Pediatric Pleomorphic Xanthoastrocytoma Treated With Surgical Resection Alone: Clinicopathologic Features.
Pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor that usually occurs in children and young adults. It has characteristic histologic features and is regarded as a WHO grade II lesion. Overall survival is reported to be >60%, but published series usually consist of a range of ages and treatment modalities. Gross total resection is associated with superior survival but recurrence rates after gross total resection are not well described, particularly in a pediatric population. We describe 16 cases over 20 years at our institution of pediatric PXA treated with surgical resection alone with a 5-year relapse-free survival of 40% (95% confidence interval, 20%-82%) and overall survival of 76% (95% confidence interval, 55%-100%). Gross total resection was associated with superior relapse-free survival (P<0.05). Some cases have a very long period between symptom onset or radiologic detection and resection, but neither length of symptoms nor radiologic signs of slow growth were associated with survival. PXA is a rare and unusual entity with unpredictable behavior. Complete surgical resection is optimal but does not guarantee relapse-free survival. We propose separation of PXA from other low-grade gliomas in childhood given differing biology and behavior.
*Children's Cancer Centre §Department of Pathology, Royal Children's Hospital †Department of Paediatrics, University of Melbourne ‡Murdoch Children's Research Institute, Melbourne, Vic., Australia.
Andrew J
AJ
Dodgshun
Alexandra
A
Sexton-Oates
Richard
R
Saffery
Duncan
D
MacGregor
Michael J
MJ
Sullivan
D000293:Adolescent; D001254:Astrocytoma / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D001932:Brain Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male