Primary Pleural Epithelioid Angiosarcoma with Lung and Bone Metastases: A Case Report

Area of Science:

• Oncology
• Pathology
• Radiology

Background:

• Primary pleural epithelial angiosarcoma (EAS) is an extremely rare malignancy with nonspecific clinical presentations, leading to frequent misdiagnosis.
• Limited clinical data exists for primary pleural EAS, underscoring the need for detailed case reports.

Observation:

• A 31-year-old patient presented with chest pain and dyspnea, initially diagnosed with hydropneumothorax.
• Imaging revealed pleural thickening and lung nodules; a thoracoscopic biopsy suggested a vasogenic tumor.
• Positron emission tomography/computed tomography (PET/CT) identified increased fluorodeoxyglucose uptake in the pleura and metastatic lesions in the chest wall, spine, and iliac crest.

Findings:

• Immunohistochemistry confirmed primary pleural epithelial angiosarcoma (EAS) through positive expression of CD31, CD34, and ETS-related genes.
• The case demonstrates the utility of PET/CT in precisely locating lesions for biopsy in suspected pleural angiosarcoma.
• Multidisciplinary discussion and remote expert consultation were crucial for achieving the final diagnosis.

Implications:

• Primary pleural EAS should be considered in cases of unexplained hydropneumothorax.
• Advanced imaging like PET/CT is vital for accurate staging and biopsy guidance.
• Pathological examination with specific markers remains the cornerstone for diagnosing primary pleural EAS, with collaborative approaches enhancing diagnostic accuracy.