Abstract
Suprasellar tumors are the second most common supratentorial tumors in children less than 12 years of age. The two most common lesions in this area are the craniopharyngioma and the hypothalamic /chiasmatic astrocytoma. The majority of the astrocytic tumors are histologically low grade, however, they are unpredictable and may act aggressively especially in infants. Our experience with more than 50 children with this tumor indicates that radiation therapy (RT) can delay the time to tumor progression and may even prolong survival. Although RT is effective its side effects in infants is unacceptable. We have shown that various chemotherapy regimens are effective in halting growth or reducing tumor size in approximately 70% of children treated (this is true for children of all ages). Chemotherapy may allow RT to be delayed until after infancy and on rare occassions for extended periods of time even avoiding RT. Surgical debulking is frequently possible witn minimal morbidity and mortality. Long term survival is common in this disorder (5- and 10-year survival has been 93 and 74% respectively in our experince) and neuroendocrine dysfunction is the rule rather than the exception. Our approach to diagnosis, management, and long term outcome will be presented. Treatment of craniopharyngioma remains controversial but, it is essentially a surgically curable tumor, with 10 year survivals in excess of 90% in those tumors with a post-operative MR scan confirming a total resection. Subtotally resected or recurrent tumors require and respond to RT which delays recurrence and prolongs survival. Neuroendocrine and neuropsychological dysfunction is common in this group of children no matter which form of therapy is employed. If time allows, our experience and recomendations for treatment of craniopharyngioma will also be presented.
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Edwards, M. HYPOTHALAMIC SURGERY AND CHEMOTHERAPY. Pediatr Res 33 (Suppl 5), S11 (1993). https://doi.org/10.1203/00006450-199305001-00049
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DOI: https://doi.org/10.1203/00006450-199305001-00049