Paraneoplastic Cushing syndrome because of corticotrophin-releasing hormone–secreting Wilms' tumor

doi:10.1016/j.jpedsurg.2008.07.014

Journal of Pediatric Surgery

Volume 43, Issue 11, November 2008, Pages 2099-2101

https://doi.org/10.1016/j.jpedsurg.2008.07.014 Get rights and content

Abstract

A 4-year-old boy with cushingoid appearance and a giant mass on his left kidney was referred to our department. Left nephroureterectomy was performed, and pathologic diagnosis was focal anaplastic nephroblastoma. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, paraneoplastic Cushing syndrome because of Wilms' tumor was confirmed. Moreover, through immunohistochemical staining, we confirmed that the Wilms' tumor secreted corticotropin-releasing hormone and caused Cushing syndrome.

Section snippets

Case report

A 4-year-old boy was admitted with cushingoid characteristics (Fig. 1A) including severe hypertension (130/100 mm Hg), overweight (23 kg), a high plasma cortisol level more than 50 μg/dL (reference range, 5-25 μg/dL), and a high serum ACTH level of 31 pg/mL (reference range, 0-46 pg/mL). Based on normal computed tomographic (CT) scan of the brain, disorders of the hypothalamus and pituitary gland were ruled out. Abdominal CT scan showed the left kidney was occupied by a mass measuring 9.2 × 9.8

Discussion

Paraneoplastic Cushing syndrome is a generic term for a number of physical findings including centripetal obesity, overweight, hypertension, and high levels of plasma cortisol that are caused by nonendocrine tumors. In the pediatric population, a variety of neoplasm, including neuroblastoma [5], Ewing's sarcoma [6], malignant carcinoid tumor of the lungs [7], and thymic carcinoid tumor [8], produce one kind of cortisol, ACTH and CRH, triggering the occurrence of Cushing syndrome. Based on a

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