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BACKGROUND Desmoplastic small round cell tumors (DSRCT) have been only recently identified. METHODS The authors report DSRCT in two pediatric patients (an 8-year-old boy and 12-year-old boy). In both patients, the initial diagnosis was rhabdomyosarcoma. The resistance to standard chemotherapy and radiation therapy prompted the authors to review the initial biopsy specimens and perform complementary immunophenotypic characterization. RESULTS These analyses revealed that the tumor cells were strongly positive for keratin epithelial marker antigen, desmin, vimentin, neurospecific enolase, and S100 protein, corresponding to pleomorphic differentiation, characteristic of DSRCT: CONCLUSIONS The authors suggest that extensive immunohistologic characterization be performed in all cases of small round cell tumors of the abdomen so that the diagnosis of DSRCT is not overlooked. These rare tumors are refractory to chemotherapy, and initial aggressive surgery is warranted.
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
January 2008,
Indian journal of pathology & microbiology,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
March 1999,
Radiology,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
November 2023,
Medicine,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
January 2007,
Singapore medical journal,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
June 2017,
Future oncology (London, England),
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
April 2007,
Pathologica,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
January 2022,
Frontiers in oncology,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
January 2015,
Journal of the West African College of Surgeons,
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
January 2013,
Internal medicine (Tokyo, Japan),
D Frappaz, and
E Bouffet, and
D Dolbeau, and
R Bouvier, and
C Carrie, and
D Louis, and
C Pondarre, and
E Tabone, and
T Philip, and
M Brunat-Mentigny
September 2018,
JAMA oncology,
