Table 1.
Cohort Characteristics.
| Number (%) of patients | Age at diagnosis (median [range] in years) | Gender distribution (n [%] female) | Metastatic disease at diagnosis (n [%]) | Subsequent recurrence or progression (n [%]) | Number (%) Alive at Last Follow-up | Time to last follow-up (median [range] in years) | |
|---|---|---|---|---|---|---|---|
| Entire cohort | 114 | 7.1 (0.1 – 29.3) | 44 (39%) | 15 (13%) | 28 (25%) | 102 (89%) | 7.7 (0.2 – 30.3) |
| By Histopathologic diagnosis: | |||||||
| Meningioma | 13 (11%) | 14.3 (6.0 – 29.3) | 7 (54%) | 0 (0%) | 4 (31%) | 13 (100%) | 11.2 (0.8 – 25.5) |
| Medulloblastoma | 38 (33%) | 6.5 (0.9 – 17.6) | 6 (16%) | 8 (21%) | 3 (8%) | 37 (97%)* | 9.0 (0.2 – 26.7) |
| Pineoblastoma | 4 (4%) | 6.8 (0.8 – 21.9) | 3 (75%) | 2 (50%) | 0 (0%) | 4 (100%) | 2.3 (1.5 – 15.3) |
| ATRT | 3 (3%) | 1.3 (0.8 – 4.9) | 2 (67%) | 0 (0%) | 0 (0%) | 2 (67%)# | 11.3 (1.3 – 13.8) |
| ETMR | 3 (3%) | 2.6 (2.2 – 3.7) | 2 (67%) | 1 (33%) | 1 (33%) | 2 (67%) | 0.3 (0.3 – 6.9) |
| Other embryonal tumors a | 7 (6%) | 2.1 (1.2 – 15.7) | 2 (29%) | 3 (43%) | 2 (29%) | 5 (71) | 10.4 (1.8 – 22.1) |
| Ependymoma | 25 (22%) | 7.0 (1.0 – 18.1) | 13 (52%) | 0 (0%) | 5 (20%) | 25 (100%) | 8.3 (0.7 – 30.3) |
| High-grade glioma | 21 (18%) | 10.3 (0.1 – 27.8) | 9 (43%) | 1 (5%) | 13 (62%) | 14 (67%) | 4.4 (0.3 – 22.8) |
Demographic and clinical features of patients included in the analysis, summarized overall and within histopathologic diagnosis subgroups.
a
The subgroup designated “other embryonal tumors” includes six patients with tumors formerly classified as CNS primitive neuro-ectodermal tumor (PNET) and one patient more recently diagnosed with embryonal tumor, not otherwise specified (NOS).
*,# Cause of death was disease recurrence or progression for deceased patients in the cohort except one patient with medulloblastoma (sequalae of treatment-related co-morbidities) and one patient with ATRT (complications of brainstem necrosis).