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

Oliveira, Ricardo Santos de; Castro, Margaret de; Antonini, Sonir Roberto Rauber; Júnior, Carlos Eduardo Martinelli; Moreira, Ayrton Custódio; Machado, Hélio Rubens

doi:10.1590/s0004-27302010000100004

Cited by 19 publications

(14 citation statements)

References 40 publications

(63 reference statements)

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“…When these occurred they were associated with other anterior and posterior pituitary defects. The percentage of endocrine deficits in our series is higher than reported in other pediatric CD studies [8, 31]. The reason for this is unclear, but may be due to the fact that these patients have more active on-going surveillance.…”

Section: Discussioncontrasting

confidence: 68%

Long-term outcomes of children treated for Cushing’s disease: a single center experience

et al. 2016

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PurposePediatric Cushing’s disease (CD) is rare and there are limited data on the long-term outcomes. We assessed CD recurrence, body composition, pituitary function and psychiatric comorbidity in a cohort of pediatric CD patients.MethodsRetrospective review of 21 CD patients, mean age at diagnosis 12.1 years (5.7–17.8), managed in our center between 1986 and 2010. Mean follow-up from definitive treatment was 10.6 years (2.9–27.2).ResultsFifteen patients were in remission following transsphenoidal surgery (TSS) and 5 were in remission following TSS + external pituitary radiotherapy (RT). One patient underwent bilateral adrenalectomy (BA). CD recurrence occurred in 3 (14.3 %) patients: 2 at 2 and 6 years after TSS and 1 7.6 years post-RT. The BA patient developed Nelson’s syndrome requiring pituitary RT 0.6 years post-surgery. Short-term growth hormone deficiency (GHD) was present in 14 patients (81 % patients tested) (11 following TSS and 3 after RT) and 4 (44 % of tested) had long-term GHD. Gonadotropin deficiency caused impaired pubertal development in 9 patients (43 %), 4 requiring sex steroid replacement post-puberty. Four patients (19 %) had more than one pituitary hormone deficiency, 3 after TSS and 1 post-RT. Five patients (24 %) had long-term psychiatric co-morbidities (cognitive dysfunction or mood disturbance). There were significant long-term improvements in growth, weight and bone density but not complete reversal to normal in all patients.ConclusionsThe long-term consequences of the diagnosis and treatment of CD in children is broadly similar to that seen in adults, with recurrence of CD after successful treatment uncommon but still seen. Pituitary hormone deficiencies occurred in the majority of patients after remission, and assessment and appropriate treatment of GHD is essential. However, while many parameters improve, some children may still have mild but persistent defects.

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Section: Discussioncontrasting

confidence: 68%

Long-term outcomes of children treated for Cushing’s disease: a single center experience

et al. 2016

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“…In our centre, we use the very stringent definition for cure of an undetectable serum cortisol in the immediate post-operative period. The cure rate of 69% in children compares very favourably with other groups (36)(37)(38), and although the strictly defined cure rate is lower in the adult group, the initial remission rate of 78% in adult CD equals or exceeds other published series (1,2). In all ages, the cure rate by TSS increased over time with the introduction of preoperative BSIPSS and as surgical experience and techniques improved.…”

Section: Bsipss (N (%))mentioning

confidence: 78%

Comparisons in the epidemiology, diagnostic features and cure rate by transsphenoidal surgery between paediatric and adult-onset Cushing's disease

