Introduction: Proton beam therapy (PBT) is being used increasingly for craniopharyngioma, as it is perceived to be a major benefit. There are some limited data relating to endocrine dysfunction following PBT, but very limited data on hypothalamic disturbance. Here we report two patients who presented with hypothalamic disruption immediately following PBT for craniopharyngioma.
Case 1: A 12 year old girl presented with seizure. MRI revealed obstructive hydrocephalus and suprasellar tumor. At surgery hydrocephalus was drained and ommaya reservoir inserted. Tumour biopsy confirmed craniopharyngioma. A near total resection was performed (minimal residual tumor adherent to the posterior cerebral artery and hypothalamus). She developed multiple pituitary hormone deficits (MPHD) post near total resection and was started on thyroxine, hydrocortisone, desmopressin and later growth hormone. Four months post-surgery, she received PBT. Immediately following PBT she became anorexic with fall in BMI of 4 SD over 8 weeks. (Table 1)
Case 2: A suprasellar tumor was identified on routine MRI at diagnosis of growth hormone deficiency in a 4 year old male. Four months following partial resection (small layer of residual tumour at pituitary stalk and base of hypothalamus) MRI revealed growth of a moderate-size cyst above residual lesion. The Child developed MPHD post second surgery requiring thyroxine, hydrocortisone and growth hormone. Eleven months post second surgery (cyst fenestration and insertion of ommaya reservoir) he received PBT which was followed by abrupt onset of anorexia (BMI ≃ 2SD) requiring supported nutrition by gastrostomy. Following sustained weight gain and recovery of appetite 1 year post PBT, gastrostomy was removed.
|Related procedure||Case 1||Case 2|
|Weight in Kg (SD)||BMI (SD)||Weight in Kg (SD)||BMI (SD)|
|Before 1st Surgery||45.7 (0.64)||23.0 (1.58)||16.6 (0)||19.7 (2.93)|
|Before 2nd Surgery||47 (0.55 )||22.8 (1.47)||16.2 (-0.48)||17.8 (1.76)|
|Before PBT||40.6 (-0.27)||19.7 (0.52)||14.6 (-1.72)||14.6 (-0.53)|
|On admission (after ≃ 8 weeks of PBT)||26.6 (-2.11)||13.0(-3.47)||13.4 (-2.56)||13.2 (-1.83)|
|After 1 Year||37.5 (-1.5)||18.0(-0.61)||21.1 (0.03)||17.6 (1.44)|
|After 2 Year||58.6 (0.45)||27.3(1.89)|
|After 3 Year||63.9 (0.82)||26.9(1.66)|
|Recent weight||76.9 (2.07)||30.2 (2.11)||26 (0.94 SD)||18.7(1.80)|
Conclusion: Although PBT is generally well-tolerated for the treatment for craniopharyngioma; long-term follow up and larger cohort studies are necessary to establish whether dosimetric advantages of PBT translates to clinical benefits in improving long-term toxicities.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology