ESPE2023 Poster Category 1 Multisystem Endocrine Disorders (28 abstracts)
Marmara University, Pediatric Endocrinology and Diabetes Department, Istanbul, Turkey
Introduction: Medulloblastoma is the most common central nervous system tumour in children representing, approximately 20% of childhood brain tumours. Cure can be achieved with surgery, chemotherapy and radiotherapy. However, disease survivors encounter long term endocrine complications including short stature due to growth hormone (GH) deficiency and spinal radiotherapy.
Patients and Methods: We aimed to investigate long-term endocrine complications of childhood Medulloblastoma by reviewing charts of 18 patients treated for medulloblastoma and reached final height in our center.
Results: The mean follow-up period was 6.60 years (1.34-11.42). All patients had GH deficiency in whom 10 patients received GH treatment. Hypothyroidism was detected in 15 patients (83.3%), Hypergonadotropic hypogonadism was detected in 14 patients (77.7%), seven of them were in the GH treated group. Additionally, one patient developed precocious puberty and was treated with GnRHa. No recurrence/secondary malignancy was observed in any patient during the follow-up. The mean age of the patients at presentation in GH treated group (5M/5F) was 9.4 years (±3.1), and the mean age of onset of GH treatment was 11.41 years (±3.59). The mean pre-treatment IGF1 and IGFBP-3 SDS values were -1.65 and 0,98, respectively. The mean peak GH value was 3.15 (±1.15), (0.72-6.09) mcg/L. The mean age at presentation of untreated patients (4M/4F) was 11.39 (±3.1) years. At admission, the mean SDS values of IGF1 and IGFBP-3 were -1.54 and -0,92, respectively. The mean IH SDS and FH-SDS were -1.55 (±1.38) and -3.90 (±1.37), respectively. While there was no significant difference between IGF-1 and IGFBP-3 SDSs and IH-SDSs between the groups (P=0.877, P=0.44, P=0.928, respectively), The loss of FH SDS from IH SDS was lesser in GH treated group than the untreated group (the difference between treated and untreated groups FHSDS was 1.6 SDS and significant, P=0.0012).
Conclusion: GH deficiency is uniformly seen in addition to hypothyroidism and hypogonadism which are seen approximately 80% in survivors of medulloblastoma treatment in our cohort. Although there is a decrease in the height SDS of the patients from diagnosis to the final height, FH loss were partially compensated in the patients who received GH treatment.