Open Access

RESIDENT’S CORNER

Ectopic ACTH-secreting neuroendocrine tumor: a rare etiology of a pediatric solid renal mass

Neil J. Kocher¹, Justin Loloi², Joshua Warrick³, Amy S. Burns¹, Ross M. Decter¹

1 Department of Surgery, Division of Urology, The Pennsylvania State University, College of Medicine, Hershey, Pennsylvania, USA
2 The Pennsylvania State University, College of Medicine, Hershey, Pennsylvania, USA
3 Department of Pathology, The Pennsylvania State University, College of Medicine, Hershey, Pennsylvania, USA
Address correspondence to Dr. Ross M. Decter, The Pennsylvania State University College of Medicine, Department of Surgery, Division of Urology, 500 University Drive, Hershey, PA 17033 USA

Canadian Journal of Urology 2019, 26(5), 9956-9959.

Abstract

Neuroendocrine tumors (NETs) are rare tumors with varying clinical presentations. We describe the case of an 11-year-old female presenting with Cushingoid features in the setting of a left-sided flank mass. Her presentation and evaluation suggested a paraneoplastic ectopic ACTH syndrome. She underwent open left radical nephrectomy and final pathology confirming a high-grade NET with nodal metastasis. Although exceedingly rare, ACTH-secreting tumors of the kidney can cause significant morbidity and mortality and so we recommend it be included in the differential diagnosis of pediatric renal masses.

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