Cardiac Angiosarcoma in a 17-Year-Old Female: A Rare Case Report and Literature Review

Area of Science:

• Cardiovascular Pathology
• Pediatric Oncology
• Medical Imaging

Background:

• Primary cardiac angiosarcoma is exceptionally rare in pediatric populations (0-18 years).
• The condition often presents with vague symptoms and has an aggressive nature, leading to a poor prognosis.
• Early diagnosis is frequently delayed due to nonspecific symptoms like cough and dyspnea.

Observation:

• A 17-year-old female presented with persistent dry cough and orthopnea.
• Echocardiography revealed a large pericardial effusion; CT and MRI identified a right atrial mass with extensive metastatic disease, including liver and skull lesions.
• Liver biopsy confirmed metastatic angiosarcoma with characteristic immunohistochemical markers (CD31, CD34, CD117; Ki-67 40%).

Findings:

• Despite six months of chemotherapy (paclitaxel and gemcitabine), the patient showed disease progression with new metastatic sites.
• The angiosarcoma demonstrated aggressive behavior with rapid progression and widespread metastasis.
• Histopathology and immunohistochemistry were crucial for confirming the diagnosis of cardiac angiosarcoma.

Implications:

• This case underscores the critical need for high clinical suspicion in young patients with unexplained cardiopulmonary symptoms.
• Current treatment strategies, including chemotherapy and surgery, offer limited efficacy for advanced cardiac angiosarcoma.
• There is an urgent requirement for developing novel therapeutic strategies to improve outcomes for this rare and aggressive malignancy.