Multicentric adrenocorticotropic hormone -producing steroid cell tumor of the fallopian tube &amp; broad ligament in a 15 year old girl

Hamdy, Omar; Saleh, Gehad A.; Eldegwi, Sara A.; Elsayed, Mahmoud; Metwally, Islam H.; Naguib, Reham

doi:10.1080/09513590.2020.1731451

Gynecological Endocrinology

2020

DOI: 10.1080/09513590.2020.1731451

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Multicentric adrenocorticotropic hormone -producing steroid cell tumor of the fallopian tube & broad ligament in a 15 year old girl

Omar Hamdy

Gehad A. Saleh

Sara A. Eldegwi

et al.

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2023

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Paediatric and adolescent ectopic Cushing's syndrome: systematic review

Yami Channaiah,

Karlekar,

Sarathi

et al. 2023

European Journal of Endocrinology

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Objective The data on clinical, biochemical, radiological characteristics and outcomes in pediatric ectopic adrenocorticotropic hormone syndrome (EAS) is limited owing to rarity of the condition. We report three new cases and perform a systematic review of pediatric EAS. Design and Method Case records of pediatric and adolescent EAS patient’s ≤20 years presenting at our center between 1997-2021 were retrospectively reviewed and a systematic review of the literature published between January 1970 to December 2022 was performed. Results A total of 161 patients including three new patients from our center were identified. Bronchial neuroendocrine tumors (NET) (28.5%), thymic NET (22.9%), primitive cell-derived tumors (PCDT, 18.6%), and gastro-entero-pancreatic-NET (13.7%) were the common causes. PCDT were the most common in the first decade (24/45, 53.4%) and were the largest [82 (60-100) mm], whereas bronchial NETs predominated during the second decade (42/116, 36.2%) and were the smallest [15 (10-25) mm]. Computed tomography localized 92.9% (118/127) of pediatric EAS patients. Immediate postoperative remission was attained in 77.9% (88/113) patients whereas 30.4% (24/79) relapsed over a median (IQR) period of 13 (8-36) months. Over a median (IQR) follow-up of 2 (0.6-4.6) years, 31.4% of patients died. The median survival was higher in bronchial NET than in other tumor groups. Distant metastasis and tumor size were independent negative predictors of survival. Conclusions Etiological profile of pediatric and adolescent EAS is distinct from that of adults. Bronchial NETs have the best long-term survival whereas distant metastasis and tumor size predict poor survival.

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Paediatric and adolescent ectopic Cushing's syndrome: systematic review

Yami Channaiah,

Karlekar,

Sarathi

et al. 2023

European Journal of Endocrinology

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Multicentric adrenocorticotropic hormone -producing steroid cell tumor of the fallopian tube & broad ligament in a 15 year old girl

Cited by 1 publication

References 21 publications

Paediatric and adolescent ectopic Cushing's syndrome: systematic review

Paediatric and adolescent ectopic Cushing's syndrome: systematic review

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