Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy. (13th October 2019)

• Record Type: Journal Article
• Authors: Refai, Fahd; Al-Maghrabi, Haneen; Al Trabolsi, Hassan; Al-Maghrabi, Jaudah
• Other Names: Tay Stacey Academic Editor.
• Is Part Of: Case reports in pediatrics. Volume 2019(2019)
• Publication Date: 2019-10-13
• Subjects: Pediatrics -- Periodicals; Pediatrics; Pediatrics; Electronic journals; Periodicals; Case Reports; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 618.92
• DOI: 10.1155/2019/3842835
• Languages: English
• ISSNs: 2090-6803

Abstract

Abstract : Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for the characteristic loss of the tumor suppressor protein SMARCB1/INI1 . Here, we describe a case of thoracic spinal ATRT in a three-year-old boy that showed characteristic aggressive progression until managed with intensive multimodal therapy to achieve durable long-term remission. In doing so, we review the histopathological features, management, and current advances in molecular biology that hold promise for personalized ATRT therapy.