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Published on: January 17, 2018
Long-term anterior pituitary function in patients with paediatric Cushing's disease treated with pituitary
L F Chan1, H L Storr, P N Plowman
1Departments of Endocrinology, St Bartholomew's and The Royal London School of Medicine and Dentistry, London, UK.
Insights
Long-term pituitary function in children treated for Cushing's disease with radiotherapy (RT) shows growth hormone deficiency may resolve. Continued surveillance is essential for pediatric patients receiving RT.
Area of Science:
- Pediatric Endocrinology
- Radiation Oncology
- Endocrine Surgery
Background:
- Cushing's disease (CD) in children often requires second-line treatment.
- Pituitary radiotherapy (RT) is an effective option for pediatric CD after surgery.
- Long-term effects of pituitary RT on pediatric endocrine function require further investigation.
Purpose of the Study:
- To evaluate long-term anterior pituitary function in pediatric Cushing's disease patients treated with pituitary RT.
- To assess the sequelae of pituitary RT in a cohort of young patients.
Main Methods:
- Retrospective analysis of 12 pediatric CD patients treated with pituitary RT (45 Gy).
- Patients received RT between 1983-2006 following unsuccessful transsphenoidal surgery.
- Long-term data focused on six male patients with a mean follow-up of 10.5 years post-RT.
Main Results:
- Growth hormone (GH) deficiency was observed in 5/6 patients at 1 year post-RT.
- GH sufficiency was regained in 3/4 patients by a mean of 9.3 years after RT.
- Other anterior pituitary functions and testicular volumes remained normal in the long-term follow-up.
Conclusions:
- Pituitary radiotherapy in pediatric Cushing's disease does not appear to cause serious long-term pituitary deficiencies.
- The study highlights the potential for recovery of endocrine function, particularly GH.
- Emphasizes the critical need for ongoing surveillance of pituitary function in these patients.
Background/Objective:
Pituitary radiotherapy (RT) is an effective second-line treatment for paediatric Cushing's disease (CD). Although the short-term effects of pituitary RT are well documented, there are less data on possible long-term sequelae. We report the long-term anterior pituitary function in a cohort of paediatric CD patients treated with pituitary RT.
Patients And Methods:
Between 1983 and 2006, 12 paediatric CD patients (10 males and 2 females) of mean age 11.4 years at diagnosis (range 6.4-17.4) underwent second-line pituitary RT (45 Gy in 25 fractions), following unsuccessful transsphenoidal surgery. Out of 12, 11 patients were cured by RT (cure interval 0.13-2.86 years) defined by mean serum cortisol of <150 nmol/l on 5-point day curve and midnight sleeping cortisol of <50 nmol/l. Long-term data are available for six male patients, who received RT at the age of 7.0-17.6 years. The mean follow-up from the completion of RT was 10.5 years (6.6-16.5).
Results:
At a mean of 1.0 year (0.11-2.54) following RT, GH deficiency (peak GH <1-17.9 mU/l) was present in five out of six patients. On retesting at a mean of 9.3 years (7.6-11.3) after RT, three out of four patients were GH sufficient (peak GH 19.2-50.4 mU/l). Other anterior pituitary functions including serum prolactin in five out of six patients were normal on follow-up. All the six patients had testicular volumes of 20-25 ml at the age of 14.5-28.5 years.
Conclusion:
This series of patients illustrates the absence of serious long-term pituitary deficiency after RT and emphasises the importance of continued surveillance.
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