Despite recent research on the therapeutic strategies against RS, surgical resection appears the only potentially curative approach. Unfortunately, a mere minority of patients is eligible to undergo surgical intervention. In addition, surgical removal of RS does not necessarily guarantee patient's long-term survival. Laparoscopic approach and enucleation of the tumor have been suggested as well. Alternative therapies, such as radio- and chemotherapy often proved insufficient. The aim of this review was to evaluate the results of surgical treatment for RS with special reference to the extent of its histological spread and to analyze the recent literature in order to provide an update on the current concepts of therapeutic management of this entity.