Epithelioid Hemangioendothelioma and Epithelioid Hemangioma: Pazopanib as a Potential Salvage Therapy

Fig. 1. A Chest X-ray with lytic lesions and fracture of the right seventh rib. B, C Chest CT scan with lytic and sclerotic lesions and deformity of the right seventh rib. D Chest X-ray after right seventh rib resection.

Fig. 2. A H&E stain showing a proliferation of small, well-formed blood vessels with epithelioid endothelium. In the stroma, there is proliferation of spindle and oval cells without vascular lumen formation. B–D Immunostains for CD31, ERG, and FLI1 (endothelial markers) are positive in both populations of cells (lumen-forming and stromal cells). The immunostains also highlight focal cord formation in the stromal population. Original magnification, ×20 (all images).

Fig. 3. A–C Surgical figure showing the mass with dimensions of 3 × 1.5 cm in the posterior aspect of the right seventh rib. D The distorted rib with two pathologic fractures (black arrows).

Fig. 4. A Chest CT-scan showed a mediastinal space-occupying lesion with 7.5 cm in diameter. B Enlargement of the mediastinal space-occupying lesion to 9 cm in diameter. C–E Heterogeneous mass in the left mediastinum measuring 84 × 50 mm with hyperdense, gross calcifications. Multiple lung nodules bilaterally, the largest measuring 13 mm in the left lower lobe. Hypodense lesion in segment 7/8 of liver up to 21 mm. Lytic lesions in the vertebral body at the level of D8. All above lesions had high F18 FDG uptake and suspected as metastasis from the mediastinal mass.

Fig. 5. A, B Tumor composed of medium size, round-to-oval epithelioid cells with a myxoid matrix resembling cartilage. Numerous multinucleated giant cells are also present. Original magnification, ×10 and ×20. C, D Immunostain for CD31 is positive. The remaining immunoprofile showed strong reactivity for other vascular markers (CD34, Factor VIII, and FLI-1) as well as some staining for CK7 and CD10. Original magnification, ×20.

Fig. 6. A Cortical destruction of the posterolateral aspect of midshaft femoral bone with additional soft tissue component. B Enlargement of the primary tumor after local radiotherapy. C Soft tissue component decreases in size and bone become more sclerotic following biological therapy.