- 4.5 months mean time to progression: These aggressive tumors remain refractory to treatment. Median time to progression is 4.5 months.
- Median survival of 6 months: In the original series of Rorke et al., the survival period was very short (13).
- Dependent on duration and severity: Recovery from neurological deficit seems inversely proportional to the severity of the deficit at presentation and the duration of symptoms. In each of the moderate or severe deficit groups in the Pediatric Oncology Group series (8), 40% showed good recovery compared with 77% in the mild deficit group. In this series poor recovery was likely for patients with paraparesis of more than 1 week in duration.
- Metastases rare but poor prognosis: CNS metastases are uncommon (prevalence for newly diagnosed disease, 4.3%) but confer a poor prognostic status. The median survival is 7.9 months with CNS metastases at presentation and 3.2 months for patients who develop CNS disease during treatment (2).
Desmoplastic Infantile Gangliogliomas
- Dependent on extent of resection: Outcome can be very good for infants with these tumors despite the often large size of the tumors. Progression-free survival rates and the potential for cure seem to be related to the extent of resection.
- 6-month expected survival: Prognosis is poor with average survival of 6 months (16).
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