Storr¹,

Alexandraki²,

Martin³

et al. 2011

European Journal of Endocrinology

124

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Objective: There are few published comparisons between paediatric and adult-onset Cushing's disease (CD). We compare the epidemiology, diagnostic features and cure rate by transsphenoidal surgery (TSS) in these groups. Design: Retrospective review of patient databases in a single university hospital centre. Patients: Totally, 41 paediatric (mean age 12.3G3.5 years; range 5.7-17.8) and 183 adult (mean age 40G13 years; range 18.0-95.0) patients with CD were investigated. Results: Paediatric CD was characterised by male (63%) and adult CD by a female predominance (79%, P!0.0001). There were small but significant differences in clinical presentation. Biochemical features of CD were comparable except the serum cortisol increase during a CRH test: mean change (105%, nZ39) in paediatric and (54%, nZ123) in adult subjects (P!0.0001). Macroadenomas were more common in adult (15%, 28/183) than in paediatric (2%, 1/41, PZ0.04) CD. Corticotroph microadenomas were more easily visualised by pituitary magnetic resonance imaging (MRI) in adult (76%, 50/66) compared with paediatric (55%, 21/38, PZ0.045) CD with poorer concordance of imaging with surgical findings in children (PZ0.058). The incidence of ACTH lateralisation by bilateral simultaneous inferior petrosal sinus sampling was comparable in paediatric (76%, 25/33) and adult (79%, 46/58; PZ0.95) patients with good surgical concordance in both (82% paediatric and 79% adult). Cure rates by TSS were comparable, with a paediatric cure rate of 69%. Conclusion: Several features of paediatric CD are distinct: increased frequency of prepubertal CD in males, the different clinical presentation, the decreased presence of macroadenomas and the frequent absence of radiological evidence of an adenoma on MRI.

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“…Although these studies confirm a central origin of ACTH excess, lateralization of the adenoma to guide hemi-hypophysectomy is less reliable. 22,28,29 TSS is an established safe and effective procedure in children 30,31 while endoscopic TSS surgery is recently being adopted as outcomes are similar and surgical complications are reduced or equivalent. 31,32 A proportion of paediatric patients who undergo TSS for ACTH adenoma do not achieve postoperative cure and may require second-line therapy such as repeat surgery, radiotherapy, long-term medical therapy to control hypercortisolaemia and rarely bilateral adrenalectomy, 31,33 as observed in our patients.…”

Section: Discussionmentioning

confidence: 99%

Obesity is common at diagnosis of childhood pituitary adenoma and may persist following successful treatment

Sethi

Didi

Dharmaraj

et al. 2020

Clinical Endocrinology

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Objective There is a paucity of data describing long‐term outcomes of paediatric patients with pituitary adenoma. In this report, we describe clinical features, treatment and outcomes of a paediatric cohort. Design Retrospective cohort study. Patients Twenty‐four white Caucasian patients aged <16 years from a single tertiary care centre in the United Kingdom at diagnosis followed for (median, range) 3.3, 0.7‐8.4 years. Measurements Clinical and radiological data at diagnosis and follow‐up. Results Thirteen patients had prolactinomas (54.1%, age: 15.2 years, 13.2‐15.8 years; all females), including ten macroadenomas (11.0‐35.0 mm). Patients presented with menstrual disorders (91%), headache (46%), galactorrhoea (46%) and obesity (body mass index [BMI] SDS > 2): (38%). Ten patients with prolactinoma were treated with dopamine agonist alone, 3 also required surgery and 2 patients, cabergoline, surgery plus radiotherapy. Five patients had Cushing's disease (20.8%, age: 14.0, 4.0‐15.7 years; 2 female), including one macroadenoma (24 mm). Patients presented with obesity (100%), short stature (60%) and headache (40%). Transsphenoidal resection resulted in biochemical cure (09.00 cortisol < 50 nmol/L). Two patients relapsed 3‐ and 6 years following surgery, requiring radiotherapy. One patient also required bilateral adrenalectomy. Six patients had nonfunctioning pituitary adenoma (25.0%, age: 15.8, 12.5‐16.0 years; 2 female), including two macroadenomas (20.0‐53.0 mm). Patients presented with obesity (67%), visual field defects (50%) and headache (50%). Four required surgical resections; two recurred following surgery and required radiotherapy. On latest follow‐up; 13 (54.1%) patients were obese (BMI 3.09 SDS; range: 2.05‐3.73 SDS). Conclusion Obesity is common at diagnosis of pituitary adenoma in childhood and may persist despite successful treatment. Adenomas were larger, more resistant to treatment, and more likely to recur than in adult populations.

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

Cited by 19 publications

References 40 publications

Long-term outcomes of children treated for Cushing’s disease: a single center experience

Long-term outcomes of children treated for Cushing’s disease: a single center experience

Comparisons in the epidemiology, diagnostic features and cure rate by transsphenoidal surgery between paediatric and adult-onset Cushing's disease

Obesity is common at diagnosis of childhood pituitary adenoma and may persist following successful treatment

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