Abstract
Background: Atypical teratoid/rhabdoid tumor (ATRT) is a rare malignant pediatric brain tumor with a dismal prognosis. We evaluated the efficacy of multimodal therapy in children with ATRT. Procedure: Nine children diagnosed with cranial ATRT, who received multimodal therapy between 2005 and 2014, including surgical resection followed by radiotherapy (RT), systemic chemotherapy (CT), and high-dose chemotherapy/stem cell transplantation (HDCT/SCT), were analyzed retrospectively. The median age at diagnosis was 32 months. Initial treatment included surgery in eight patients (88%), CT in all (100%), RT in eight (88%), and HDCT/SCT in seven (78%). Results: The median follow-up period for survivors was 21 months. The 2-year progression-free rate was 66.7%. Two patients had progression 4 and 17 months after diagnosis. One received multimodal treatment, including surgery and upfront CT with delayed RT; the other underwent surgery and upfront CT without RT. The 2-year event-free survival and overall survival rates were 46.7% and 62.2%, respectively. Hematologic toxicity of grade 3 or more was observed in six patients treated with HDCT/SCT and two who underwent craniospinal irradiation. Deaths were attributed to progressive disease (n = 2) and treatment-related toxicity (n = 2) from sepsis and acute respiratory failure after CT and HDCT/SCT. Conclusions: Maximal safe resection in conjunction with upfront RT is a reasonable multimodal treatment in patients with ATRT for prolonging progression-free survival. Further research may help determine the optimal parameters for reducing treatment toxicity, such as intensity of HDCT/SCT and the RT field.
| Original language | English |
|---|---|
| Article number | e26663 |
| Journal | Pediatric Blood and Cancer |
| Volume | 64 |
| Issue number | 12 |
| DOIs | |
| Publication status | Published - 2017 Dec 1 |
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All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Hematology
- Oncology
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Atypical teratoid/rhabdoid tumors in children treated with multimodal therapies : The necessity of upfront radiotherapy after surgery. / Lee, Jeongshim; Kim, Dong Seok; Han, Jung Woo; Suh, Chang-Ok.
In: Pediatric Blood and Cancer, Vol. 64, No. 12, e26663, 01.12.2017.Research output: Contribution to journal › Article
TY - JOUR
T1 - Atypical teratoid/rhabdoid tumors in children treated with multimodal therapies
T2 - The necessity of upfront radiotherapy after surgery
AU - Lee, Jeongshim
AU - Kim, Dong Seok
AU - Han, Jung Woo
AU - Suh, Chang-Ok
PY - 2017/12/1
Y1 - 2017/12/1
N2 - Background: Atypical teratoid/rhabdoid tumor (ATRT) is a rare malignant pediatric brain tumor with a dismal prognosis. We evaluated the efficacy of multimodal therapy in children with ATRT. Procedure: Nine children diagnosed with cranial ATRT, who received multimodal therapy between 2005 and 2014, including surgical resection followed by radiotherapy (RT), systemic chemotherapy (CT), and high-dose chemotherapy/stem cell transplantation (HDCT/SCT), were analyzed retrospectively. The median age at diagnosis was 32 months. Initial treatment included surgery in eight patients (88%), CT in all (100%), RT in eight (88%), and HDCT/SCT in seven (78%). Results: The median follow-up period for survivors was 21 months. The 2-year progression-free rate was 66.7%. Two patients had progression 4 and 17 months after diagnosis. One received multimodal treatment, including surgery and upfront CT with delayed RT; the other underwent surgery and upfront CT without RT. The 2-year event-free survival and overall survival rates were 46.7% and 62.2%, respectively. Hematologic toxicity of grade 3 or more was observed in six patients treated with HDCT/SCT and two who underwent craniospinal irradiation. Deaths were attributed to progressive disease (n = 2) and treatment-related toxicity (n = 2) from sepsis and acute respiratory failure after CT and HDCT/SCT. Conclusions: Maximal safe resection in conjunction with upfront RT is a reasonable multimodal treatment in patients with ATRT for prolonging progression-free survival. Further research may help determine the optimal parameters for reducing treatment toxicity, such as intensity of HDCT/SCT and the RT field.
AB - Background: Atypical teratoid/rhabdoid tumor (ATRT) is a rare malignant pediatric brain tumor with a dismal prognosis. We evaluated the efficacy of multimodal therapy in children with ATRT. Procedure: Nine children diagnosed with cranial ATRT, who received multimodal therapy between 2005 and 2014, including surgical resection followed by radiotherapy (RT), systemic chemotherapy (CT), and high-dose chemotherapy/stem cell transplantation (HDCT/SCT), were analyzed retrospectively. The median age at diagnosis was 32 months. Initial treatment included surgery in eight patients (88%), CT in all (100%), RT in eight (88%), and HDCT/SCT in seven (78%). Results: The median follow-up period for survivors was 21 months. The 2-year progression-free rate was 66.7%. Two patients had progression 4 and 17 months after diagnosis. One received multimodal treatment, including surgery and upfront CT with delayed RT; the other underwent surgery and upfront CT without RT. The 2-year event-free survival and overall survival rates were 46.7% and 62.2%, respectively. Hematologic toxicity of grade 3 or more was observed in six patients treated with HDCT/SCT and two who underwent craniospinal irradiation. Deaths were attributed to progressive disease (n = 2) and treatment-related toxicity (n = 2) from sepsis and acute respiratory failure after CT and HDCT/SCT. Conclusions: Maximal safe resection in conjunction with upfront RT is a reasonable multimodal treatment in patients with ATRT for prolonging progression-free survival. Further research may help determine the optimal parameters for reducing treatment toxicity, such as intensity of HDCT/SCT and the RT field.
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U2 - 10.1002/pbc.26663
DO - 10.1002/pbc.26663
M3 - Article
VL - 64
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 12
M1 - e26663
ER -