Successful dose-intensive treatment of desmoplastic small round cell tumor in three children

Peter Kurre, Judy L. Felgenhauer, James S. Miser, Kathleen Patterson, Douglas S. Hawkins

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


Desmoplastic small round cell tumor (DSRCT) is a rare soft tissue tumor of primitive origin occurring primarily in children and young adults. Based on published reports in the literature, the response to conventional chemotherapy is poor. We report three pediatric patients successfully treated with dose-intensive, multimodal therapy. Between August 1994 and March 1998, we evaluated three consecutive patients with DSRCT at Children’s Hospital and Regional Medical Center, Seattle, Washington. We established the diagnosis based on clinical presentation, radiologic staging, and pathologic review with immunohistochemical staining. All patients received a combined modality protocol including dose-intensive chemotherapy (two of them with peripheral blood stem cell [PBSC] support), second look surgery, and consolidative local irradiation. The patients remain in continuous remission at 66, 42, and 26 months after diagnosis, respectively. Two of our patients were younger than any previously reported patient, extending the age group for which DSRCT should be considered on diagnosis of small round cell tumors. The uniform survival achieved in our series indicates potential benefit for the combination of dose-intensive multiagent chemotherapy, local irradiation, and aggressive surgical approach in this disease.

Original languageEnglish (US)
Pages (from-to)446-450
Number of pages5
JournalAmerican Journal of Pediatric Hematology/Oncology
Issue number5
StatePublished - 2000
Externally publishedYes


  • Chemotherapy
  • Desmoplastic small round cell tumor
  • Dose intensification
  • G-CSF
  • PBSC support
  • Radiation therapy
  • Translocation t(11;22) (p13;q11.2)

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology


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