[A pediatric case of Alveolar Soft Part Sarcoma].
BACKGROUND
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.


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


CONCLUSIONS
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.
Marchac
A
A
Service de chirurgie maxillofaciale et plastique, APHP, hôpital d'enfants Armand-Trousseau, 75012 Paris, France.
Picard
A
A
Landman-Parker
J
J
Larroquet
M
M
Vazquez
M-P
MP
Franchi
G
G
D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D005500:Follow-Up Studies; D005927:Glossectomy; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary / Q000601:surgery; D008297:Male; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D018234:Sarcoma, Alveolar Soft Part / Q000175:diagnosis* / Q000556:secondary / Q000601:surgery; D014062:Tongue Neoplasms / Q000175:diagnosis* / Q000601:surgery