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

Zainab Al Balushi, Sabrina Bulduc, Claudia Mulleur, Michel Lallier

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)949-952
Number of pages4
JournalJournal of Pediatric Surgery
Volume44
Issue number5
DOIs
Publication statusPublished - May 2009

Keywords

  • Autologous bone marrow transplant
  • Desmoplastic small round cell tumor
  • Diagnosis
  • Pathology

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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