Case reportIntimal Sarcoma of the Pulmonary Valve
Section snippets
Comment
Intimal sarcoma of the pulmonary artery is a rare malignant mesenchymal tumor. It can arise from the intimal layer of the right, left, and main pulmonary arteries. In rare cases, it may extend in a retrograde manner to the pulmonary valve and right ventricle [4]. This disease mainly occurs in adults with a female sex predominance (females to males = 1.3 to 1), with the mean age at diagnosis being 48 years. The prognosis is very poor, and survival is usually 12 to 18 months [5] after onset of
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Cited by (20)
A cardiac intimal sarcoma mimicking infective endocarditis
2024, Cardiovascular PathologyEchocardiographic Characteristics of Pulmonary Artery Intimal Sarcoma: Comparison With CTPA
2023, Heart Lung and CirculationAggressive Surgical Resection of Pulmonary Artery Intimal Sarcoma
2018, Annals of Thoracic SurgeryCitation Excerpt :MDM2 was overexpressed or amplified in all tumors that met the diagnostic criteria for intimal sarcoma [2]. Because it is characterized by intraluminal growth with progressive obstruction of the lumen and potential for embolization, this tumor is often misdiagnosed as acute or chronic PTE [3]. Differentiating between PA sarcoma and PTE is difficult because of their similar clinical symptoms and radiologic findings.
Diagnostic Pathology: Soft Tissue Tumors
2015, Diagnostic Pathology: Soft Tissue TumorsRecurrent pulmonary intimal sarcoma involving the right ventricular outflow tract
2011, Annals of Thoracic SurgeryCitation Excerpt :Intimal pulmonary sarcomas are twice as common as intimal aortic sarcomas. When the patient is symptomatic, the prognosis is poor and life expectancy is usually 12 to 18 months [2, 3]. Patients have a prolonged asymptomatic course, followed by insidious onset of symptoms of pulmonary hypertension and RV failure [3].