Treatment with paclitaxel in a 48-year-old female with epithelioid angiosarcoma resulted in approximately 90% resolution of pulmonary lesions.
Case Report (n=1)
This case highlights the diagnostic challenges of epithelioid angiosarcoma and demonstrates a significant imaging response to paclitaxel in a patient with an atypical presentation.
Abstract Introduction Epithelioid Angiosarcoma (EA) is a rare, aggressive vascular malignancy characterized by malignant endothelial cells with predominantly epithelioid morphology. It most commonly arises in deep soft tissues of the extremities but may originate in various sites, including the adrenal glands. EA typically presents as a painful, enlarging mass with early local invasion and distant metastasis, most frequently to the lungs. Risk factors include prior radiation exposure, chronic lymphedema, environmental carcinogens, and genetic predispositions. It primarily affects older adults, with a male predominance and peak incidence in the seventh to eighth decades of life. This case diverges from expected epidemiologic and clinical patterns, occurring in a younger female with no identifiable risk factors, an atypical diagnostic course, and an exceptional treatment response. Case Presentation A 48-year-old Southeast Asian female with adrenal adenoma and asthma presented with 11 days of progressive hemoptysis, dyspnea, pleuritic chest pain, night sweats, abdominal pain, and headache. She was initially treated for COVID-19 infection and presumed bacterial pneumonia with nirmatrelvir, prednisone, and azithromycin, but symptoms persisted. On evaluation, she was tachycardic, tachypneic, and hypoxic. CT chest showed a 16-mm right pleural-based mass, bilateral pulmonary nodules, and ground-glass opacities. CT abdomen demonstrated a right adrenal mass measuring 6.2 × 5 cm, previously 3 cm and biopsied as an adenoma two years earlier. Bronchoscopy revealed diffuse alveolar hemorrhage without identifiable pathogens. Lung nodule biopsy showed neutrophilic inflammation, and adrenal biopsy demonstrated necrotic tissue without viable tumor—both nondiagnostic. Extensive infectious, autoimmune, and vasculitis evaluation was negative. She continued to experience hemoptysis causing anemia and hypoxemia, with interval progression of pulmonary nodules and ground-glass opacities, along with new subsegmental pulmonary emboli. Video-assisted thoracoscopic biopsy of the right lower lobe confirmed EA. She was started on paclitaxel, with follow-up imaging showing approximately 90% resolution of pulmonary lesions. Discussion EA accounts for only 1-2% of sarcomas, contributing to significant diagnostic challenges. It exhibits high metastatic potential, with rates up to 50% at presentation. Prognosis remains poor, with median survival ∼17 months and worse outcomes in older patients or those with advanced disease. Core needle biopsy with immunohistochemistry is the diagnostic gold standard; however, sampling maybe nondiagnostic, as seen in this case. Given its aggressive metastatic behavior, treatment requires an individualized multidisciplinary approach, including surgery, radiation, systemic chemotherapy, and emerging targeted or immunotherapy options. This case underscores the diagnostic challenges of EA and the importance of comprehensive evaluation to guide optimal treatment. This abstract is funded by: None
Dahab et al. (Fri,) conducted a case report in Epithelioid Angiosarcoma (n=1). Paclitaxel was evaluated. Treatment with paclitaxel in a 48-year-old female with epithelioid angiosarcoma resulted in approximately 90% resolution of pulmonary lesions.