Clinical Features and Surgical Results of Pediatric Pleomorphic Xanthoastrocytoma: Analysis of 17 Cases with a Literature Review

BACKGROUND: Pediatric pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor. To date, there are few studies dedicated to this kind of pediatric tumor. The aim of this study was to investigate the clinicopathologic characteristics of pediatric PXA. METHODS: We retrospectively analyzed 17 pediatric patients diagnosed with PXA histologically between July 2009 and December 2018. We also reviewed the relevant literature. RESULTS: The majority of pediatric PXAs had cystic components and peritumoral edema, and approximately 40% of the tumors had calcifications. All large tumors (& Dagger;5 cm) were located in the nontemporal lobes except 1 (P = 0.05). Furthermore, the large tumors were primarily solid cystic or cystic with mural nodules radiologically, while tumors measuring <5 cm were mainly solid or solid with cystic changes (P = 0.02). All patients underwent surgery, and 15 patients experienced complete tumor removal. Histologically, 11 patients had grade II PXAs and 6 patients had grade III PXAs. After the operation, most of the patients recovered uneventfully and the seizures were well controlled. The mean follow-up time was 43 months. Five patients received radiotherapy or chemotherapy. One patient had tumor recurrence 5 years after the first operation and underwent repeat surgery. CONCLUSIONS: Cystic components and peritumoral edema could be seen in most pediatric PXAs, and calcification was also not uncommon. The size of the tumor was correlated with the tumor site and radiologic subtype. Maximal safe resection of pediatric PXA is recommended and was shown to be beneficial for seizure control and survival.