Brain tumors medulloblastomas

Earlier in this chapter, I introduced Ei-Fraumeni syndrome (EPS), a germline mutation of the p53 gene on the short arm of chromosome 17. Medulloblastoma and PNET account for about 10% of all brain tumors associated with a germline mutation of the p53 gene... 

Raimondi AJ, Tomita T The disadvantages of prophyiactic whole CNS postoperative radiation therapy for medulloblastoma, in Multidisciplinary Aspects of Brain Tumor Therapy. Edited by Paoletti P, Walker MD, Butti G, et al Amsterdam, Elsevier, 1979, pp 209-218... 

Notch. Anaplastic astrocytoma, ependymoma, medulloblastoma, Jag-activated Notch. Click Feletti A et al Brain Tumor Pathol 2014 March 19 in print Fouladi M et al J Neurooncol 2013 114 173-9 Killela PJ et al Oncotarget 2013 Oct 16 in print Mascaro Cordeiro B et al Childs Nerv Syst 2014 Apr 3 in print. 

As far as we know persistent thymine-uraciluria has been described once before, in a child with a fatal medulloblastoma (10). It seemed likely that the brain tumor caused the high excretion of uracil and thymine, because there was a correlation between their excretion rates and the clinical progress of symptoms, remission after therapy and relapse. In our patient, however, no signs of malignancy have developed during the four years of observation. Therefore an inherited enzyme defect is held as the most probable cause. 

Figure 1. (Continued) (3) Brain with a malignant tumor of childhood (medulloblastoma) spin echo images m (a) transverse and (b) sagittal planes The tumor is much lighter than brain (4) Spinal cord, spin echo images (a) normal spine, and (b) cord expanded and irregular due to the presence of an intramedullary tumor...

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