Case Of Pulmonary Artery Sarcoma Research Articles

A rare clinical case of pulmonary artery sarcoma

Pulmonary artery sarcoma (PAS) is a malignant and very rare tumor with frequent misdiagnosis. A rare clinical case with this pathology is presented. The patient had daily fever for 6 months, he was examined in various hospitals, but the cause of fever could not be determined. In our hospital, echocardiography revealed a mass in the left pulmonary artery (PA) with obstruction. CT-angiopulmonography showed a filling defect in the left PA, blocking the lumen of the artery by 90%. According to histopathology, the patient was diagnosed with pulmonary artery sarcoma. Subsequently, the following was performed: thrombendarterectomy from the pulmonary artery, combined pneumonectomy on the left side, suturing of the secondary atrial septal defect, followed by 3 courses of monochemotherapy with paclitaxel. After 1 year and 9 months, due to disease progression the patient died. The prognosis of PAS depends on early diagnosis, so doctors should be aware of this disease and use the best treatment options.

Mdm2 and Egfr Expression and Prognosis in 16 Pulmonary Artery Sarcomas

There are few studies investigating the biomarker signatures and their relations with prognosis of pulmonary artery sarcoma patients. In the present study, we performed a cohort study on 16 cases of pulmonary artery sarcoma in an attempt to identify the potential diagnostic markers and molecular therapeutic targets of that vascular sarcoma. Patients who had undergone pulmonary artery sarcoma tumorectomy over 7 ½ years in our hospital were enrolled in this retrospective study. The resected tumors were analyzed by histopathology, the expression and mutation of Murine Double Minute 2 (MDM2), Epidermal Growth Factor Receptor (EGFR), and Platelet-Derived Growth Factor Alpha (PDGFRA) by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). The correlations between patients’ outcomes and tumor histological, IHC and FISH features were statistically analyzed. MDM2 immunostaining was seen mainly in intimal sarcoma. At univariate analysis, MDM2 expression had a positive correlation with shorter progression free survival (PFS). MDM2 and EGFR co-expression also had a positive correlation with shorter PFS and tumor metastasis. This study investigated MDM2, EGFR, and PDGFRA as potential biomarkers for diagnosis of pulmonary artery sarcoma and therapeutic targets.

Pulmonary Artery Sarcoma Diagnosed Using an Endovascular Catheter Forceps Biopsy.

We herein report a case of pulmonary artery sarcoma (PAS) in a 64-year-old woman. She was admitted to our hospital because of massive genital bleeding from endometrial cancer. Contrast-enhanced computed tomography (CT) revealed a left pulmonary artery mass and deep vein thrombosis. She underwent anticoagulant therapy for one year. However, the mass lesion gradually expanded. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT showed a positive uptake of FDG by the mass. An endovascular catheter biopsy was performed for the differentiation of endometrial cancer metastasis or primary sarcoma. The biopsy specimen tissue comprised spindle-shaped cells. Thus, the patient was diagnosed with PAS.

Spindle-Cell Sarcoma Involving the Major Pulmonary Arteries

Primary pulmonary vasculature tumors are exceptionally rare, with only a few reported cases. Signs and symptoms of such neoplasms vary but include dyspnea, cough, and chest pain. This condition is associated with a high mortality rate and is easily misdiagnosed as a pulmonary artery embolism. We present a case of pulmonary artery sarcoma that demonstrates the value of cardiac magnetic resonance imaging for accurate diagnosis.

Clinical features of pulmonary artery sarcoma: A report of three cases.

Pulmonary artery sarcoma (PAS) is a rare and highly malignant tumor of pulmonary artery origin. Since 1923, when the first case was reported, <300 cases have been reported worldwide. PAS has a poor prognosis, and early diagnosis with radical surgical resection offers patients with PAS the only chance of survival. However, due to its rarity and the non-specificity of its clinical manifestations and imaging presentation, PAS is frequently misdiagnosed as a pulmonary thromboembolic disease, including pulmonary thromboembolism (PTE) and chronic thromboembolic pulmonary hypertension (CTEPH). The present study reports three cases of PAS that were initially misdiagnosed as PTE or CTEPH, and were later shown to be PAS following surgery. In addition, the clinical features of these patients are examined in order to improve the differential diagnosis of PAS during the early stages of the disease, when the prognosis of patients with PAS is at its optimum.

