Primary pulmonary artery tumors (PPATs), originating from the pulmonary artery intima, are rare tumors characterized by pulmonary artery luminal occlusion and pulmonary hypertension. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of PPATs. computed tomographic pulmonary angiography of PPATs may show filling defects, which are easily misdiagnosed. The radionuclide scan, along with other imaging examinations, can assist with the diagnosis, but the pathological diagnosis requires a puncture or surgical resection. Most primary pulmonary artery tumors are malignant, with poor prognosis and lack of specificity in clinical manifestations. However, there is no unified understanding and standard for diagnosis and treatment. In this review, we discuss the status, diagnosis, and treatment of primary pulmonary artery tumors, as well as how clinicians can better understand and treat the disease.
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| http://dx.doi.org/10.1097/MD.0000000000033337 | DOI Listing |
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