Medulloblastoma Variants and Survival at a Children's Hospital
DW Sandquist, JM Nadell, RD Craver. Louisiana State University Health Science Center, New Orleans, LA; Children's Hospital of New Orleans, New Orleans, LA
Background: Medulloblastoma variants recognized by WHO- classic (NOS), desmoplastic (DES), large cell (LC), anaplastic (ANA), excessive nodularity (EN)- may have prognostic differences. ANA and LC may be poor, EN and DES may be more favorable.
Design: To assess the spectrum, frequency, and survival of variants, we reviewed our 25 year experience with medulloblastomas diagnosed, treated, and followed by Children's Hospital. Slides were reviewed and classified according to WHO . Follow-up was provided by the tumor registry. The Kaplan-Meier method was used for cumulative survival statistics.
Results: 35 children had slides for review, the variants included NOS 22 (63%), ANA 5(14%), DES 4(11%), EN 3(9%), and LC 1(3%). 34 had follow-up. Cumulative survival statistics were calculated.
|Variant||N||1 yr survival||2 yr survival||5 yr survival||10 yr survival||15 yr survival|
|< 3 yrs||8||.875||.4379||.4379||.4379||.4379|
|3 yrs or older||26||.9200||.9200||.7375||.6392||.5114|
8 children under 3 yrs at diagnosis included 4 NOS, 2 ANA, 2 EN. 4 died within 2 yrs. The 2 with follow-up more than 2 yrs have survived 17(NOS) and 19 (EN) yrs. In the 3yrs or older children, 2 deaths occurred between 5 and 10 yrs (NOS and DES, both at 8 yrs), and 1 recurred and died at 14 yrs (DES). All 4 with DES died, 2 with late recurrences (8 and 14 yrs). 2 EN have survived 19yrs (dx'd at 1 yr) and 17 yrs(dx'd at 4 yrs). 2/5 ANA were < 3yrs at dx. None of the 4 DES were < 3yrs at dx. The one LC is alive at 18 yrs. No second malignancies have occurred.
Conclusions: NOS variant was the most frequent. None of our DES survived, with 2/4 deaths occurring after 5 yrs. Our data supports that EN carries a good prognosis, with no deaths (follow-up of 1,17, and 19 yrs). Of the 4 ANAs, 1 died, but the others are recent without 5 yrs of follow-up. Those less than 3 yrs of age continue to do poorly, with deaths within 2 yrs, unless the variant is EN. Late deaths occur, and continued follow-up of these children is required.
Tuesday, March 10, 2009 9:30 AM
Poster Session III # 198, Tuesday Morning