Desmoplastic small round cell tumor in children: a new therapeutic approach.
OBJECTIVE
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.


CONCLUSIONS
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.
Al Balushi
Zainab
Z
CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada. zainab2@hotmail.com
Bulduc
Sabrina
S
Mulleur
Claudia
C
Lallier
Michel
M
D000008:Abdominal Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016026:Bone Marrow Transplantation; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics / Q000648:ultrastructure; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics / Q000648:ultrastructure; D003131:Combined Modality Therapy; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008113:Liver Neoplasms / Q000188:drug therapy / Q000556:secondary; D008175:Lung Neoplasms / Q000188:drug therapy / Q000556:secondary; D008297:Male; D020360:Neoadjuvant Therapy; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D010386:Pelvic Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D018228:Sarcoma, Small Cell / Q000188:drug therapy / Q000235:genetics / Q000532:radiotherapy / Q000556:secondary / Q000601:surgery / Q000628:therapy*; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000556:secondary; D014178:Translocation, Genetic; D014182:Transplantation, Autologous; D014750:Vincristine / Q000008:administration & dosage
C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D016190:Carboplatin; D007069:Ifosfamide