ABSTRACT
We present a 62-year-old woman who was diagnosed with primary pulmonary arterial sarcoma with pulmonary hypertension. CT showed a large defect inside both main pulmonary arteries in accordance with the accumulation of FDG-PET. To relieve the symptom and to prevent sudden death, removal of a massive pulmonary tumor and postoperative chemotherapy were planned. Utilizing the intermittent systemic circulatory arrest under deep hypothermia (18°C), the pulmonary artery trunk and both main pulmonary arteries were opened. The tumor stacking inside the pulmonary artery was removed and its origin at the commissure of the pulmonary artery valve was resected. The defect was repaired with a pulmonary valve replacement. Histopathological examination revealed high grade sarcoma. Her postoperative course was uneventful ; however, she died of cerebral hemorrhage during chemotherapy six months after surgery.
ABSTRACT
El sarcoma de la arteria pulmonar es una patología rara y de pronóstico letal. Se desconoce su etiología, pero con mayor frecuencia afecta al tronco de la arteria pulmonar y a sus ramas principales. Los estudios complementarios mediante resonancia magnética con gadolinio y tomografía por emisión de positrones son de utilidad para orientar el diagnóstico, pero este sólo se puede establecer al realizar un estudio histológico. Presentamos este caso dada la baja incidencia y su importancia para el diagnóstico diferencial del tromboembolismo pulmonar.
Pulmonary artery sarcoma is a rare tumor with lethal prognosis. Its etiology is unknown; most frequently the pulmonary trunk and its main branches are affected. Magnetic resonance imaging with gadolinium and positron emission tomography are complementary studies that can guide the diagnosis, but this can only be established with the histological study. We present this case because of the low incidence and its importance in the differential diagnosis of pulmonary thromboembolism.
Subject(s)
Pulmonary Artery , SarcomaABSTRACT
Pulmonary artery sarcomas (PAS) are rare malignant neoplasms. Right heart failure due to tumour location is the main cause of death in PAS patients. The hemodynamic influence of PAS may effect prognosis, but this has not been proven. We aimed to identify the clinical characteristics and prognostic factors of PAS in Korea, their association with pulmonary hypertension (PH). PAS patients treated at the Asan Medical Center between 2000 and 2014 were reviewed. We examined demographic characteristics, diagnostic and treatment modalities. Potential prognostic factors were evaluated by univariate and multivariate analysis. Twenty patients were diagnosed with PAS. Ten patients were male, the median age was 54 years (range, 33–75 years). The most common symptom observed was dyspnea (65%). The most common histologic type was spindle cell sarcoma (30%). Ten patients had a presumptive diagnosis of pulmonary embolism (PE) and received anticoagulation therapy. Seventeen patients underwent surgery, but only 5 patients had complete resection. Eleven patients received post-operative treatment (chemotherapy = 3, radiotherapy = 5, chemoradiotherapy = 3). PH was observed in 12 patients before treatment and in 6 patients after treatment. Overall median survival was 24 months. Post-treatment PH was associated with poor prognosis (HR 9.501, 95% CI 1.79–50.32; P = 0.008) while chemotherapy was negatively associated with mortality (HR 0.102, 95% CI 0.013–0.826; P = 0.032) in univariate analysis. Post-treatment PH was also associated with poor prognosis in multivariate analysis (HR 5.7, 95% CI 1.08–30.91; P = 0.041). PAS patients are frequently misdiagnosed with PE in Korea. Post-treatment PH is associated with a poor prognosis.
