TY - JOUR
TI - Familial medulloblastoma
AU - Moschovi, M
AU - Sotiris, Y
AU - Prodromou, N
AU - Tsangaris, GT and
AU - Van-Vliet, CC
AU - Kalpini-Mavrou, A
AU - Tzortzatou-Stathopoulou, F
JO - Pediatric Hematology and Oncology
PY - 1998
VL - 15
TODO - 5
SP - 421-424
PB - HEMISPHERE PUBL CORP
SN - 0888-0018, 1521-0669
TODO - 10.3109/08880019809016570
TODO - familial medulloblastoma; medulloblastoma
TODO - Medulloblastomas account for 20 % of all primary brain tumors. The vast
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
ages and all of them have died, usually within the first 2 years
following diagnosis. The authors describe a case of familial
medulloblastoma with unusual characteristics: Two siblings of different
sex and a second-degree relative have presented at exactly the same age
of 18 months. The histologic pattern was the same in all patients, that
of desmoplastic 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.
ER -