1. Long-term follow up of patients with craniopharyngioma
N Glynn, R Sanchez Windt, M Waterhouse, S Akker, WM Drake, MR Druce
Department of Endocrinology, St Bartholomew’s Hospital, London
INTRODUCTION
Craniopharyngioma is a rare, benign tumour of the sellar region which causes local destruction and has a high rate of recurrence.
Patients with craniopharyngioma are characterised by a high incidence of hypopituitarism, visual failure and hypothalamic dysfunction. Furthermore, standardised mortality is markedly elevated.
Management strategies have evolved in recent decades. However, controversy still exists about the optimal treatment approach.
74% (35/47) presented with symptoms from tumour compression – typically visual failure
A minority (6/47) were diagnosed following investigation of an endocrine disorder – pubertal delay or cranial diabetes insipidus.
Median clinical follow-up 30 years (range 1-59)
Figure 2. Patient cohort divided according to BMI (kg/m2)
Long-Term Metabolic Characteristics
Median current body mass index (BMI) was 30.02kg/m2
Majority of patients were obese (Fig. 2)
13% were treated for type 2 diabetes
Median HbA1c 47mmol/mol
23% were treated for hypertension
30% were treated for hyperlipidaemia
AIMS
Describe temporal trends in the treatment of craniopharyngioma at our centre
Examine treatment needs and long-term morbidity in this unique patient group
Figure 1. Surgical route of tumour debulking in each consecutive study quartile (Study period 1951 – 2013)
PATIENTS & METHODS
We performed a retrospective review of all patients with craniopharyngioma currently attending the out-patient clinic at St Bartholomew’s Hospital. Patients were identified from the Department of Endocrinology patient database.
Data recorded include demographic, clinical, biochemical and radiological variables.
Mean and standard deviation were determined for normally distributed, continuous data and median (range) was used for data not normally distributed.
Study period for date of diagnosis ( ) was divided into quartiles and trend analysis performed using Chi square test.
46 patients underwent primary surgical treatment
30 patients (64%) had surgery via the transcranial route.
There was a significant temporal trend towards use of the transsphenoidal approach in the treatment of contemporary cases; p=0.02. (Figure 1)
15% required repeat surgery for tumour recurrence; median time to recurrence was 3 years. Surgical route did not affect recurrence rate.
31 patients received post-operative external beam radiotherapy.
77% had immediate radiotherapy following the first operation while the remainder had radiotherapy following tumour recurrence.
The majority (45/47) had multiple pituitary hormone deficiencies.
81% had cranial diabetes insipidus.
51% (24/47) had permanent visual impairment.
Median dose of hydrocortisone was 20mg/day in divided doses
Median dose of thyroxine was 1.35mcg/kg/day
DISCUSSION
We describe the presentation pattern and morbidity suffered by patients with both childhood and adult-onset craniopharyngioma.
Our study highlights high rates of hypopituitarism and visual loss, both of which are associated with poor quality of life.
We found a significant trend towards more conservative, transsphenoidal surgery in contemporary cases. However, this has not yet led to a reduction in rates of visual loss, hypopituitarism or recurrence.
The high rate of obesity (56%) and metabolic derangement, during long-term follow up, is broadly in keeping with similar case series.1,2
We conclude that patients with craniopharyngioma suffer from high rates of hypopituitarism, visual loss and an adverse metabolic phenotype. Further follow-up is required to determine the impact of contemporary treatment approaches on long-term morbidity.
RESULTS
47 patients (25 male) were identified.
Median age at presentation was 30.5 years (range 2-79)
32% (15/47) had childhood-onset craniopharyngioma (<18 years at time of diagnosis)
REFERENCES
Crowley RK et al Morbidity and mortality in patients with craniopharyngioma after surgery. Clin Endocrinol;73:
Karavitaki et al Craniopharyngioma in children and adults. Clin Endocrinol;62: