[A pediatric case of Alveolar Soft Part Sarcoma]

Alveolar Soft Part Sarcoma (ASPS) is a rare malignant tumor arising from skeletal muscles and occurring usually in young adults. It is a solid chemo resistant tumor. At time of diagnosis the disease is usually advanced and lung metastasis are present in 65% of the cases. The translocation (X; 17), producing an ASPL-TFE3 transcript fusion which is detected on tumoral cells wall, is specific of ASPS. The curative treatment is surgical.

We report the case of a child with an ASPS of the tongue and lung metastasis.

This report presents two particularities. Firstly this type of sarcoma is very exceptional in a 2-year-old child. Secondly ASPS is not usually susceptible to chemotherapy.