonary trunk and metastases are only rarely described. The management of patients with this rare tumour depends on early surgical resection and the prognosis is unfavourable.

Pulmonary Artery Leiomyosarcoma Successfully Treated by Right Pneumonectomy

A 79-year-old woman had a primary tumor of the pulmonary artery, which was initially diagnosed as chronic pulmonary thromboembolism. Multislice angio-computed tomography showed a solid mass in the right pulmonary artery. Radical resection of the tumor was achieved by right pneumonectomy via a transsternal transpericardial approach. The patient was alive and free of disease 36 months after surgery.

Thromboembolism in Pulmonary Artery Sarcoma

Pulmonary artery sarcoma, although rare, must be considered in the differential diagnosis of pulmonary thromboembolism. Clinically and radiologically, it may imitate pulmonary embolism, making diagnosis difficult and delaying treatment. Patients often have no symptom resolution despite therapeutic anticoagulation. Visualization of filling defects within a pulmonary artery on contrast-enhanced CT cannot reliably differentiate between pulmonary thromboembolism and malignant lesions like leiomyosarcoma. FDG PET-CT offers the potential for identification of malignant lesions. The authors report a case with pulmonary artery thromboembolism due to thrombi formed on a pulmonary artery leiomyosarcoma. Integrated FDG PET-CT showed no FDG-uptake along the major part of the filling defect within the right main pulmonary artery suggesting blood clot and increased uptake along the posterior wall of the right main pulmonary artery and the left lower lobar artery suggesting malignancy.

Sarcome de l’artère pulmonaire dans le post-partum: aspects scanographiques

We report the case of a 28 year-old female, who gave birth seven weeks previously, presenting with a pulmonary artery leiomyosarcoma discovered on a thoracic CT performed for clinical suspicion of pulmonary embolism. This case presents two major points of interest: on the first hand, it is a particular context (young post-partum patient), with classic symptoms of routine pulmonary embolism. On the other hand, the exam clearly demonstrates tumor enhancement, which is characteristic but rarely described.

Pulmonary Artery Leiomyosarcoma Expressing Epstein-Barr Virus in an Immunocompetent Individual

Primary leiomyosarcoma of the pulmonary artery is extremely uncommon and its cause remains unclear. We document a case of pulmonary artery leiomyosarcoma expressing Epstein-Barr virus DNA sequences in an immunocompetent patient 4 years after symptomatic Epstein-Barr virus infection.

Primary pulmonary artery leiomyosarcoma

Primary pulmonary artery sarcomas are uncommon neoplasms with up to 20% of cases being leiomyosarcomas. In many instances, the diagnosis is difficult and delayed. We report the case of a 75-year-old male who presented with symptoms suggestive of recurrent pulmonary thromboemboli, in which a primary leiomyosarcoma of the pulmonary artery was successfully removed surgically. Three years after surgery, the patient is well and with no evidence of recurrence or metastatic disease.

Primary Leiomyosarcoma of the Pulmonary Artery Confirmed by Catheter Suction Biopsy

A patient with clinical features consistent with pulmonary embolism showed no improvement despite therapy with tissue-plasminogen activator and full-dose heparin. Transvenous catheter suction biopsy was successful in establishing an antemortem histologic diagnosis of primary pulmonary artery leiomyosarcoma. Urgent surgical intervention was performed.

Leiomyosarcoma of the pulmonary artery

Successful resection of a pulmonary artery leiomyosarcoma was performed in a patient with clinical features of chronic pulmonary embolism. With radical radiotherapy to the mediastinum and solitary right hip metastasis, the patient remained well 6 months later. The difficulty of accurate diagnosis is considered in the light of recent advances in the techniques of cardiac investigation.

Pulmonary artery leiomyosarcoma

A 47-year-old man presented to his general practitioner complaining of a persistent cough associated with wheezing. It was felt that he may have had asthma and was treated with a salbutamol inhaler. However, returned to his G.P. 1 month later with the same symptoms. Oral theophylline was commenced. Due to the persisting symptoms he was referred to our chest clinic. In the clinic, he reported his history of a cough with wheeze and also that he had had bouts of dyspnoea and episodes of pain in his left shoulder, but these had largely resolved. He was a life long non-smoker, who had worked as a reception engineer in a garage. He had no past history of any illnesses. On examination he was a little overweight and mildly hypertensive. His peak flow was 560 1 mint and his chest X-ray showed his heart to be slightly enlarged. His ECG was normal. At the time, it was felt that the symptoms may have been due to bronchial hyper-reactivity, possibly related to a resolved chest infection. Eight months later he was admitted to hospital acutely dyspnoeic, following a 3-week history of progressive exertional dyspnoea, only being able to walk about 20 m on the flat. He had also had exertional nearsyncope, intermittent left-sided chest pains and a cough productive of yellow sputum. On examination he was mildly centrally cyanosed but not distressed, nor clubbed. He had a resting tachycardia at 120 beats per rain and a blood pressure of 130]70. His JVP was raised 4 cm. On auscultation of his heart, he had a loud P2 component of the second heart sound and a pansystolic murmur at the left sternal edge, and a third sound over the right ventricle. A systolic bruit could be heart over his right hemithorax. His breath sounds were vesicular with no extra sounds. The chest X-ray revealed a large heart with an oligaemic left lung and a left hemithorax slightly

Pulmonary Artery Leiomyosarcoma Treated Surgically

To date, 12 cases of primary leiomyosarcomas of the pulmonary artery have been described in the literature. In only 3 instances was a surgical treatment attempted. We describe a patient with pulmonary artery leiomyosarcoma presenting in obstructive shock. Surgical excision under extracorporeal circulation allowed temporary relief, but the patient died in septic shock on the 10th postoperative day. Relevant clinical and pathological data are briefly reviewed.