Subject(s)
Humans , Male , Cause of Death , Chemoradiotherapy , Diagnosis , Drug Therapy , Dyspnea , Heart Failure , Hemodynamics , Hydrogen-Ion Concentration , Hypertension, Pulmonary , Korea , Mortality , Multivariate Analysis , Prognosis , Pulmonary Artery , Pulmonary Embolism , Radiotherapy , SarcomaABSTRACT
Objective To investigate the clinical characteristics of pulmonary artery sarcoma (PAS) and pulmonary thromboembolism(PTE), to improve doctors' awareness and the early diagnosis of PAS.Methods The clinical data of 10 PAS cases confirmed with biopsy were retrospectively analyzed,and 10 cases with PTE were selected as control group.Results (1) Main clinical manifestations of the two groups were chest tightness, shortness of breath, intermittent syncope, palpitations, chest pain and cough, and there were no statistical significance differences between the two groups (P＞0.05).(2)There were 2 cases (20.0％) PaO2 ＜80 mmHg in patients with PAS.However, there were 8 cases (80.0％)PaO2 ＜ 80 mmHg in control group.The two groups had statistically significant difference (x2 =7.200, P =0.023).(3) Wells score : the cases with PAS was in low risk (80.0％ and 10.0％),however, the cases of control group was in medium and high risk(90.0％ and 20.0％).The two groups had statistically significant difference (P =0.005, 0.001).(4) The two groups had no statistically significant difference in ECG, UCG, X-ray, lung ventilation/perfusion (P＞ 0.05).(5) There had statistically significant difference in terms of LDH and CRP between PAS and PET group (100％ vs.0, x-2 =10.796,P=0.003;100％ vs.0, x2 =15.000, P =0.000).There was faster ESR in PAS group than control group,and the two groups had statistically significant difference (75％ vs.0, x2=1.400, P =0.011).There was no case of D-Dimer＞500 μg,/L in PAS group, while 10 cases in control group, and the two groups had significant statistical difference (x2 =17.000, P =0.000).(6) There was 1 case (12.5％) with DVT in PAS group, 6 cases (60.0％) in PTE group, and the two groups had significant statistical difference (x2=10.568, P =0.001).(7) The CTPA in PAS group showed filling defect in the main pulmonary artery trunk (85.7％ vs.0) ,left pulmonary artery (85.7％ vs.10.0％) ,right pulmonary artery(100％ vs.10.0％) and both left and right pulmonary artery (85.7％ vs.10.0％), the two groups had significant statistical difference (x2 =13.247, P =0.001;x2 =9.746, P=0.004;x2 =13.388, P =0.000;x2 =9.746, P =0.004).Conclusion PAS and PTE can' t be distinguished from the clinical symptoms, ECG, UCG, X-ray,lung ventilation/perfusion imaging.PAS is easily misdiagnosed as PTE.More attention should be given.PAS can be identified early through the blood gas analysis, hypoxemia,Wells score, LDH, CRP, ESR, D-Dimer, DVT and CTPA.
ABSTRACT
Primary pulmonary artery sarcoma is a rare malignant tumor arising from the pulmonary artery. Diagnosis of primary pulmonary artery sarcoma is quite difficult and the conditon is often misdiagnosed as a more common disease, such as a pulmonary embolism. PET can help in diagnosing a pulmonary artery sarcoma due to the increased uptake of 18F-FDG in the area of the tumor. However, the poor anatomic resolution of PET has limited its clinical applications in pulmonary vascular disease. The recently developed PET/CT is the fusion of PET and CT that improves the anatomical resolution of PET. We report a case of a primary pulmonary artery sarcoma mimicking a pulmonary embolism that was diagnosed with PET/CT and confirmed with a surgical resection.
Subject(s)
Diagnosis , Diagnosis, Differential , Embolism , Fluorodeoxyglucose F18 , Positron Emission Tomography Computed Tomography , Pulmonary Artery , Pulmonary Embolism , Sarcoma , Vascular DiseasesABSTRACT
A primary pulmonary artery sarcoma is a rare malignant tumor derived from the intimal layer of the pulmonary artery. Its clinical presentation can lead to a misdiagnosis of more common diseases such as thromboembolic disease. It is known to have a very poor prognosis. Therefore, the correct diagnosis of a primary pulmonary artery sarcoma is difficult and often delayed. We experienced a case of primary pulmonary artery sarcoma mimicking a pulmonary thromboembolism. The patient was admitted as a result of progressive dyspnea and coughing. The lung perfusion scan showed a large perfusion defect involving almost the entire right lung and suspicious small perfusion defects in the left upper lobe. Magnetic resonance imaging of the chest showed an enhancing nodule within the thrombus in the right pulmonary artery. The mass was removed completely by surgery, but the patient died as a result of shock.
Subject(s)
Humans , Cough , Diagnosis , Diagnostic Errors , Dyspnea , Lung , Magnetic Resonance Imaging , Perfusion , Prognosis , Pulmonary Artery , Pulmonary Embolism , Sarcoma , Shock , Thorax , ThrombosisABSTRACT
A primary sarcoma of the pulmonary artery is a rare disease. The symptoms of the disease are nonspecific, so it is very difficult to get an exact diagnosis in an emegency situation. A 48-year-old man was admitted to our emergency center with the history of syncope. The patient had no symptoms of a chest problem. During the diagnostic work up, we found an increased density in the left upper lung field on the chest PA, which was the only diagnostic clue to the patient 's illness. A chest CT, a chest MRI, and other studies were done to diagnose the undelying cause of syncope. We found a large intraluminal mass in the pulmonary artery and anterior mediastinal lymph node enlargement. A firm tumor, which arose from the main pulmonary artery and caused a near total obstruction of the left main pulmonary artery, was found during operation, and a partial resection of the tumor mass was done. Histologic and immunohistochemical staining revealed the tumor to be poorly differentiated spindle cell sarcoma. We report the case of a pulmonary artery sarcoma patient, along with a brief review of related literature.