Key result
Rare pulmonary artery spindle cell sarcoma mimics supravalvular pulmonic stenosis by causing extrinsic compression.
Why the study?
Pulmonary artery malignancies are extremely rare and imaging modalities may not adequately identify the tumor, posing diagnostic challenges.
Case Report (n=1)
Highlights the diagnostic challenges and need for high clinical suspicion for rare pulmonary artery sarcomas masquerading as pulmonary stenosis.
Consider pulmonary artery sarcoma in atypical pulmonic stenosis; this case leaves open optimal preoperative diagnostic strategies.
Pulmonary artery malignancies are extremely rare, with a reported occurrence of 0.001-0.03% and almost all being sarcomas from the intima and worldwide less than 250 cases have been reported [1, 2]. A high degree of suspicion is required to diagnose this tumor as imaging modalities may not adequately identify this tumor [1-3] posing challenges in diagnosis as illustrated in our case. Because of the high mortality associated with this malignancy, early and aggressive intervention is crucial.
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A 2021 study conducted a case report in Pulmonary Artery Spindle Cell Sarcoma (n=1). Surgical resection and debulking was evaluated. A 59-year-old woman initially diagnosed with supravalvular pulmonic stenosis was intraoperatively found to have a rare high-grade pulmonary artery spindle cell sarcoma causing extrinsic compression.
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