Primary XXXX in @entity1 , adolescents, and young adults: single institution experience and review of the literature.

@entity1 @entity675 @entity632 @entity932 @entity511 @entity8958 @entity242 @entity470 @entity5 @entity984 @entity786 @entity2879 @entity876 @entity2467 @entity1891 @entity1260 @entity980 @entity817 @entity1722
UNASSIGNED: There is a paucity of literature reporting the outcome of @entity932 ( @entity932 ) in @entity1 , adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with @entity932 , treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry ( @entity675 and @entity470 ) and DNA sequencing were performed on 5 @entity5 samples. A literature review of @entity932 was also conducted. We reviewed 13 @entity1 (median age, 7  years) with a primary diagnosis of @entity932 between 1990 and 2015. Diagnoses included unclassified @entity242 (n  =  9), @entity511 (n  =  2), and @entity980 (n  =  2). Five @entity1 underwent upfront gross total resection (GTR) of the @entity5 . The 5-drug regimen ( @entity1891 , @entity632 , @entity786 , @entity2879 , and @entity1260 ) was the most common treatment used. Nine @entity1 died due to progression or recurrence (n  =  8) or secondary malignancy (n  =  1). The median follow-up period of the 4 surviving @entity1 was 1.69  years (range 1.44-5.17  years). The 5-year progression-free survival and overall survival rates were 21 and 44  %, respectively. @entity817 , @entity876 , @entity984 , @entity2467 , @entity1722 , MET, @entity8958 , ATM, and @entity470 mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for @entity675 , and only 2 were positive for @entity470 . @entity932 remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate @entity5 biology to improve the disease outcome.
