Cushing's Syndrome Due to a Non-Adrenal Ectopic Adrenocorticotropin-Secreting Ewing's Sarcoma in a Child

Abstract

Ectopic ACTH syndrome (EAS) is extremely rare in the pediatric age group. Sarcomatous tumors causing EAS are even rarer. We report a 9 year-old girl presenting with Cushing's syndrome caused by ectopic ACTH production by a Ewing's sarcoma. This case illustrates that rapid appearance of Cushingoid symptoms, absence of growth retardation and the presence of hypokalemia are suggestive clues for ectopic ACTH production as the source of Cushing's syndrome.

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

“…The second case reported was a 9-year-old girl with a history of rapid weight gain, acne and acanthosis of neck skin (8). Laboratory findings confirmed the suspicion of CS.…”

“…After ten courses of chemotherapy in 6 months, abdominopelvic MRI revealed a reduction in the tumor and a surgical performed. Cushing's syndrome disappeared after 6 months of treatment (8).…”

“…The biopsy confirmed the Ewing sarcoma diagnosis. The patient received combination chemotherapy with vincristine, adriamycine, cyclophosphamide and radiotherapy (8). After ten courses of chemotherapy in 6 months, abdominopelvic MRI revealed a reduction in the tumor and a surgical performed.…”

“…In pediatric age, the tumors that most frequently cause an EAS are neuroblastomas and neuroendocrine neoplasms, while in adolescents, are carcinoid tumors, both sporadic and in the context of multiple endocrine malignancies (6). Soft tissue sarcomas causing EAS are uncommon (7,8). We report a case of a child with EAS due to metastatic Ewing's sarcoma.…”

Cabergoline therapy of paraneoplastic cushing syndrome in children

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To the Editor: Segers et al [1] recently reported an interesting review of Cushing syndrome in children diagnosed with a renal tumor. Indeed, paraneoplastic Cushing syndromes due to an ectopic secretion of adrenocorticotropin (EAS) are rare, and have been described in other types of pediatric cancers including neuroblastoma [2,3], pancreatoblastoma [4], ovarian steroid cell tumor [5], Ewing sarcomas [6,7], and adrenal ganglioneuroma [8]. Like many other paraneoplastic syndromes, EAS is often difficult to cure, and its treatment depends on the tumor localization and resectability.The treatment usually relies on the resection of the tumor, with or without neoadjuvant chemotherapy, but also can include somatostatin analogs, ketoconazole, chemoembolization, radiofrequency ablation, and radiation therapy [9].

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