Role of post-operative radiation therapy in single brain metastasis from clear cell sarcoma in children: a case report with systemic review

Abstract

Brain metastasis is relatively uncommon in children with solid tumors.  Less than 4.5% of children with solid tumors will develop parenchymal intracranial metastasis, whereas brain relapse of clear cell sarcoma of the kidney occurs in about 5% of cases during the follow-up. The prognosis of patient with relapsed clear cell sarcoma of the kidney (CCSK) is generally reserved with a 5-year event-free survival of 18%, and a 5-year overall survival of 26% in one of the largest series. However, several cases of solitary brain metastasis were successfully managed by a multimodality aggressive approach including radiation therapy, with long term survivals reported in the literature data. Because of the relative rarity of this particular entity, information regarding management and prognosis are still missing.  Through the report of a rare case of a young child with isolated cerebellar relapse of CCSK treated by a multimodality approach with a summary literature review, we try to emphasize the role of post-operative radiation therapy in the management of this rare entity.