Treatment of Patients With Newly Diagnosed Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumor, or Atypical Teratoid Rhabdoid Tumor

The frequency of mutations for the main genes associated with SHH and WNT tumors identified via targeted sequencing based on formalin fixed paraffin embedded material is provided.

Time frame: within 3.5 years following completion of accrual

Population: Only patients with WNT and SHH tumors with available tissue for targeted sequencing were analyzed for frequency of mutation. WNT and SHH subgroups were identified by methylation profiling.

122 participants with a diagnosis of medulloblastoma were grouped by ERBB2 positive/negative assessment and risk group into 4 groups. Progression-free survival was calculated from the date of diagnosis to the date of disease progression/relapse, the date of death, or the date of last contact. The log-rank test was used to compare the PFS distributions of ERBB2 groups.

Time frame: 2 years after tumor cell analysis in 122 participants

Population: Analysis was completed for the first 122 participants with a diagnosis of medulloblastoma and with fresh tissue and ERBB2 protein assessments. Participants with a diagnosis of PNET, PNET variants, or ATRT were not included in this analysis.

The relationship between ERBB2 protein expression in tumors and progression-free survival was assessed in 122 participants with a diagnosis of medulloblastoma and with ERBB2 protein assessments. If the ERBB2 value was greater than zero, the ERBB2 was defined as positive for the participant. If the ERBB2 value was zero, the ERBB2 was defined as negative. Progression-free survival was calculated from the date of diagnosis to the date of disease progression/relapse, the date of death, or the date of last contact. The log-rank test was used to compare the PFS distributions of ERBB2 groups.

Time frame: 2 years after tumor cell analysis in 122 participants

Population: Analysis included the first 122 participants with a diagnosis of medulloblastoma and with fresh tissue and ERBB2 protein assessments. Participants with a diagnosis of PNET, PNET variants, or ATRT were not included in this analysis.

Assessment of associative memory at 5 years after enrollment. Assessment of associative memory score at enrollment. Associate memory score is a representative measurement of learning and recalling pictograph representations of words. It measures associative memory (Long-Term Retrieval). Mean=100, SD=15, Average Range 85-115. Higher is better.

Time frame: At 5 years after enrollment

Population: Children with medulloblastoma who were age 3-21 years at diagnosis and were enrolled on SJMB03 at St Jude Children's Research Hospital. Patients were excluded if the cumulative RT plan (Craniospinal Irradiation (CSI) and boost) could not be retrieved; if the baseline preRT Magnetic Resonance Image (MRI) scan was of poor quality, preventing delineation of brain substructures; or if the patient had completed fewer than two specific neurocognitive evaluations for this objective.

Assessment of associative memory at enrollment. Associate memory score is a representative measurement of learning and recalling pictograph representations of words. It measures associative memory (Long-Term Retrieval). Mean=100, SD=15, Average Range 85-115. Higher is better.

Time frame: At enrollment

Population: Children with medulloblastoma who were age 3-21 years at diagnosis and were enrolled on SJMB03 at St Jude Children's Research Hospital. Patients were excluded if the cumulative RT plan (Craniospinal Irradiation (CSI) and boost) could not be retrieved; if the baseline preRT Magnetic Resonance Image (MRI) scan was of poor quality, preventing delineation of brain substructures ; or if the patient had completed fewer than two specific neurocognitive evaluations for this objective.

To monitor for treatment failure in the posterior fossa of patients whose tumor bed receives a reduced volume of radiation.

Time frame: Annually for 6 years post irradiation

Population: Analysis included 100 average risk participants with a diagnosis of medulloblastoma who had cumulative radiotherapy dose profiles and treatment failure volumes data available. All participants included in analysis who were still at risk and on-study were followed for a minimum of 6 years. Participants with a diagnosis of PNET, PNET variants, or ATRT were not included in this analysis.

Assessment of Perceptual Speed at 5 years after enrollment. It measures rapidly locating and circling identical numbers from a set of numbers which reflects perceptual speed. Mean=100, SD=15, Average Range 85-115. Higher is better.

