Management of Cushing syndrome in children and adolescents: experience of a single tertiary centre

Güemes, María; Murray, Philip; Brain, Caroline; Spoudeas, Helen; Peters, Catherine; Hindmarsh, Peter C.; Dattani, Mehul

doi:10.1007/s00431-016-2727-5

Cited by 34 publications

(30 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There was requirement of postoperative hydrocortisone in our patient as she developed postoperative vomiting, headache and weakness, which was tapered and discontinued over a period of one month. This is similar to reports by other authors [18,38] who were able to discontinue hydrocortisone within 1-2 years. The postoperative treatment of CS is challenging with high prevalence of hypertension, increased BMI and visceral fat mass, impaired cognitive functions and decreased quality of life [18,[39][40][41].…”

Section: Bronchial Carcinoids In Childrensupporting

confidence: 92%

“…When ectopic ACTH secreting lesion is suspected, CT neck, thorax, abdomen and pelvis is done to localise the lesion. Octreotide scan, positron-emission tomography (PET), DOTATE scan and octreotide PET scan can also help in identification of lesion [11,18]. In our patient CT thorax with using 0.5 cm cuts identified lesion of 1.5 cm in apical segment of upper lobe of right lung, which was the source of ectopic ACTH secretion.…”

Section: Discussion 41 Endogenous Cushing's Syndrome (Cs)mentioning

confidence: 65%

“…The overall incidence is 3-5 tumours per million people per year but incidence of bronchial carcinoids in children is not known but they constitute 70-80 percent of all primary malignant lung tumors in children [2,27,29]. Contrast-enhanced CT of the chest, with 5mm thick sections or MRI of neck, chest and abdomen is considered first line for diagnosing ectopic ACTH secreting lesions [18,27,30]. In our patient, the suspicious lesion was detected on CT chest with 5mm sections.…”

Section: Bronchial Carcinoids In Childrenmentioning

confidence: 67%

“…To identify the cases of CD, Corticotrophinreleasing hormone (CRH) test and the HDDST are useful but they are not reliable to differentiate between CD and other causes of ectopic ACTH secretion as patients with ectopic CS may also show a decrease in cortisol level which also happened in our patient [8,13,18]. Bilateral Inferior petrosal sinus sampling (BIPSS) is considered to be the gold standard in distinguishing CD form ectopic ACTH syndrome and for lateralisation of lesions [8,13,18]. In our patient, BIPSS gradients confirmed that there was no lesion in pituitary and there is ectopic secretion of ACTH.…”

Section: Discussion 41 Endogenous Cushing's Syndrome (Cs)mentioning

confidence: 91%

“…CT scan of neck and chest every 6 to 12 months is required in node positive cases [9]. Although the surgical removal of source of hypercostisolaemia is treatment of choice, medical agents such as antihypertensives and inhibitors of steroidogenesis like metyrapone and u n c o r r e c t e d p r o o f ketoconazole can be used in preoperative period to reduce the surgical risk, when surgery is contraindicated and in postoperative period when the patient is not cured by surgical resection [18]. Resolution of hypertension is more common in children as compared to adults due to vascular protective mechanism and shorter lasting hypercortisolemia [37] as is seen in our patient who became normotensive within 3 months after surgery.…”

Section: Bronchial Carcinoids In Childrenmentioning

confidence: 99%

See 4 more Smart Citations

Bronchial Carcinoid Tumour as a Rare Cause of Cushing’s Syndrome in Children: A Case Report and Review of Literature

Saxena

Pathak

Shukla

et al. 2020

Jcrpe

View full text Add to dashboard Cite

What is already known on this topic?The incidence of Endogenous Cushing's syndrome is 0.7-2.4 per million people per year with only 10 percent of cases occur in children and adolescents. Ectopic ACTH syndrome (EAS) accounts for 10 percent of cases of CS in adults but is it is a very rare condition in the paediatric population. Furthermore, the bronchial carcinoid causing Cushing's syndrome is extremely rare with only a few cases reported in literature and very less is known about its response to the treatment. What this study adds?The rarity of disease necessitates the accumulation of data of each case to understand the clinical behaviour and the response achieved by appropriate management and strict follow up. This paper presents the current state of knowledge about Cushing's syndrome due to bronchial carcinoid in paediatric patients and includes the clinical presentation, pathophysiology, approach to diagnose and treat this rare condition and its clinical outcome. The illustration of imaging, pathological slides and clinical photographs also contributes to better understanding of these aspects of disease. AbstractIntroduction-Cushing's syndrome (CS) is rare in childhood and adolescence. The most common cause for CS in children is exogenous administration of glucocorticoids in the form u n c o r r e c t e d p r o o f of topical, inhaled or oral corticosteroids. Endogenous causes can be classified into adrenocorticotropic hormone (ACTH) independent and ACTH dependent causes. Material and methods-The English literature was searched from 2019 to oldest via PubMed, Google and Google scholar using keywords; "Ectopic ACTH Syndrome in children", "Bronchial carcinoid in children" and "Cushing's Syndrome in children. The patients of Bronchial carcinoids causing Ectopic ACTH syndrome (EAS) and reviewed their variables like age, sex, type of carcinoid, investigations, surgery, recurrences and outcome. Results-We found 14 cases of pediatric bronchial carcinoid producing ACTH in literature with mean age of 15.8 years and female preponderance. Most of the patients had right lung lesion and typical carcinoid in histology. We also describe our experience of managing a 12 year old, female, who presented to us with features of Cushing's syndrome and found to have ectopic ACTH secreting bronchial carcinoid, which was resected surgically. Conclusion-Bronchial carcinoid is extremely rare in children and only 4% of them are associated with CS. The postoperative treatment of CS is challenging with high prevalence of hypertension, increased BMI and visceral fat mass, impaired cognitive functions and decreased quality of life. Our patient was managed successfully by multidisciplinary approach and has recovered from hypertension and Cushing's habitus. A careful follow up is indispensable to monitor recurrence of carcinoid and complete remission of CS.

show abstract