Medulloblastomas account for 20 % of all primary brain tumors. The vas
t majority of them are sporadic. Familial medulloblastoma is very rare
-only a few cases have Deem reported worldwide. Most were observed in
siblings of the same sex. The affected children presented at various a
ges and all of them have died, usually within the first 2 years follow
ing diagnosis. The authors describe a case of familial medulloblastoma
with unusual characteristics: Two siblings of different sex and a sec
ond-degree relative have presented at exactly the same age of 18 month
s. The histologic pattern was the same in all patients, that of desmop
lastic medulloblastoma. All patients are alive and remain in remission
12, 5, and 11 years, respectively, after diagnosis. The genetics and
the pathogenesis of the disease remain obscure.