Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group

Détails

ID Serval
serval:BIB_5161EAE8A208
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group
Périodique
American Journal of Surgical Pathology
Auteur(s)
Begueret  H., Galateau-Salle  F., Guillou  L., Chetaille  B., Brambilla  E., Vignaud  J. M., Terrier  P., Groussard  O., Coindre  J. M.
ISSN
0147-5185 (Print)
Statut éditorial
Publié
Date de publication
2005
Volume
29
Numéro
3
Pages
339-346
Notes
PT - Journal Article PT - Research Support, Non-U.S. Gov't
Résumé
Synovial sarcoma (SS), an aggressive neoplasm accounting for up to 14% of soft tissue sarcomas, was recently recognized as a primary tumor in the lung and pleura. SS is characterized by the chromosomal translocation t(X;18)(SYT-SSX) found in more than 95% of the tumors. We report a cooperative study from the French Sarcoma Group and the Mesopath Group on 40 t(X;18)(SYT-SSX)-positive primary intrathoracic SS. There were 22 males and 18 females, whose age ranged from 16 to 79 years (median, 47 years). Neoplasms were mostly circumscribed and of large size (median, 7.5 cm; range, 2-16 cm). Thirty-nine tumors were monophasic SS, including 24 (60%) monophasic fibrous and 15 (37.5%) poorly differentiated cases, and one lesion was a biphasic SS. A larger proportion of poorly differentiated tumors were observed among intrathoracic SS as compared with soft tissue SS. Immunohistochemically, 90% of the cases reacted with at least one epithelial marker. CD34 was focally expressed in 3 cases. SYT-SSX1 fusion transcripts were detected in 22 cases (56.4%) and SYT-SSX2 fusion transcripts in 17 cases. Median and 5-year disease-specific survival in 33 patients was 50 months and 31.6%. Median and 5-year disease-free survival was 24 months and 20.9%. Patient sex, age, tumor size, histologic subtype, grade, and SYS-SSX fusion type had no significant impact on outcome. In conclusion, intrathoracic SS are rare but aggressive tumors with poor prognosis. In this unusual location, the detection of SYT-SSX fusion transcripts is a valuable diagnostic adjunct
Mots-clé
Adolescent/Adult/Aged/Chromosomes,Human,Pair 18/Chromosomes,Human,X/Female/Humans/Immunoenzyme Techniques/Male/Middle Aged/Oncogene Proteins,Fusion/analysis/Sarcoma,Synovial/chemistry/genetics/secondary/Soft Tissue Neoplasms/Pathology/Thoracic Neoplasms/Translocation,Genetic/Tumor Markers,Biological
Pubmed
Web of science
Création de la notice
29/01/2008 19:33
Dernière modification de la notice
03/03/2018 17:11
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