Time frame: At 5 years after enrollment

Population: Children with medulloblastoma who were age 3-21 years at diagnosis and were enrolled on SJMB03 at St Jude Children's Research Hospital. Patients were excluded if the cumulative RT plan (Craniospinal Irradiation (CSI) and boost) could not be retrieved; if the baseline preRT Magnetic Resonance Image (MRI) scan was of poor quality, preventing delineation of brain substructures; or if the patient had completed fewer than two specific neurocognitive evaluations for this objective.

Assessment of Perceptual Speed at enrollment. It measures rapidly locating and circling identical numbers from a set of numbers which reflects perceptual speed. Mean=100, SD=15, Average Range 85-115. Higher is better.

Time frame: At enrollment

Population: Children with medulloblastoma who were age 3-21 years at diagnosis and were enrolled on SJMB03 at St Jude Children's Research Hospital. Patients were excluded if the cumulative RT plan (Craniospinal Irradiation (CSI) and boost) could not be retrieved; if the baseline preRT Magnetic Resonance Image (MRI) scan was of poor quality, preventing delineation of brain substructures; or if the patient had completed fewer than two specific neurocognitive evaluations for this objective.

Assessment of Processing Speed at 5 years after enrollment. This score measure of Processing Speed based on Visual Matching and Decision Speed tests. Mean=100, SD=15, Average Range 85-115. Higher is better.

Time frame: At 5 years after enrollment

Population: Children with medulloblastoma who were age 3-21 years at diagnosis and were enrolled on SJMB03 at St Jude Children's Research Hospital. Patients were excluded if the cumulative RT plan (Craniospinal Irradiation (CSI) and boost) could not be retrieved; if the baseline preRT Magnetic Resonance Image (MRI) scan was of poor quality, preventing delineation of brain substructures; or if the patient had completed fewer than two specific neurocognitive evaluations for this objective.

Assessment of Processing Speed at enrollment. This score measure of Processing Speed based on Visual Matching and Decision Speed tests. Mean=100, SD=15, Average Range 85-115. Higher is better.

Time frame: At enrollment

Population: Children with medulloblastoma who were age 3-21 years at diagnosis and were enrolled on SJMB03 at St Jude Children's Research Hospital. Patients were excluded if the cumulative RT plan (Craniospinal Irradiation (CSI) and boost) could not be retrieved; if the baseline preRT Magnetic Resonance Image (MRI) scan was of poor quality, preventing delineation of brain substructures; or if the patient had completed fewer than two specific neurocognitive evaluations for this objective.

SOC is standard-of-Care control group. Patients randomly assigned to the control group received the current standard of care. RI is Reading Intervention Group. Patients randomly assigned to Reading Intervention Group which is with The Fast ForWord program. Assessment of reading decoding was completed using the Woodcock Johnson, Third Edition (WJIII) Tests of Achievement (Woodcock, McGraw, & Mather, 2001), with particular attention given to the reading and reading-related abilities. Two subtests were completed: (1) Letter-Word Identification, and (2) Word Attack, a test requiring the patient to read phonologically regular nonwords. The combination of these two subtests provided a standardized composite score of overall reading decoding ability with a population mean of 100 and a standard deviation of 15. Scores of 90-110 are considered to be in the average range, while those 80-89 are considered low-average (refer: Journal of Pediatric Psychology 39(4) pp. 450-458, 2014).

Time frame: 5 years postdiagnosis

Population: Of the 126 MB patients enrolled, 41 were not eligible. This left 85 patients considered eligible for the randomization study. Of the 85 eligible patients,81 were randomized to either SOC (n= 38) or to RI (n =43). The parents of only four patients did not consent to the intervention study. Two patients randomized to the RI group, and four patients in the SOC group, were excluded due to having only a single assessment, which resulted in 75 patients (RI=41, SOC=34) over a 5-year follow-up period.