J Korean Med Sci.  2012 Feb;27(2):135-140. 10.3346/jkms.2012.27.2.135.

Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Young Children with Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System

• Park ES
• Sung KW
• Baek HJ
• Park KD
• Park HJ
• Won SC
• Lim DH
• Kim HS

• KMID: 1120149
• DOI: http://doi.org/10.3346/jkms.2012.27.2.135

Abstract

The feasibility and effectiveness of tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT) were evaluated in children younger than 3 yr of age with atypical teratoid/rhabdoid tumors (ATRT). Tandem HDCT/autoSCT was administered following six cycles of induction chemotherapy. Radiotherapy (RT) was administered if the tumor relapsed or progressed, otherwise, it was administered after 3 yr of age. Tumors relapsed or progressed during induction chemotherapy in 5 of 9 patients enrolled; 3 of these 5 received tandem HDCT/autoSCT as a salvage treatment. One patient died from sepsis during induction chemotherapy. The remaining 3 patients proceeded to tandem HDCT/autoSCT; however, 2 of these patients showed tumor relapse/progression after tandem HDCT/autoSCT. All 7 relapses/progressions occurred at primary sites even in patients with leptomeningeal seeding. Toxicities during tandem HDCT/autoSCT were manageable. A total of 5 patients were alive with a median follow-up of 20 (range 16-70) months from diagnosis. Four of 5 patients who received RT after relapse/progression are alive. The probability of overall survival at 3 yr from diagnosis was 53.3% +/- 17.3%. Our tandem HDCT/autoSCT is feasible; however, early administration of RT prior to tandem HDCT/autoSCT should be considered to improve the outcome after tandem HDCT/autoSCT.

Keyword

Rhabdoid Tumor; Central Nervous System; Drug Therapy; Stem Cell Transplantation; Radiotherapy; Child

MeSH Terms

Antineoplastic Combined Chemotherapy Protocols/*therapeutic use
Carboplatin/administration & dosage
Central Nervous System Neoplasms/drug therapy/radiotherapy/*therapy
Child, Preschool
Combined Modality Therapy
Cyclophosphamide/administration & dosage
Etoposide/administration & dosage
Female
Follow-Up Studies
Humans
Induction Chemotherapy
Infant
Male
Prospective Studies
Recurrence
Rhabdoid Tumor/drug therapy/radiotherapy/*therapy
Salvage Therapy
*Stem Cell Transplantation
Survival Rate
Thiotepa/administration & dosage
Transplantation, Autologous

Figure

• 

  Fig. 1 A total of five patients are alive with a median follow-up of 20 (range 16-70) months from diagnosis. The probabilities of overall survival (OS) and event-free survival (EFS) at 3 yr from diagnosis were 53.3% ± 17.3% and 0%, respectively (A). The probabilities of overall survival and progression-free survival (PFS) at 3 yr from the first HDCT/autoSCT were 80.0% ± 17.9% and 31.2% ± 24.5%, respectively (B). The probabilities of overall survival and progression-free survival at 3 yr from the initiation of RT were 80.0% ± 17.9% and 53.3% ± 24.8%, respectively (C).

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