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Primary pulmonary artery sarcoma in two children
Z Q Farooki1, C H Chang, W L Jackson
1Section of Cardiology, Hospital of Michigan, Detroit 48201.
Insights
Primary pulmonary artery sarcoma, a rare childhood cancer, was diagnosed as hemangiopericytoma in two pediatric patients. One child survived after surgery, while the other did not survive cardiac arrest.
Area of Science:
- Pediatric Oncology
- Cardiovascular Pathology
- Surgical Oncology
Background:
- Primary pulmonary artery sarcoma is an extremely rare malignancy.
- This report details the first known cases in pediatric patients.
Observation:
- Two children presented with symptoms related to right heart compromise.
- One patient experienced right ventricular outflow obstruction, the other right heart failure.
Findings:
- Both patients were pathologically diagnosed with hemangiopericytoma.
- Surgical resection was successful in one case.
- The second patient experienced fatal cardiac arrest.
Implications:
- Highlights the aggressive nature of pediatric pulmonary artery sarcoma.
- Emphasizes the need for early diagnosis and tailored treatment strategies.
- Suggests hemangiopericytoma as a potential diagnosis for primary pulmonary artery tumors in children.
Abstract:
The clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.