Atypical teratoid rhabdoid tumor (ATRT) – pro
Atypical teratoid rhabdoid tumor (ATRT) is a rare and fast-growing cancerous tumor of the brain and spinal cord. Current advances in therapy have helped older children (who have been reproted to have up to 50-70% survival), and children with tumors that can be completely removed have an improved survival.
In 2008, The Dana-Farber Cancer Institute in Boston reported two-year overall survival of 53% and event-free survival of 70% (median age at diagnosis of 26 months). In 2013, The Medical University of Vienna reported five-year overall survival of 100%, and event-free survival of 89% (median age at diagnosis of 24 months).
Due to the rarity of this tumor and the lack of randomized controlled trials it has been challenging to define optimal therapy and advance treatment.
A number of case reports and series suggest that high dose chemotherapy and stem cell transplantation result in some of the highest survivals, but, due to the rarity of this disease, randomized trials are not possible.
CLINICAL / MEDICAL REFERENCES
Park ES1, Sung KW, Baek HJ, Park KD, Park HJ, Won SC, Lim DH, Kim HS. Tandem high-dose chemotherapy and autologous stem cell transplantation in young children with atypical teratoid/rhabdoid tumor of the central nervous system. J Korean Med Sci. 2012 Feb;27(2):135-40.