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BACKGROUND: Atypical Teratoid Rhabdoid Tumor (AT/RT) is a malignant embryonal tumor of the central nervous system (CNS) that presents most often in patients 3 to 6 years of age. Most pediatric oncology centers have employed a multimodal approach to treatment though no standard of care exists. Phoenix Children’s Hospital (PCH) has provided treatment following “Boston Protocol.” This 53-week protocol includes surgical resection and intensive mutiagent chemotherapy followed by radiation, if indicated. This retrospective chart review analyzed historical data of patient demographics, treatment and outcomes for children undergoing therapy per the Boston protocol at PCH. METHODS: Data collected included age, sex, date of diagnosis, race and molecular classifications. We analyzed location of tumor, extent of surgical resection, and treatment modalities utilized. We evaluated the toxicity profiles, as well as intervals of progression free and overall survival. RESULTS: Our cohort of 11 patients had a median age of 21 months (range: 3–54 mos). All of the patients had gross total resection and underwent chemotherapy. In addition, 58% also received radiation therapy. The most prevalent side effect was ototoxicity. Three patients (27%) are deceased and 73% under surveillance with a 2-year progression-free survival of 7.72 months. |
