AUTHOR=Di Ruscio Valentina , Del Baldo Giada , De Pasquale Maria Debora , De Vito Rita , Miele Evelina , Colafati Giovanna Stefania , Deodati Annalisa , De Ioris Maria Antonietta , Tornesello Assunta , Milano Giuseppe Maria , Mastronuzzi Angela 

TITLE=Ectopic ACTH Secretion in a Child With Metastatic Ewing's Sarcoma: A Case Report

JOURNAL=Frontiers in Oncology

VOLUME=10

YEAR=2020

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2020.00574

DOI=10.3389/fonc.2020.00574

ISSN=2234-943X

ABSTRACT=<p>Ectopic ACTH syndrome is rare in pediatric age. Sarcomas that cause Ectopic ACTH Syndrome (EAS) are even more uncommon. Currently, only three cases of EAS caused by Ewing' sarcoma have been reported. We detail a 10-year-old boy with Cushing's syndrome symptoms caused by ectopic ACTH production by a metastatic Ewing's sarcoma of the right ischio-pubic and ileo-pubic branches. The rapid appearance of cushingoid symptoms, with significant weight gain, acne, hirsutism, and hypercortisolism were implications of ectopic ACTH production as paraneoplastic Cushing's Syndrome. The very high levels of ACTH and non-suppression at the high dose dexamethasone test confirmed the clinical suspicion. We underline the possibility EAS was caused by an ACTH-secreting tumor, including soft tissue sarcomas.</p>