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

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.