Objective and importance: Pleomorphic xanthoastrocytoma (PXA) is a rare, low-grade astrocytoma of adolescence. Relatively favorable outcomes have been achieved with complete surgical resection. However, few data exist regarding the treatment of recurrent, deep-seated, or multicentric lesions. We report the first case to our knowledge of synchronous multicentric PXA and discuss the related therapeutic challenges.
Clinical presentation: A 13-year-old Hispanic girl presented with a 1-year history of progressive headaches, polyuria, and generalized fatigue. Findings from the neurological examination were notable only for the presence of papilledema. Results of laboratory studies revealed diabetes insipidus and hypothyroidism. The magnetic resonance imaging study revealed numerous nodular, homogeneously enhancing lesions, approximately 1 cm in size, scattered throughout both cerebral hemispheres.
Intervention: A right frontal craniotomy was performed for excisional biopsy of a superficial lesion beneath the coronal suture. Results of the histological examination were consistent with a diagnosis of PXA. The patient was treated with whole-brain radiation of 3600 cGy, with additional intensity-modulated boosts to the enhancing lesions of 1440 cGy. Three years after treatment, the patient remains neurologically nonfocal and shows no evidence of disease progression. Surgical intervention will be considered if accessible lesions progress in size on later imaging studies.
Conclusion: Synchronous multicentric PXA presents unique challenges in that gross total resection would impose significant surgical morbidity; histological homogeneity among the lesions cannot be confirmed; and the well-described potential for anaplastic transformation may be increased with multiple lesions. The optimal treatment for patients with this rare and challenging diagnosis awaits further study.