Primitive neuroectodermal tumor
Medulloblastoma is an intracranial primitive neuroectodermal tumor that occurs primarily within the posterior fossa.
Medulloblastoma is one of the most frequent intracranial tumors in children, accounting for 3.74% of all intracranial tumors and 28–69% of all posterior fossa tumors. The peak frequency is between ages 3 and 8 years (Choux et al. 2001). Medulloblastomas have a male predominance that ranges from 1.4–4.8 to 1.0 depending on the study (Albright 1999). These neoplasms usually occur as independent tumors, although familial occurrences have been reported. There is an increased frequency of medulloblastomas in Gorlin’s and Turcot’s syndromes. Gorlin’s syndrome is characterized by nevoid basal cell carcinomas, jaw cysts, and skeletal and CNS abnormalities (Albright 1999). Turcot Syndrome is an association between familial adenomatous polyposis and brain tumors (Chen 1998).
References and Readings
- Albright, A. L. (1999). Medulloblastomas. In A. L. Albright, I. F. Pollack, & P. D. Adelson (Eds.), Principles and practice of pediatric neurosurgery (1st ed., pp. 591–608). New York: Thieme.Google Scholar
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