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Ectopic ACTH-secreting neuroendocrine tumor: a rare etiology of a pediatric solid renal mass
Department of Surgery, Division of Urology, The Pennsylvania State University, College of Medicine, Hershey, Pennsylvania, USA
Oct  2019 (Vol.  26, Issue  5, Pages( 9956 - 9959)
PMID: 31629447


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  • 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.