First adult case of sporadic localized glomerulocystic kidney mimicking a tumor

Glomerulocystic kidneys (GCKs) are mainly observed in infants and young children, and are characterized by the cystic dilatation of Bowman's space to form glomerular cysts (GCs). GCKs are associated with various conditions. Additionally, the cystogenesis of GCKs remains controversial. The present study describes a rare adult case of a sporadic localized GCK that radiologically mimicked a multilocular cystic tumor, and analyses the features of GC. A 42-year-old male with hematuria underwent a right partial nephrectomy for a cystic mass. The majority of the cyst was distributed in the cortex and contained a single collapsed glomerulus. Using serial sections, narrow and serpiginous proximal tubules that continued to the GCs were detected. These findings suggested that obliteration at the glomerulotubular junction was not the primary cause of GC in this case. To the best of our knowledge, this is the first adult case of a sporadic localized GCK mimicking a tumor. Unnecessary surgical procedures may be avoided by careful evaluation of computed tomography scans and magnetic resonance imaging, although localized GCKs are quite rare.