Embryonal sarcoma of adult and pediatric kidneys: report of a case with localized submucosal invasion of the renal pelvis and long-term survival

A case of cystic embryonal sarcoma of the kidney (CESK) with a rapidly fatal outcome was recently reported.1 Here, we report another case of a 12-year-old boy with a localized but ill-defined submucosal lesion of CESK in the right renal pelvis. The tumor consisted principally of small mesenchymal cells with oval to spindle nuclei and scanty cytoplasm, infiltrating in dense arrangements. Two growth patterns were distinguished in the tumor cells: (i) a diffuse infiltrating pattern without an epithelial component; and (ii) a foliated pattern with an epithelial lining over the surface. Foci of the diffuse pattern predominated over those that were lobular, infiltrating superficial layers of renal sinuses and along pyramids, in both of which remarkable intravenous invasion was evident. Foci of the foliated pattern invaded deeper portions of a few sinuses and frequently penetrated into their veins, producing together with their epithelial lining a characteristic foliated structure. Lining epithelial cells around lobular foci often appeared hob-nailed or eosinophilic in the cytoplasm. Despite the remarkable intravenous encroachment, the patient has remained well without a recurrence for more than 26 years after a simple nephrectomy. The present case report expands our understanding of the biological nature of CESK.