Two cases of pulmonary artery sarcoma

Two cases of pulmonary artery sarcoma

Pulmonary artery sarcoma diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration

Pulmonary artery sarcoma is a rare disease with poor prognosis that has not been reported in Hong Kong. Its clinical and radiological presentation frequently mimics pulmonary embolism. Diagnosis is usually delayed until surgery, which is the treatment option that provides the best survival. Endobronchial ultrasound-guided transbronchial needle aspiration is an effective non-surgical technique for lymph node staging of lung cancer and diagnosis of mediastinal lesions via bronchoscopy. Here we discuss a case of pulmonary artery sarcoma diagnosed by this method, the second one in the literature, which serves to illustrate its potential use for early and minimally invasive diagnosis of the condition. Although such aspiration is a safe procedure, tissue sampling of extravascular extensions is advisable wherever possible.

Transthoracic Echocardiographic Assessment of Spindle Cell Sarcoma of the Pulmonary Artery in a Child

In this report, we present a case of spindle cell sarcoma of the pulmonary artery diagnosed by transthoracic echocardiography. To the best of our knowledge, this case is the youngest reported case of pulmonary artery sarcoma (PAS) to date. PAS is frequently confused for pulmonary embolism; in this case, echocardiographic findings allowed for differentiation between pulmonary embolism and solid tumor.

A case of pulmonary artery sarcoma presented as cavitary pulmonary lesions.

Pulmonary artery sarcoma (PAS) is a rare, poorly differentiated malignancy arising from the intimal layer of the pulmonary artery. Contrast-enhanced chest computed tomography (CT) is a good diagnostic modality that shows a low-attenuation filling defect of the pulmonary artery in PAS patients. An 18-year-old man was referred to our hospital for the evaluation and management of cavitary pulmonary lesions that did not respond to treatment. A contrast-enhanced CT of the chest was performed, which showed a filling defect within the right interlobar pulmonary artery. The patient underwent a curative right pneumonectomy after confirmation of PAS. Although lung parenchymal lesions of PAS are generally nonspecific, it can be presented as cavities indicate pulmonary infarcts. Clinicians must consider the possibility of PAS as well as pulmonary thromboembolism in patients with pulmonary infarcts. So, we report the case with PAS that was diagnosed during the evaluation of cavitary pulmonary lesions and reviewed the literatures.

Pulmonary Artery Sarcoma Mimicking Pulmonary Thromboembolism: Integrated FDG PET/CT

4Department of Thoracic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. ulmonary artery sarcoma is a rare malignancy arising from the mesenchymal cells of the intima of the pulmonary artery [1]. It is frequently misdiagnosed as pulmonary thromboembolism, although chest CT can help differentiate pulmonary artery sarcoma from pulmonary thromboembolism by showing a low-attenuation filling defect occupying the entire lumen of the proximal or main pulmonary artery, expansion of the involved arteries, or extraluminal tumor extension [2]. As much as the standardized uptake values (SUVs) at 18F-FDG PET have helped in differentiating between benign and malignant tumors [3, 4], visualization of a low-attenuation filling defect within a pulmonary artery on contrast-enhanced chest CT can be suggestive of a malignancy, such as pulmonary artery sarcoma, if the lesion shows high FDG uptake at PET. We present a case of pulmonary artery sarcoma that showed high FDG uptake on integrated FDG PET/CT.

Primary Pulmonary Artery Sarcoma Detected with a Pulmonary Infarction

Primary malignant tumors of the pulmonary arteries occur infrequently. The clinical presentation and diagnostic imaging features of the tumor are usually nonspecific and correct diagnosis is often delayed. In this report, we present a case of pulmonary artery sarcoma. MRI and PET-CT were found to be useful for differentiating the tumor from a thromboembolism.

Pulmonary Artery Sarcoma

We report 12 cases of pulmonary artery sarcoma. The mean age at diagnosis was 48.4 years. Based on histomorphologic features and immunohistochemical findings, 2 tumors were classified as rhabdomyosarcoma, 4 as leiomyosarcoma, 1 as osteogenic sarcoma, 1 as angiosarcoma, and 4 as high-grade sarcoma. All patients underwent surgery. In addition, 7 patients received neoadjuvant or adjuvant therapy. Five patients died 3 to 23 months after surgery. Three patients were still alive at 8, 27, and 68 months at last follow-up. Another 3 patients were alive at 2, 15, and 40 months and then lost to follow-up. The 2 patients with the longest survival (40 months and 68 months) had a diagnosis of leiomyosarcoma. Both patients with rhabdomyosarcoma died at 3 months after surgery. Pulmonary artery sarcoma is an uncommon entity with a poor prognosis. The role of early diagnosis, histologic classification, surgical treatment, and adjuvant therapy in patient outcome is discussed.

