Nodule Formation and Desmoplasia in Medulloblastomas—Defining the Nodular/Desmoplastic Variant and Its Biological Behavior

摘要: Among the variants of medulloblastoma in current WHO classification nervous system tumors, desmoplastic variant, which has been reported to constitute 5%-25% pediatric medulloblastomas, is defined by its nodular collections neurocytic cells bounded internodular zones. We have studied frequency, morphological features and biological behavior medulloblastomas two contemporaneous SIOP/UKCCSG trial cohorts children with CNS9102 (n = 315) CNS9204 35), focusing on tumors phenotypes. In aged 3-16 years (CNS9102), nodular/desmoplastic represented 5% all while infants <3 (CNS9204) this variant 57% medulloblastomas. Using iFISH detect molecular cytogenetic abnormalities a architecture, we demonstrated distinct genetic profiles non-desmoplastic (classic anaplastic) tumors; particular, chromosome 17 occurred latter, but not former. Significantly different outcomes were for classic, large cell/anaplastic both cohorts. conclusion, appears clinical, characteristics that set it apart from other tumor.