Nephrogenic adenomas in pediatric patients: a morphologic and immunohistochemical study of 21 cases

Similar to nephrogenic adenomas in adults, those in children are rare benign lesions that often occur in the setting of previous surgery or chronic irritation of the urinary tract. These lesions often present with hematuria and/or as polypoid or papillary lesions on cystoscopy, which may indicate malignancy. We sought to evaluate the various patterns of nephrogenic adenoma occurring in the pediatric population and better characterize the immunophenotype of these lesions. We reviewed 21 cases of nephrogenic adenomas from urinary bladder biopsies of 16 patients. Most patients had a history of urinary bladder augmentation with recurrent urinary stones and urinary tract infections. Many cases presented as a papillary or polypoid mass on cystoscopy. The most common morphologic patterns are papillary, tubulocystic, and a mixed pattern of papillary and tubulocystic, followed by cystic and tubular. On immunostaining, PAX-2, PAX-8, CK7, and MUC-1 provided the most diffuse and intense positive reactivity for nephrogenic adenoma, whereas CD10 and P504S were focal and lesser in intensity when positive. p63 and PAX-5 were consistently negative. We conclude that, although rare in children, nephrogenic adenoma should be included in the differential diagnosis of papillary/polypoid lesions in the urinary tract, especially in the context of previous surgery, chronic irritation from recurrent urinary tract infections, or stones. The immunohistochemistry profile of nephrogenic adenomas in our study also provides evidence that these are derived from distal renal tubular cells. In difficult cases, an immunohistochemical panel consisting of cytokeratin 7, PAX-2, PAX-8, and MUC-1 may be useful.