TY - JOUR
T1 - Desmoplastic small round cell tumor in children
T2 - a new therapeutic approach
AU - Al Balushi, Zainab
AU - Bulduc, Sabrina
AU - Mulleur, Claudia
AU - Lallier, Michel
PY - 2009/5
Y1 - 2009/5
N2 - Purpose: Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. Methods: Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. Results: Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. Conclusion: The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.
AB - Purpose: Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease. Methods: Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT. Results: Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT. Conclusion: The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.
KW - Autologous bone marrow transplant
KW - Desmoplastic small round cell tumor
KW - Diagnosis
KW - Pathology
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UR - http://www.scopus.com/inward/citedby.url?scp=65449182310&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2009.01.071
DO - 10.1016/j.jpedsurg.2009.01.071
M3 - Article
C2 - 19433176
AN - SCOPUS:65449182310
SN - 0022-3468
VL - 44
SP - 949
EP - 952
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 5
ER -