Purpose: To present the effect of radiotherapy after subtotal excision of craniopharyngioma in childhood and the problems associated with the side-effects after radiotherapy of that rare benign tumor in children.
Patients And Methods: The study included 8 children with craniopharyngioma, treated with subtotal excision followed by radiotherapy. The median period of observation was 4 years. Radiotherapy was delivered with Co60 telegammatherapeutic machine (Rokus), with daily radiation dose (DRD) of 1.5-1.8 Gy to a total radiation dose (TRD) of 50-56 Gy.
Results: All 8 treated children achieved local tumor control (LTC) which was verified with dynamic observations by computed tomography (CT) and magnetic resonance imaging (MRI). In 50% of the cases the eye changes (vision, papillae, perimetric vision) were normalized, and in the remainder an improvement was noted. No child developed neurological or psychical disturbances and no memory or concentration problems ever appeared. From them 50% are very good students.
Conclusion: Radiotherapy is the main method for achieving a long-term LTC after subtotal excision for craniopharyngioma in childhood. The radiation dose used was well tolerated by brain structures and no late neurological sequelae were observed in our patients.
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Ann Pediatr Endocrinol Metab
December 2024
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated.
View Article and Find Full Text PDFJ Neurooncol
January 2025
Department of Endocrinology, Genetics and Metabolism, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, Beijing, 100045, China.
Background: Craniopharyngioma (CP), a benign tumor originating from remnants of Rathke's pouch in the sellar region, accounts for approximately 30% of all cases of craniopharyngioma. Radiation therapy has been used to treat CP patients for decades; however, there is still a lack of systematic reviews on the long-term tumor control outcomes in pediatric CP patients treated with external radiation therapy.
Methods: We conducted a comprehensive search of multiple databases for studies on the tumor progression rates of childhood-onset CP(COCP) patients who received external radiotherapy.
Purpose: Craniopharyngiomas (CPs) often lead to growth hormone deficiency (GHD) in children. Growth hormone replacement therapy (GHRT) is essential for managing GHD but its impact on body mass index (BMI) and metabolic outcomes is controversial. Concerns exist that GHRT might contribute to tumor recurrence, with guidelines varying on when to start therapy post-surgery.
View Article and Find Full Text PDFBMC Cancer
December 2024
Key Laboratory of Endocrinology of National Health Commission, Department of Endocrinology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Background: The treatment of craniopharyngiomas (CPs) poses challenges due to their proximity to critical neural structures, the risk of serious complications, and the impairment of quality of life after treatment. However, long-term prognostic data are still scarce. Therefore, the purpose of this retrospective study is to evaluate the long-term outcomes of patients with CPs after treatment.
View Article and Find Full Text PDFChilds Nerv Syst
November 2024
Department of Neurology - Discipline of Neurosurgery, Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, SP, Brazil.
Purpose: Adjuvant radiotherapy has been a standard of care for craniopharyngioma. Nevertheless, it is a rare disease with multiple presentations, and results with conservative surgery followed by radiotherapy (RT) can vary. We compared treatment results for both adult and pediatric patients.
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