Diagnosis, treatment and prognostic analysis of pediatric intracranial atypical teratoid/rhabdoid tumor / 中华实用儿科临床杂志

ABSTRACT

Objective:To explore the diagnosis, treatment and prognostic of pediatric intracranial atypical teratoid/rhabdoid tumor(AT/RT).Methods:A total of 15 pediatric patients with intracranial AT/RT were treated between January 2012 and June 2019 at the First Affiliated Hospital of Zhengzhou University.The clinical data were retrospectively analyzed.Overall survival (OS) rate and progression free survival (PFS) rate were calculated by adopting Kaplan- Meier method.The differences between the 2 groups were tested by performing Log- rank method, and the prognostic factors were analyzed by COX regression. Results:There were 12 males and 3 females, with the median age of 5.5 years (ranging from 8 months to 17.1 years). All patients underwent surgical resection.Gross-total resection (GTR) was achieved in 10 cases and subtotal resection (STR) was carried out in 5 patients.The conducted treatments were as follows surgery+ radiotherapy+ chemotherapy+ intrathecal injection in 6 cases, surgery+ chemotherapy+ intrathecal injection in 4 cases, surgery+ radiotherapy in 2 cases, and surgery alone in 3 cases.Until January 2020, the median survival time of all the 15 patients was 18 months (ranged 1-27 months), and the survival rate was 33.3%.The 1-year OS rate and PFS rate for all 15 cases were 71.5% and 49.7%, respectively.The 2-year OS rate and PFS rate were 17.9% and 0, respectively. Log- rank analyses revealed that the 1-year OS rates of children less than 3 years old and those older than 3 years were 87.5% and 57.1%, respectively ( χ2=6.057, P=0.014). The 1-year OS rates of children with GTR and those with STR were 90.0% and 40.0%, respectively ( χ2=6.057, P=0.014). The 1-year OS rates of children with tumor dissemination and those without tumor dissemination were 100.0% and 33.3%, respectively( χ2=9.865, P=0.002). The 1-year OS rates of children in the standard-risk group and those in the high-risk group were 88.9% and 41.7%, respectively ( χ2=5.111, P=0.024). COX regression analyses proved that age, the extent of tumor resection, tumor dissemination and risk stratification are independent risk factors for prognosis [hazard radio( HR)=3.411, 3.795, 5.245, 3.397; P=0.025, 0.011, 0.001, 0.017]. Conclusions:Pediatric intracranial AT/RT is rare.The preliminary diagnosis and prognosis are difficult and poor, respectively.The complete resection of tumors with maximal safety remains the primary treatment.Age, the extent of tumor resection, tumor dissemination and risk stratification are independent prognostic factors for AT/RT children.