Massive primary pulmonary artery rhabomyosarcoma: A case report

Area of Science:

• Cardiovascular Pathology
• Oncology
• Pulmonary Medicine

Background:

• Pulmonary artery sarcomas (PAS) are rare tumors causing progressive pulmonary circulation obstruction.
• Clinical presentation often mimics chronic thromboembolic pulmonary hypertension (CTEPH).
• Atypical CT pulmonary angiography (CTPA) findings warrant further investigation.

Observation:

• A young man presented with massive hemoptysis, acute respiratory distress, and dyspnea.
• Echocardiography showed severe right ventricular dysfunction and pulmonary hypertension.
• CTPA revealed a filling defect concerning for PAS, leading to urgent pulmonary artery endarterectomy.

Findings:

• A massive, tree-like tumor was excised.
• Post-operative complications included reperfusion injury, pulmonary edema, and need for ECMO.
• ECMO led to hemolysis and acute kidney injury, resulting in multi-organ failure and death.
• Histopathology confirmed embryonal rhabdomyosarcoma.

Implications:

• PAS should not be mistaken for thrombi; anticoagulation must be avoided.
• Urgent conditions may preclude biopsy and neoadjuvant therapy.
• Postoperative complications like reperfusion injury and pulmonary edema require anticipation and management, possibly with ECMO.
• More data is needed to establish a consensus for PAS management.