Document Detail


Clinical and endocrine responses to pituitary radiotherapy in pediatric Cushing's disease: an effective second-line treatment.
MedLine Citation:
PMID:  12519825     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Transsphenoidal surgery (TSS) is considered first-line treatment for Cushing's disease (CD). Options for treatment of postoperative persisting hypercortisolemia are pituitary radiotherapy (RT), repeat TSS, or bilateral adrenalectomy. From 1983 to 2001, we treated 18 pediatric patients (age, 6.4-17.8 yr) with CD. All underwent TSS, and 11 were cured (postoperative serum cortisol, <50 nM). Seven (39%) had 0900-h serum cortisol of 269-900 nM during the immediate postoperative period (2-20 d), indicating lack of cure. These patients (6 males and 1 female; mean age, 12.8 yr; range, 6.4-17.8 yr; 4 prepubertal; 3 pubertal) received external beam RT to the pituitary gland, using a 6-MV linear accelerator, with a dose of 45 Gy in 25 fractions over 35 d. Until the RT became effective, hypercortisolemia was controlled with ketoconazole (dose, 200-600 mg/d) (n = 4) and metyrapone (750 mg-3 g/d) +/- aminoglutethimide (1 g/d) or o'p'DDD (mitotane, 3 mg/d) (n = 3). All patients were cured after pituitary RT. The mean interval from RT to cure (mean serum cortisol on 5-point day curve, <150 nM) was 0.94 yr (0.25-2.86 yr). Recovery of pituitary-adrenal function (mean cortisol, 150-300 nM) occurred at mean 1.16 yr (0.40-2.86 yr) post RT. At 2 yr post RT, puberty occurred early in one male patient (age, 9.8 yr) but was normal in the others. GH secretion was assessed at 0.6-2.5 yr post RT in all patients: six had GH deficiency (peak on glucagon/insulin provocation, <1.0-17.9 mU/liter) and received human GH replacement. Follow-up of pituitary function 7.6 and 9.5 yr post RT in two patients showed normal gonadotropin secretion and recovery of GH peak to 29.7 and 19.2 mU/liter. The seven patients were followed for mean 6.9 yr (1.4-12.0 yr), with no evidence of recurrence of CD. In conclusion, pituitary RT is an effective and relatively rapid-onset treatment for pediatric CD after failure of TSS. GH deficiency occurred in 86% patients. Long-term follow-up suggests some recovery of GH secretion and preservation of other anterior pituitary function.
Authors:
Helen L Storr; P Nicholas Plowman; Paul V Carroll; Inge François; Gerasimos E Krassas; Farhad Afshar; G Michael Besser; Ashley B Grossman; Martin O Savage
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of clinical endocrinology and metabolism     Volume:  88     ISSN:  0021-972X     ISO Abbreviation:  J. Clin. Endocrinol. Metab.     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2003-01-09     Completed Date:  2003-02-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0375362     Medline TA:  J Clin Endocrinol Metab     Country:  United States    
Other Details:
Languages:  eng     Pagination:  34-7     Citation Subset:  AIM; IM    
Affiliation:
Department of Endocrinology, St. Bartholomew's and The Royal London School of Medicine and Dentistry, London EC1A 7BE, United Kingdom.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adrenal Glands / physiopathology
Child
Cushing Syndrome / blood,  physiopathology*,  radiotherapy*,  surgery
Endocrine Glands / physiopathology*,  secretion
Female
Hormones / secretion
Human Growth Hormone / secretion
Humans
Hydrocortisone / blood
Male
Pituitary Gland / physiopathology,  radiation effects*
Postoperative Care
Remission Induction
Time Factors
Treatment Outcome
Chemical
Reg. No./Substance:
0/Hormones; 12629-01-5/Human Growth Hormone; 50-23-7/Hydrocortisone
Comments/Corrections
Comment In:
J Clin Endocrinol Metab. 2003 Jan;88(1):31-3   [PMID:  12519824 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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