A fatal collecting duct carcinoma, presenting with pleural metastases,
arose from the right kidney in an 8-year-old child. A distal nephron
origin of the tumor is supported by positive tumor staining with Ulex
europaeus and Arachis hypogaea, and a lack of staining with Tetragonol
obus lotus. The ultrastructural features of short stubby microvilli, s
mooth basal cell membranes, and lateral membrane infoldings also suppo
rt a distal nephron origin (inner most inner medullary collecting duct
). This rare childhood renal neoplasm behaved similarly to that report
ed in adults with metastatic disease at presentation and a short fatal
clinical course.