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

Primary cardiac angiosarcoma is an extremely rare tumor, especially in pediatric populations (0-18 years), which often carries a poor prognosis due to vague symptoms and its aggressive nature. We present a rare case involving a 17-year-old female, illustrating the diagnostic and therapeutic difficulties posed by this condition in an atypical age group. A 17-year-old female presented with a one-month history of dry cough, exacerbated when lying down, and orthopnea requiring two pillows for sleep. After an unremarkable outpatient evaluation, an echocardiogram revealed a large pericardial effusion with preserved ejection fraction (60%), leading to hospital admission. Computed tomography (CT) identified a 3.0 × 4.0 cm irregular mass invading the right atrium, multiple mediastinal and pulmonary nodules, a solitary 3 cm liver lesion, and a skull mass. Cardiac magnetic resonance imaging (MRI) confirmed a 5.0 × 3.0 × 4.0 cm mass located in the right atrium with hyperintensity on first-pass perfusion imaging, heterogeneous contrast enhancement, and significant pericardial effusion. Liver biopsy confirmed metastatic angiosarcoma, likely of cardiac origin, with positive immunohistochemical markers (CD31, CD34, CD117; Ki-67 40%). The patient underwent six months of chemotherapy (paclitaxel and gemcitabine) without improvement. Follow-up MRI showed disease progression with new liver and kidney lesions. The patient discontinued treatment and died shortly thereafter. Cardiac AS is an aggressive malignancy with nonspecific symptoms, such as dyspnea and cough, complicating early diagnosis. These tumors most often present with distant metastases. Imaging modalities, including echocardiography, CT, and MRI, are critical for diagnosis, with histopathology and immunohistochemistry confirming the diagnosis. Treatment typically involves surgical resection and chemotherapy. However, there is no standard treatment for cardiac angiosarcoma. This case underscores the need for heightened clinical suspicion in young patients with persistent cardiopulmonary symptoms and the urgent need for novel therapeutic strategies to improve outcomes.