Endocrine Abstracts

Craniopharyngiomas are rare, sellar/parasellar tumours diagnosed during childhood or adult life.

Despite their benign histological appearance, they are associated with substantial morbidity (endocrine, visual, hypothalamic, as well as neurobehavioural and cognitive) compromising psychosocial integration, quality of life and survival. These complications are the result of the damage by the primary (they tend to be large lesions, most of which with significant suprasellar extensions and often with sharp and irregular margins adhering to vital neurovascular structures), or by the recurrent tumour. They may also be attributed to the adverse effects of the therapeutic interventions (mainly surgery±radiotherapy).

Overall, mortality rates of 3–6 times higher than that of the general population have been reported in subjects with craniopharyngioma. Apart from those deaths directly attributed to the tumour and those related to the surgical interventions, the risk of cardio-/cerebrovascular and respiratory mortality is increased. In childhood populations, hypoadrenalism and the consequences of diabetes insipidus and absent thirst also contribute to the raised mortality. Furthermore, it is widely accepted that tumour recurrence has an unfavourable effect on survival rates.

Although the advances in neuroendocrinology, neuroradiology, microsurgery, supportive care, and radiation oncology techniques have led to improvements in morbidity and mortality rates, the outcome of these patients still remains problematic. Aggressive surgery, particularly in the hypothalamic area, lack of surgical experience/expertise, recurrence(s) and subsequent operations, and radiation-induced late toxicity (particularly harmful for the developing brain) are factors compromising the prognosis in the current clinical practice. Efforts to reduce the impact of these parameters, combined with early identification of patients at risk to develop severe morbidities (especially obesity and adipsic diabetes insipidus) and lifelong management by a multidisciplinary support team are required for optimising the morbidity and mortality associated with these challenging tumours.