Clin Pediatr Hematol Oncol.  2015 Oct;22(2):112-119. 10.15264/cpho.2015.22.2.112.

Clinical Characteristics and Treatment Outcome of the Desmoplastic Small Round Cell Tumor

• Lee HJ
• Choi JY
• Hong CR
• Lee JW
• Kang HJ
• Park KD
• Shin HY
• Ahn HS

• KMID: 2084610
• DOI: http://doi.org/10.15264/cpho.2015.22.2.112

Abstract

BACKGROUND
Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy with a poor prognosis. DSRCT is a rare disease, and therefore a standard treatment regimen has not been established. In this study, we reviewed the clinical characteristics and treatment outcomes of pediatric DSRCT patients.
METHODS
We retrospectively reviewed the medical records of 5 DSRCT patients (2 boys, 3 girls) that were diagnosed and treated with DSRCT at Seoul National University Children's Hospital from January 1999 to January 2015.
RESULTS
The median age at diagnosis was 11 years 5months (range 4 years 10 months-17 years 2 months). The most frequent symptoms were abdominal pain (60%). The primary sites were gastrointestinal tract, bladder, and omentum, and the involved sites were the liver, gastrointestinal tract, bladder and bone. Three patients had multiple metastases at diagnosis. Two patients underwent upfront surgical excision of primary tumor, and the remaining 3 patients received neo-adjuvant chemotherapy after the diagnosis was confirmed by using needle biopsy. Combination chemotherapy was administered to all patients in addition to radiotherapy (median dose 45 Gy, range 17.5-54 Gy). Four patients showed disease progression or relapse, resulting in a 20% overall survival rate. At the time of analysis, one patient is alive. She had localized disease at the time of diagnosis and were treated with upfront surgery, chemotherapy, and high-dose chemotherapy with autologous stem cell transplantation and radiotherapy.
CONCLUSION
Patients with DSRCT have a poor prognosis, even after multimodal treatment. Further studies are needed to determine the prognostic factors of DSRCT.

Keyword

Desmoplastic small round cell tumor; Pediatrics; Korea

MeSH Terms

Abdominal Pain
Biopsy, Needle
Combined Modality Therapy
Desmoplastic Small Round Cell Tumor*
Diagnosis
Disease Progression
Drug Therapy
Drug Therapy, Combination
Gastrointestinal Tract
Humans
Korea
Liver
Medical Records
Neoplasm Metastasis
Omentum
Pediatrics
Prognosis
Radiotherapy
Rare Diseases
Recurrence
Retrospective Studies
Seoul
Stem Cell Transplantation
Survival Rate
Treatment Outcome*
Urinary Bladder