The excess mortality in craniopharyngiomas is attributable to their size, site and the traditional surgical approach; aggressive resection predisposes to hypothalamic complications such as obesity, somnolence, thirst disorders and neurocognitive dysfunction. Recently, treatment has been modified to partial resection and radiotherapy. The role of the endocrinologist has expanded from identification and replacement of hormone deficits to include management of hypothalamic disease. Future treatment of craniopharyngioma with neo-adjuvant chemotherapy to minimise surgical resection may improve the outcomes for these patients.
Rachel K Crowley has received an unrestricted educational grant from Novo Nordisk. Christopher J Thompson has no conflicts of interest to disclose.
No funding was received for the publication of this article.
July 06, 2015 Published Online:
August 18, 2015
Rachel K Crowley, St Vincent’s University Hospital, Elm Park, Dublin 4, Ireland. E: R.Crowley@st-vincents.ie
This article is published under the Creative Commons Attribution Noncommercial License, which permits any non-commercial use, distribution,
adaptation and reproduction provided the original author(s) and source are given appropriate credit.
Craniopharyngiomas are large tumours that extend beyond the sella and suprasellar region into the hypothalamus.1 Every case series has indicated a higher mortality in craniopharyngioma than has been reported in pituitary adenoma series or in other cases of hypopituitarism. There is now a better understanding of the factors that contribute to both the morbidity and mortality associated with craniopharyngioma, which has led to a change in clinical practice and an expansion of the role of the endocrinologist.
Traditional Approach to Management of the Patient with Craniopharyngioma
Although the histology is described as benign, craniopharyngiomas form micropapillary extensions into surrounding hypothalamic tissue, which promote a gliotic reaction.2 Surgical resection of these lesions is difficult because of their adherence to surrounding hypothalamic structures. The traditional surgical approach was to attempt gross total resection (GTR), often via a frontal or pterional craniotomy. Aggressive surgery was associated with high rates of panhypopituitarism and diabetes insipidus, morbid obesity, disorders of appetite and thirst, somnolence, poikilothermia and a range of neurocognitive deficits.3–8 Despite attempts at surgical clearance, rates of tumour recurrence were as high as 40 %.3
Traditional methods of management of craniopharyngioma are also associated with high standardised mortality, despite adequate replacement of pituitary hormone deficiencies.3,9,10 Routine management has changed to address some of the morbidities associated with craniopharyngioma.
In the last 10 years there has been a change in surgical approach to a modified transsphenoidal resection in parallel with radiotherapy and radiosurgery. It is not possible to conduct large-scale trials for this rare tumour, but published data indicate that less-aggressive surgery is not complicated by an increase in tumour recurrence rates.11,12 Hypothalamus-sparing surgery in children with craniopharyngioma results in lower rates of obesity than in children who have undergone a traditional surgical approach.11
Analysis of large pituitary disease registers has revealed that patients with craniopharyngioma differ from other hypopituitary patient groups in the rates of hormone deficiency and response to replacement therapy. Data show that they have higher rates of pre-receptor activation of cortisone to cortisol by the enzyme 11βHSD1.13 There may be less benefit from growth hormone (GH) replacement therapy in reduction of visceral adiposity and improved lipid profile.14 Although GH replacement does not appear to reduce the mortality associated with craniopharyngioma, GH therapy is safe for these patients and does not lead to tumour recurrence.15 Craniopharyngioma is also associated with higher rates of diabetes insipidus that may be complicated by adipsia or polydipsia.16 It is unclear whether modification of hormone replacement therapy will reduce the morbidity and mortality associated with craniopharyngioma; however, the recognition of the unique abnormalities seen in craniopharyngioma patients does prompt the need for further research into accurate hormone replacement in this cohort.
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