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J Pediatr Hematol Oncol. 2009 Jan;31(1):61-4. doi: 10.1097/MPH.0b013e31818ab2cf.

Primary mixed glioneuronal tumor of the central nervous system in a patient with noonan syndrome: a case report and review of the literature.

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Children's Center for Cancer and Blood Diseases, Los Angeles, CA, USA.


Noonan syndrome is an autosomal dominant condition with variable phenotypic expression. Although an association between Noonan syndrome and various neoplasms has been identified, a relationship with primary glial or neuronal tumors of the central nervous system (CNS) has not yet been established. We describe the case of a 6-year-old male patient with Noonan syndrome and leptomeningeally disseminated low-grade mixed glioneuronal tumor. After a literature review, this case emerges as the third patient to present with Noonan syndrome and primary CNS glial tumor and the first with mixed glioneuronal tumor, indicating the possible association between these individual entities.

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