Clinical Study
Modern radiotherapy approaches in the management of craniopharyngiomas

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Abstract

An audit of treatment regimens at presentation, recurrence and survival rates spanning 37 years were reviewed for children and adults with craniopharyngioma treated at The Prince of Wales or the Sydney Children’s hospitals. Eligibility criteria stipulated all patients (n = 41) received radiotherapy as part of their treatment course. The primary end point for evaluation was the incidence of radiological progression post-radiotherapy, and secondary end-points were the symptomatic and hormonal status post-radiotherapy. There were 12 paediatric patients (age, <16 years) and 29 adults (age range, 16–80 years). Of the 41 patients, 39 had a suprasellar tumour component, and 38 had radiologically persistent disease post-surgery. Four patients were treated by radiosurgery (median volume treated, 3.5 cm3); four patients progressed post-radiotherapy, of whom two were salvaged by further radiotherapy, one succumbed to progressive local disease, and one had a small intrasellar recurrence surgically resected. Progression-free survival (95.12%) was followed for up to 23 years. Thus, subtotal resection and the modern radiotherapy approach is likely to provide excellent patient and disease outcomes.

Introduction

Craniopharyngiomas are benign intracranial tumours derived from epithelial cells, and thought by most to originate from a remnant of Rathke’s pouch.1 Epidemiologically there are two age peaks: (i) patients aged <16 years, who represent 40% of all patients with craniopharyngiomas (10% of all paediatric intracranial tumours); and (ii) patients aged >16 years (peak period 50–60 years), in whom 60% of craniopharyngiomas occur (1–3% of all adult intracranial tumours).[2], [3] The commonest site of craniopharyngioma presentation is the hypothalamus, which is the origin of 75%: suprasellar and intrasellar locations are found in 21% of patients, and purely intrasellar in only 4% of patients.[4], [5] The site of origin helps explain the clinical features at presentation: hormonal dysfunction and/or mass effect, hydrocephalus, and visual field deficit.[5], [6]

Craniopharyngiomas are typically slow-growing tumours that can often reach a large size before causing the patient to seek medical attention.7 Headache is a common feature, which occurs in 50% of patients with obstructive hydrocephalus. Visual disturbance occurs in 40% to 70% of patients, the most frequent abnormality being a bitemporal hemianopia with papilloedema, present in about 30%.[7], [8] All anterior pituitary hormones can be affected singularly or in combination, with diabetes insipidus present in 10% to 20% of all age groups.[7], [9] Growth failure or delay can occur in up to 40% of the paediatric group.[7], [10], [11]

The cystic and solid components of a craniopharyngioma are shown on MRI in a predominantly suprasellar site,4 but a CT scan may be necessary to define the extent of calcification.12 Endocrine assessment, which is critical if there is a hypoadrenal state and/or diabetes insipidus, is important in planning post-operative management.[7], [8], [9], [11] Neuro-ophthalmological review will reveal the patient’s visual status and enable appropriate post-treatment monitoring.13

The optimal treatment of choice for craniopharyngioma is still keenly debated. Many favour radical resection as this avoids the concerns regarding additional surgery, and the toxicity of radiotherapy.[6], [12], [14], [15], [16] The argument against this is the high risk of morbidity with impaired hypothalamic function and endocrine deficiencies. Others favour subtotal resection and added therapy, which now includes stereotactic radiosurgery (SRS), stereotactic radiotherapy (SRT), intracavity irradiation, or chemotherapy.[7], [8], [17], [18]

A single centre’s experience cannot provide a definitive answer. This review evaluates a mixed paediatric and adult practice over a 37-year period as more sophisticated treatment approaches were introduced.

Section snippets

Materials and methods

To be included in this review, patients had an established diagnosis (both radiological and histological) of craniopharyngioma, and received radiotherapy as a treatment component of either the initial treatment or at the time of recurrence.

Radiotherapy was delivered with 6 Mev photons to all patients. SRS was available from 1990 onwards and SRT after 1995. Intensity modulated radiotherapy (IMRT) was available from 2000 onwards. All stereotactic irradiation was linear accelerator (LinAc) based.

Results

A total of 56 patients were seen during the study duration of 1967 to March 2007, of whom 41 patients were eligible for evaluation. Fifteen “consultation only” patients did not meet the eligibility criterion and thus were excluded from the study.

The presenting details (i.e. those features present prior to any radiotherapy) are listed in Table 1. A greater number of patients were referred at the time of recurrence/progressive disease after their last surgical procedure. Only three patients had

Discussion

The presentation of craniopharyngiomas is usually on a background of mass effect relating to a suprasellar lesion.5 This mass lesion exerts a clinical effect by: obstructing the flow of cerebrospinal fluid (CSF) leading to symptoms of obstructive hydrocephalus; pressing on the optic chiasm/optic pathway causing visual deficit; and interrupting the hypothalamic/pituitary axis resulting in pituitary hormonal deficit.[4], [5] While surgical resection may relieve some of the effect of this mass, it

Conclusion

This study presents no new findings. As with most, it is retrospective, covering many years of experience. With a mixed population of children and adults it would be difficult to define a specific feature differentiating the two populations. The study does confirm, however, many features apparent in other studies. Of importance is that patients who present with recurrent disease are more likely to have multiple overt endocrine deficits that harbinger impaired quality of life. More precisely

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