Radiotherapy in paediatric Cushing’s disease: efficacy and long term follow up of pituitary function Shrikrishna V. Acharya • Raju A. Gopal • Joe Goerge • Padma S. Menon • Tushar R. Bandgar • Nalini S. Shah Published online: 22 April 2010 Ó Springer Science+Business Media, LLC 2010 Abstract Pituitary radiotherapy (RT) is an effective second-line treatment for paediatric Cushing’s disease (CD). We report long-term efficacy and anterior pituitary function in a cohort of paediatric CD patients treated with RT. Between 1988 and 2008, from our cohort of 48 pae- diatric CD patients, eight paediatric CD patients (5 males and 3 females) underwent second-line pituitary RT (45 Gy in 25 fractions), following unsuccessful transsphenoidal surgery. Out of eight whose long term follow up is avail- able, four patients were cured by RT. Minimum follow up of 2 years is available for all patients. Four patients achieved cure after RT. Two patients were not cured even after follow up of 60 and 132 months, respectively. Out of four uncured patients, two of them had suppressible low dose dexamethasone cortisol with altered circadian rhythm suggesting possibility of response in near future with fol- low up of just 26 months. Five patients were hypogonadal and one patient was hypothyroid. All patients were below their target height at the time of last follow up. None of the patients had posterior pituitary dysfunction. This series of patients illustrates the efficacy and long-term follow up of pituitary function in children with CD treated with RT. This study also emphasizes the need of growth hormone statues assessment and timely intervention. Keywords Radiotherapy Á Cushing’s disease Á Panhypopituitarism Introduction Cushing’s disease (CD) is characterized by hypersecretion of ACTH from a pituitary corticotroph adenoma. The resulting hypercortisolaemia causes significant morbidity in childhood and adolescence [1, 2]. Though rare in pedi- atric age group, CD is responsible for approximately 75% of pediatric Cushing’s syndrome [1–3]. Transsphenoidal surgery (TSS) with selective microadenomectomy is now recognized as the primary treatment for both pediatric and adult patients with CD. Remission or cure of CD after TSS is reported to vary from 50 to 90% [3–6]. Most published series report a significant percentage of failure, even by the most experi- enced surgeons [5, 6]. As a result, a proportion of children will require another or second-line therapy. There is, however, no clear conformity on optimal therapy after failed TSS. The options are; repeat TSS, pituitary radio- therapy (RT), or bilateral adrenalectomy. Pituitary RT has been established to be effective treatment for adult [7, 8] and pediatric CD [9, 10]. However, there are only few published series of short- and long-term outcome after this treatment in children and adolescents with CD. Storr et al. and L F Chan et al. described 7 patients with CD who were given RT and their post RT follow up [11, 12]. We report our experience with pituitary RT in eight pediatric and adolescent patients with CD after failed TSS. We analysed data with respect to the duration required for cure following RT and long-term follow up of pituitary function. Patients and methods Between 1988 and 2008, 48 paediatric CD patients (29 males and 19 females; mean age, 14.85 years; range, S. V. Acharya (&) Á R. A. Gopal Á J. Goerge Á P. S. Menon Á T. R. Bandgar Á N. S. Shah Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai 12, Maharashtra, India e-mail: drshri25@rediffmail.com 123 Pituitary (2010) 13:293–297 DOI 10.1007/s11102-010-0231-x