[Clear cell sarcoma of the tendons and aponeuroses].
BACKGROUND
Clear cell sarcoma of tendons and aponeuroses is a rare mesenchymal tumor in childhood. It is difficult to treat and its prognosis is bad.


METHODS
A 9 year-old girl was admitted because of persistent pain in the left lower part of her thorax. Clinical examination showed a mass, 4 cm in diameter, embedded in the thoracic wall. Ultrasonography and CT-scan showed that this mass extended between the last ribs to the pleural cul-de-sac. There was no adenopathy and myelogram was normal. Biopsy of the mass showed features of clear cell sarcoma of tendons and aponeuroses. Cytogenetic studies showed translocation t(12;22) (q13-14;q12) in tumor cells. The patient was given chemotherapy followed by complete resection, but the tumor recurred locally 3 months later and the patient died, despite chemotherapy plus radiotherapy, 23 months after the apparent onset of disease.


CONCLUSIONS
Treatment of this type of tumor remains difficult. The existence of a break point on 22q12, as in Ewing sarcoma, suggests that this tumor in of neural crest cell origin.
Drouin
V
V
Service de Pédiatrie et Génétique médicales, Hôpital Ch.-Nicolle, Rouen.
Prudent
M
M
Eurin
D
D
Cambon-Michot
C
C
Tron
P
P
Vannier
J P
JP
D002648:Child; D002881:Chromosomes, Human, Pair 12; D002892:Chromosomes, Human, Pair 22; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D011379:Prognosis; D018227:Sarcoma, Clear Cell / Q000235:genetics / Q000473:pathology / Q000628:therapy; D013710:Tendons; D013899:Thoracic Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy; D014178:Translocation, Genetic
Sarcome à cellules claires des tendons et aponévroses.