Pulmonary Artery Sarcoma

We report 12 cases of pulmonary artery sarcoma. The mean age at diagnosis was 48.4 years. Based on histomorphologic features and immunohistochemical findings, 2 tumors were classified as rhabdomyosarcoma, 4 as leiomyosarcoma, 1 as osteogenic sarcoma, 1 as angiosarcoma, and 4 as high-grade sarcoma. All patients underwent surgery. In addition, 7 patients received neoadjuvant or adjuvant therapy. Five patients died 3 to 23 months after surgery. Three patients were still alive at 8, 27, and 68 months at last follow-up. Another 3 patients were alive at 2, 15, and 40 months and then lost to follow-up. The 2 patients with the longest survival (40 months and 68 months) had a diagnosis of leiomyosarcoma. Both patients with rhabdomyosarcoma died at 3 months after surgery. Pulmonary artery sarcoma is an uncommon entity with a poor prognosis. The role of early diagnosis, histologic classification, surgical treatment, and adjuvant therapy in patient outcome is discussed.

Successful Treatment of Pulmonary Artery Sarcoma by a Two-drug Combination Chemotherapy Consisting of Ifosfamide and Epirubicin

We describe a case of 63-year-old woman with pulmonary artery sarcoma successfully treated with chemotherapy. She developed acute shortness of breath, and left chest and shoulder pain. Although a diagnosis of acute pulmonary embolism was made at a local hospital and she received anticoagulation and thrombolysis therapy, no improvement was achieved. Thereafter, she underwent a pulmonary thromboectomy in our hospital, and the histological diagnosis was intimal sarcoma of the pulmonary artery. Since post-operative computed tomography (CT) scans of the chest showed obvious persistence of an intraluminal hypoattenuated area in the left main pulmonary artery, the patient was treated with four cycles of a doublet chemotherapy consisting of ifosfamide (2.5 g/m(2)/day) on days 1-5 and epirubicin (45 mg/m(2)/day) on days 2 and 3. CT scans of the chest after four cycles showed marked regression of the intraluminal hypoattenuated area in the left main pulmonary artery. This is the first case of pulmonary artery sarcoma responding to chemotherapy. Surgical resection is currently the most hopeful treatment for pulmonary artery sarcoma. However, intensive chemotherapy is worth trying in unresectable patients.

ＣＴガイド下生検にて術前診断し得た肺動脈原発肉腫の１手術例

症例は60歳, 男性. 1999年5月頃から少量の血痰を認めたため近医を受診し, 胸部X線写真にて左中肺野に大きさ3×2cm大の異常陰影を指摘され, 精査目的にて同年6月当院を紹介された. 胸部CTでは, 左主肺動脈を中心とし周囲の肺実質に連続性に浸潤する最大径4cm大の腫瘤を認め, 肺動脈造影では, 左右の肺動脈分岐部から約1cmの部位で左肺動脈が完全閉塞していた. 気管支鏡生検を行ったが確定診断には至らず, CTガイド下生検にて肺動脈原発の肉腫と術前診断された. 他の臓器に転移を示唆する所見は認めず, 胸骨正中切開と左前方腋窩開胸によるアプローチにて両側縦隔リンパ節郭清と左肺全摘術を施行した. 病理組織学的検査では, 左肺動脈の前上区域枝 (A3) 原発のintimal sarcomaと診断され, 完全切除例と判定された. 現在, 術後2年が経過するが, 再発の徴候は認めない.

Pulmonary artery sarcoma mimicking pulmonary embolism: Successful surgical intervention

Because of its rarity and the similarity of its presentation to that of pulmonary thromboembolic disease, the diagnosis of pulmonary artery sarcoma is often not considered early in patients presenting with recurrent or chronic pulmonary emboli. We present a case of pulmonary artery sarcoma that was treated as pulmonary embolism for 3 years before surgical resection was carried out. Two years after the resection the patient is well with no clinical or radiologic evidence of recurrent or metastatic disease.

Pulmonary Artery Sarcoma

Primary malignant tumors of the pulmonary arteries occur infrequently and diagnosis is often delayed since symptoms are nonspecific. We present a case of pulmonary artery sarcoma and its interesting magnetic resonance findings.