Title: Multidisciplinary management and long‑term outcomes in paediatric craniopharyngioma: An 8‑year single‑centre experience

Authors: Dr. Naga Bhagyasri Mangam, Dr. Prasun Deb, Dr. Smitha Nalla, Dr. Sandeep Devireddy, Dr.Manas Panigrahi, Dr. M Sailaja, Dr. I Satish Rao

 DOI: https://dx.doi.org/10.18535/jmscr/v13i08.07

Abstract

 

Background: Craniopharyngiomas are benign epithelial tumors derived from remnants of Rathke’s pouch, typically arising in the sellar and parasellar regions. Despite their benign histology, they demonstrate locally aggressive behaviour, often involving critical neurovascular structures such as the hypothalamus. Management is challenging, and long-term morbidity is common.

Methods: We retrospectively analysed 10 paediatric patients (≤18 years) with histologically confirmed craniopharyngioma treated in the Department of Endocrinology, Krishna Institute of Medical Sciences, over 8 years, focusing on clinical presentation, treatment strategies, and long-term endocrine and metabolic outcomes.

Results: The median age at diagnosis was 12 years (range 3–18 years); 6 patients were boys. Presenting symptoms included headache (n=10), vomiting (n=4), visual impairment (n=2), seizures (n=1), motor weakness (n=1), irregular menstrual cycles (n=1), and absent secondary sexual characteristics (n=1). Pre-operative interventions included ventriculoperitoneal shunt (n=4), endoscopic cyst aspiration (n=1), and Ommaya reservoir placement (n=2). All patients underwent surgical resection, followed by adjuvant radiotherapy (8 conventional, 1 stereotactic, 1 proton beam). Post-treatment hormone replacement therapy was required in most patients, primarily glucocorticoids (n=8) and levothyroxine (n=7). At follow-up, 4 patients were obese, 4 overweight, 1 had impaired glucose tolerance, 1 had diabetes mellitus, 1 had hypertension, and 1 had dyslipidaemia. Seven patients remain progression-free (median follow-up: 8 years); three patients died, two due to recurrence.

Conclusion: Paediatric craniopharyngiomas demonstrate excellent overall survival but high rates of long-term endocrine insufficiency and metabolic syndrome, especially with hypothalamic involvement. Long-term follow-up focusing on cardiovascular risk factor screening and optimal hormone replacement therapy is essential for improving quality of life and outcomes.

Keywords: Craniopharyngioma, paediatric brain tumour, Rathke’s pouch, panhypopituitarism, metabolic syndrome, proton therapy.

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