Background: SPT are late effects of childhood PFBT. Low dose radiation scatter at the edge of craniospinal field has traditionally been blamed for meningiomas and thyroid tumours but the effect of chemotherapy, genetic predisposition and GH replacement on their prevalence is less clear.
Methods and aims: As part of a descriptive study of long term (>10 years) functional, endocrine and cognitive outcomes in 36 (20 males) adults aged 21.2 (1632) years, of whom 34 had childhood GH after surviving therapies for PFBT at 6.9 (0.116.6) years, we performed surveillance MRI Brain scans. We also recorded non-CNS SPTs, prior oncological therapy and genetic predisposition.
Results: Over a median 14.7 (9.327.4) years follow-up, we documented ten SPT in seven patients all of whom had prior craniospinal irradiation as well as two screen-detected cavernomas. One patient had two SPTs before 16 years of age (thyroid Ca and bowel) after a medulloblastoma (MB) at 5 years and was found to have familial APC gene defect. Another treated for MB at 2 years with additional chemotherapy had BCC, thyroid adenoma and asymptomatic meningioma, all resected before 32 years of age and had never received childhood GH. Five other patients had one SPT each; three screen-detected meningiomas (two resected, one with prior chemotherapy), and two BCCs.
Conclusion: Approximately 20% (1:5) of our PFBT cohort developed at least one SPT within a median 15 years period, of whom 2 (0.5%), one with a familial predisposition, the other with additional chemotherapy (and no GH) had more than one. Excepting the familial patient, all SPTs were low grade and curable by surgery. These data are useful baselines against future changes in prevalence with increasing use of high intensity adjuvant chemotherapy can be potentially assessed. It is unlikely that GH treatment has contributed.
09 - 11 Nov 2011
British Society for Paediatric Endocrinology and Diabetes