Childhood cushing disease: Results of bilateral adrenalectomy

McArthur, Robert G.; Hayles, Alvin B.; Salassa, Robert M.

doi:10.1016/s0022-3476(79)80654-x

Cited by 47 publications

(22 citation statements)

References 20 publications

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“…Although adrenalectomy is the only treatment that offers an immediate control of hypercortisolism with 100% certainty, it is necessary to consider its side effects, including potential adrenal insufficiency crisis. Therefore, lifelong need for glucocorticoid and mineralocorticoid replacement therapy hyperpigmentation, elevated ACTH levels, and an enlarged sella turcica attributable to Nelson's syndrome have been described in 12 to 67% of cases (38)(39)(40)(41). In addition, there is a continued need for glucocorticoid and mineralocorticoid replacement therapy.…”

Section: Discussionmentioning

confidence: 99%

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Oliveira

Castro²,

Antonini³

et al. 2010

Arq Bras Endocrinol Metab

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Objective: The aim of this study was to review the results of surgery for pediatric patients with Cushing's disease who were less than 18 years old and underwent transsphenoidal surgery in a specialized center during a 25-year period. Subjects and methods: Retrospective study, in which the medical records, histology and pituitary imaging of 15 consecutive pediatric patients with Cushing's disease (mean age: 13 years) were evaluated by the same team of endocrinologists and a neurosurgeon from 1982 to 2006. Patients were considered cured when there was clinical adrenal insufficiency and serum cortisol levels were below 1.8 μg/dL or 50 nmol/L after one, two, three, or seven days following surgery; they therefore required cortisone replacement therapy. Follow-up was for a median time of 11.5 years (range: 2 to 25 years). Results: Clinical and biochemical cure was achieved in 9/15 patients (60%) exclusively after transsphenoidal surgery. Hypopituitarism was observed in four patients; growth hormone deficiency, in two; permanent diabetes insipidus, in one case. Conclusions: Cushing's disease is rare in children and adolescents. Transsphenoidal surgery is an effective and safe treatment in most of these patients. Plasma cortisol level < 1.8 μg/dL following surgery is the treatment goal and is a good predictive factor for long-term cure of Cushing's disease. Arq Bras Endocrinol Metab. 2010;54(1):17-23 Keywords Cushing's disease; pituitary tumor; transsphenoidal surgery; pediatric neurosurgery resumo Objetivo: O objetivo deste estudo foi avaliar os resultados cirúrgicos em pacientes pediátricos com doença de Cushing com idade inferior a 18 anos, submetidos à cirurgia transfenoidal num centro especializado, durante um período de acompanhamento de 25 anos. Sujeitos e métodos: Estudo retrospectivo dos prontuários médicos de 15 pacientes pediátricos com doença de Cushing (idade média de 13 anos), sendo avaliados aspectos clínicos, laboratoriais, histológicos e radiológicos. Todos os pacientes foram avaliados pela mesma equipe de endocrinologistas e operados por um mesmo neurocirurgião, entre 1982 e 2006. O tempo médio de seguimento foi 11,5 anos (2 a 25 anos). Os pacientes foram considerados curados quando houve insuficiência adrenal e níveis de cortisol plasmático inferiores a 1,8 μg/dL ou 50 nmol/L no pós-operatório um, dois, três ou sete dias após a cirurgia; estes pacientes necessitaram de reposição de corticosteroide. Resultados: Cura clínica e bioquímica foi alcançada em 9/15 pacientes (60%) após a cirurgia transfenoidal. Hipopituitarismo foi observado em quatro pacientes; déficit de hormônio de crescimento, em dois; diabetes insípido permanente, em um. Conclusões: A doença de Cushing é rara na infância e na adolescência. A cirurgia transfenoidal é um tratamento efetivo e seguro para a maioria dos pacientes. Uma concentração de cortisol plasmático < 1,8 μg/dL nos primeiros dias pós-cirurgia transfenoidal é o objetivo do tratamento e um fator preditivo tardio para a cura da doença de Cushing. Arq Bras Endocrinol...

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Section: Discussionmentioning

confidence: 99%

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Oliveira

Castro²,

Antonini³

et al. 2010

Arq Bras Endocrinol Metab

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“…The alternative treatments, namely bilateral adrenalectomy or repeated TSS, carry significant risks of complications including Nelson's syndrome and permanent pituitary deficiencies respectively (10,16). The possible sequelae of pituitary radiotherapy (RT) include visual impairment, radiation oncogenesis and late hypopituitarism; however, due to advances in the understanding of the radiobiological effects, these first two complications are fortunately very rare (17,18).…”

Section: Introductionmentioning

confidence: 99%

Long-term anterior pituitary function in patients with paediatric Cushing’s disease treated with pituitary radiotherapy

Chan

Storr

Plowman³

et al. 2007

eur j endocrinol

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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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“…This method is possible only if catheterization of these sinuses is done before the operation, and a significant difference in ACTH levels is found between the right and left sinus [3, 11]. Other treatment modalities such as anterior lobe hypophysectomy, bilateral adrenalectomy and X-ray irradiation therapy are unacceptable for children because of their maiming character, making further somatic development and sexual maturation impossible for children [3, 10, 12, 13, 14, 15]. Transsphenoidal surgery, especially the sublabial route, is well tolerated in children and has fewer side effects than other modalities [6, 8, 9, 11, 16].…”

Section: Discussionmentioning

confidence: 99%

Clinical and Therapeutic Properties of Cushing’s Disease in Childhood

Çırak

Palaoğlu²,

Akalan³

1999

Pediatr Neurosurg

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Cushing's disease (CD) in adolescence and childhood is a rare pathology and has some different clinical and therapeutic characteristics than in adults. We report 3 cases treated surgically, with the diagnosis of CD. Their common complaints were short stature and obesity. Endocrinological investigations and radiological evaluation pointed out the diagnosis of CD for all children. They were operated on by the transsphenoidal route, and adenoma resection was performed. Histopathologic and immunohistochemical examination of the tissue specimens confirmed the diagnosis of CD. Long-term follow-up demonstrated weight loss and increase in height.

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Childhood cushing disease: Results of bilateral adrenalectomy

Cited by 47 publications

References 20 publications

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

Long-term anterior pituitary function in patients with paediatric Cushing’s disease treated with pituitary radiotherapy

Clinical and Therapeutic Properties of Cushing’s Disease in Childhood

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