Endocrine Abstracts

Craniopharyngiomas are rare solid or mixed solid-cystic tumours. Although benign histologically, these tumours frequently shorten life and should be considered low-grade malignancies.

We present the case of a 12-year-old boy diagnosed in 2008, at age 5, with a suprasellar tumour of 22/21/20 mm with mixed solid and cystic areas. The tumour was operated twice by left transfrontal approach in 2008 and right transfrontal approach in 2009. The pathology exam revealed an adamantinomatous craniopharyngioma, with a MIB1 of 5%. Postoperatory, the patient developed hypopituitarism: the insulin tolerance test revealed maximum GH: 0.52 ng/ml, maximum cortisol: 0.33 μg/dl, glycaemic nadir: 25 mg/dl, normal prolactinaemia: 15.48 ng/ml, LH: undetectable, FSH: 0.15 mUI/ml, and TSH: 1 mU/l with low T₄. He also developed a polyuro-polydipsic syndrome of 6 l/day–insipidus diabetes. The hypopituitarism was well controlled on levothyroxine 50 μg/day, hydrocortisone 15 mg/day (with dose adjustments), and desmopressin 240 μg/day. Even though the patient associated GH deficiency, this did not prejudice his growth (at 12 years and 5 months he had −0.55 S.D.).

No imagistic recurrence of the tumour was identified until 2014 when he presented with a left frontal tumefaction with progressive expansion which was operated in 2015 – pathology exam: frontal bone tumour of 30/25 mm with solid and cystic parts, IHC: β-catenin positive, Ki-67=10% – ectopic recurrence of the adamantinomatous craniopharyngioma in the left frontal bone, 6 years after the first resection.

Conclusion: We emphasise the importance of preventing iatrogenic tumour implantation. Long-term follow up is mandatory even if the resection is complete and no local recurrence is detected in early check-ups. External radiotherapy should be taken into consideration in such cases.