Rapidly Fatal Ectopic Adrenocorticotropic Hormone Syndrome in a 9-Year-Old Girl With Ewing Sarcoma

Ewing sarcoma (ES) with ectopic adrenocorticotropic hormone (ACTH) syndrome (ectopic ACTH) is extremely unusual. This report details the first case in English literature of pediatric ES involving the proximal aspect of the humerus with florid ectopic ACTH. A 9-year-old girl presented with mooning of...

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Bibliographic Details
Published in:AACE clinical case reports 2022-03, Vol.8 (2), p.69-72
Main Authors: Varghese, Johann, Ayyagari, Mythili, Reddy, Sagar S.L., Mandapati, Navya Sruthi, Subrahmanyam, K.A.V.
Format: Article
Language:English
Subjects:
Case Report
ectopic ACTH
Ewing sarcoma
pediatric
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Summary:Ewing sarcoma (ES) with ectopic adrenocorticotropic hormone (ACTH) syndrome (ectopic ACTH) is extremely unusual. This report details the first case in English literature of pediatric ES involving the proximal aspect of the humerus with florid ectopic ACTH. A 9-year-old girl presented with mooning of the face and abdominal distension for the past 5 months with an unremarkable history. Her serum cortisol level measured at 8 AM was 42 μg/dL (reference range, 4.3-22.4 μg/dL). Serum ACTH level of 225 pg/mL (reference range, 10-46 pg/mL) suggested ACTH-dependent Cushing syndrome. Her serum cortisol level after the overnight dexamethasone suppression test was 60 μg/dL (reference value,
ISSN:2376-0605
2376-0605
DOI:10.1016/j.aace.2021.09.003