Complete Remission of an Extracranially Disseminated Anaplastic Pleomorphic Xanthoastrocytoma With Everolimus: A Case Report and Literature Review

Background: Surgical resection is the treatment of choice for pleomorphic xanthoastrocytoma, while chemotherapy and radiation therapy are typically used in patients with anaplasia, metastasis, or sometimes in subtotally resected cases, especially upon recurrence. Extracranial dissemination has been only rarely reported. We describe a five year old boy with the rare occurrence multiply recurrent and extracranially disseminated anaplastic pleomorphic xanthoastrocytoma. A complete resolution of his tumor was achieved for greater than two years thus far after administering everolimus.

Methods: We performed a comprehensive literature review of all pleomorphic xanthoastrocytoma cases; 359 cases were described, and 132 of these individuals were less than 18 years of age.

Results: Gross total resection was achieved in only 132 (36.7%) cases, while additional therapy was administered in 186 patients. Only four patients in additon to our own have been documented with extracranial dissemination (four of five in the pediatric population); two patients who succumbed to their disease underwent subtotal resection of the primary tumor.

Conclusions: We report the first patient with extracranially disseminated anaplastic pleomorphic xanthoastrocytoma to be successfully maintained on everolimus as a single oral chemotherapy agent with complete resolution of the tumor. Pleomorphic xanthoastrocytoma can rarely disseminate extracranially in the pediatric population, hence pathologists and neuro-oncologists should be aware of this possibility.