Vascular-endothelial-growth-factor (VEGF) expression and possible response to angiogenesis inhibitor bevacizumab in metastatic alveolar soft part sarcoma.

Alveolar soft part sarcoma is a rare tumour of unknown histogenesis. Although tumour growth is slow and often asymptomatic, vascular invasion and metastatic dissemination (especially to the brain and lung) take place early in the course of disease. Resection is the treatment of choice and could lead to complete remission. If complete resection is not possible, the prognosis of alveolar soft part sarcoma is poor, since conventional cytotoxic chemotherapy regimens have little effi cacy in most patients. Therefore, alternative treatment approaches are needed. We report tumour regression in a patient with disseminated alveolar soft part sarcoma during antiangiogenic treatment with the antibody against vascular endothelial growth factor (VEGF), bevacizumab. An 8-year-old boy presented with a 2-week history of headaches in July, 2003. The boy was in good general condition, but physical examination revealed minor gait ataxia, dysdiadochokinesis, bilateral pyramidal signs, papilloedema, and homonymous hemianopsia. MRI of the brain detected three solid contrast-enhancing lesions in the left cerebellum (3·2×3·7 cm), left occipital lobe (4·8×3·7×5·2 cm), and left parietal lobe (1·3×1 cm). Digital subtraction angiography showed all lesions to be highly vascularised. No notable fi ndings were seen in MRI of the spinal cord. A multislice CT of the thorax and abdomen revealed 15 intra pulmonary lesions (the largest measur ing 2·7×2·8 cm). Whole-body MRI detected a lesion (2·4×1·2 cm) in the left psoas muscle, which was regarded as the primary site. Resection of the infratentorial tumour was undertaken to prevent brain-stem compression. Histopathological examination of the tissue revealed tumour cells arranged in solid nests and separated by thin sinusoidal vessels, characteristic of alveolar soft part sarcoma (fi gure 1A). The Lancet Oncol 2006; 7: 521–23