Primary pulmonary artery sarcoma: a close associate of pulmonary embolism—20-year observational analysis

Bandyopadhyay, Debabrata; Panchabhai, Tanmay S.; Bajaj, Navkaranbir S.; Patil, Pradnya D.; Bunte, Matthew C.

doi:10.21037/jtd.2016.08.89

Cited by 113 publications

(195 citation statements)

References 138 publications

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“…Primary large artery sarcoma is extremely rare, and primarily occurs as intimal sarcoma in large arteries and veins; the pulmonary trunk, RPA and LPA; and the pulmonary valve . About 400 cases of PAS have been reported since Mandelstamm first reported, finding it during an autopsy in 1923 …”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary artery sarcoma is highly malignant, often resulting in the obstruction of pulmonary blood flow and metastasis to other organs, such as the brain, pancreas, adrenal glands and lungs . The disease has a poor prognosis, with an average duration of survival of approximately 1.5 months without surgical treatment . The main cause of death from PAS is pulmonary obstruction and right heart failure .…”

Section: Discussionmentioning

confidence: 99%

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Clinical and radiological features of pulmonary artery sarcoma: A report of nine cases

Song

Huang

et al. 2017

Clinical Respiratory J

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The clinical features of PAS that may help to differentiate it from PTD are its insidious onset, constitutional symptoms, increased serum inflammatory marker levels, lack of predisposing factors and unresponsiveness to thrombolytic and/or anticoagulation therapy. Typical CTPA features are crucial for suspicion of PAS. Early biopsy or surgery should be performed to confirm the diagnosis of PAS and improve its prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Clinical and radiological features of pulmonary artery sarcoma: A report of nine cases

Song

Huang

et al. 2017

Clinical Respiratory J

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“…Pulmonary artery sarcoma is a rare malignancy with a poor prognosis . Although prompt recognition and treatment may prolong survival, half of cases are misdiagnosed as pulmonary embolism . Familiarity with its identification on TTE is therefore important, as it may be the first imaging test performed, such as in this case, where, in retrospect, a subtle outline of the tumor can be appreciated in the parasternal short‐axis view (Figure ; Movie ).…”

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confidence: 93%

Multimodality imaging of a pulmonary artery sarcoma

et al. 2017

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Pulmonary artery sarcoma is a rare malignant neoplasm. Here, we describe a patient with a pulmonary artery sarcoma, which was only subtly visible and therefore not fully appreciated on initial transthoracic echocardiogram. Characterization of the tumor was aided by the use of multimodality imaging that included computed tomography, magnetic resonance imaging, and positron emission tomography. Familiarity with its appearance on multiple imaging modalities including echocardiography is important to ensure timely diagnosis, although the optimal treatment strategy is still unknown, and the prognosis remains poor. K E Y W O R D Scomputed tomography, echocardiography, magnetic resonance imaging, positron emission tomography, pulmonary artery sarcoma A 21-year-old man presented with pleuritic chest pain and dyspnea.Due to an enlarged cardiac silhouette on chest x-ray, a transthoracic echocardiogram (TTE) was performed but was reported to be unremarkable. Subsequent computed tomography (CT) of the chest with contrast revealed a filling defect in the main pulmonary artery (PA) extending into the right PA, along with multiple lung nodules. Despite anticoagulation for a presumed pulmonary embolism, his symptoms worsened. On repeat CT, the filling defect extended into the right PA below the pulmonic valve with no filling defects in the left PA, which was considered atypical for a thrombus ( Figure 1A). Biopsy of the lung nodules revealed sarcoma and magnetic resonance imaging confirmed a pedunculated tumor arising from a pulmonary valve cusp ( Figure 1B).On positron emission tomography, there was moderately intense fludeoxyglucose activity within the filling defect and minimal activity within several lymph nodes ( Figure 1C). As the disease was not curable due to multiple pleural-based metastases, he received palliative chemotherapy (doxorubicin, ifosfamide-mesna) and radiation therapy. Repeat imaging showed a decrease in size of the sarcoma, which was this time appreciated on TTE ( Figures 1D and 2, Movies S1 and S2). Nevertheless, due to ongoing symptoms, he underwent a palliative PA and pulmonic valve resection with homograft reconstruction. The resected tumor was a 5.5-cm, firm, white-tan, cylindrical, pedunculated mass that adhered to the anterior pulmonary valve ( Figure 1E). It was classified as a French Sarcoma Group Grade 3 synovial sarcoma on pathology ( Figure 1F). He thereafter received second-line therapy with the tyrosine kinase inhibitor, pazopanib, followed by oncogenomics-guided third-line therapy with the histone deacetylase inhibitor, vorinostat. Unfortunately, his disease progressed and he died 32 months after his initial presentation.Pulmonary artery sarcoma is a rare malignancy with a poor prognosis. 1 Although prompt recognition and treatment may prolong survival, half of cases are misdiagnosed as pulmonary embolism. 1 Familiarity

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“…All tumor cells express vimentin and the most of them alpha-SMA and CD31, but and in some cases also desmin, myogenin, and CD34. [5] According to Bode-Lesniewska et al ., the most of PAS express mdm2, implicating that mdm2/p53 pathway may be involved in possible pathogenesis of this tumor. [6] However, in our case, mdm2 was not expressed in tumor cells of PAS.…”

Section: Discussionmentioning

confidence: 99%

Rare histological subtype of pulmonary artery intimal sarcoma diagnosed by multidisciplinary approach

et al. 2018

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Pulmonary artery intimal sarcoma (PAS) is a rare mesenchymal tumor mostly diagnosed in middle-aged women. In a 63-year-old female, the radiological findings showed cavitation in the left upper lobe of the lung and infiltrative tumor mass around the left pulmonary artery. PAS consisted of small, round tumor cells with about 80% of mitotic activity and with myxoid background and specific immunoprofile and diagnosed as undifferentiated sarcoma with round cell features type. The final diagnosis of PAS was established according to the pathohistological, chest computed tomography scan, and surgery finding.

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Primary pulmonary artery sarcoma: a close associate of pulmonary embolism—20-year observational analysis

Abstract: PPAS has a radiological appearance similar to PE, which makes accurate and timely diagnosis challenging. More rapid diagnosis may lead to earlier, appropriate surgical treatment and improved outcomes, when combined with adjuvant treatment.

Cited by 113 publications

References 138 publications

Clinical and radiological features of pulmonary artery sarcoma: A report of nine cases

Clinical and radiological features of pulmonary artery sarcoma: A report of nine cases

Multimodality imaging of a pulmonary artery sarcoma

Rare histological subtype of pulmonary artery intimal sarcoma diagnosed by multidisciplinary approach

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