Abstract Details

Accounting for <1% of astrocytic tumors, pleomorphic xanthoastrocytoma (PXA) has a relatively favorable prognosis with a 5-year survival rate of 80%. Despite its indolent clinical course, PXA has been shown to undergo malignant transformation into secondary anaplastic pleomorphic xanthoastrocytoma (aPXA). Both de novo and secondary aPXAs are characterized by necrosis, five or more mitoses per 10 high-power fields, microvascular proliferation, and high Ki-67 signals. In addition, aPXAs are considerably more aggressive than PXA and have a predicted 5-year survival rate of 57%.

aPXA metastasis is an extremely infrequent consequence with only eleven (7 primary and 4 secondary) reported cases in literature. To the best of our knowledge, only three cases had documented extracranial metastasis, and all were associated with primary aPXA. Herein, we describe the first reported case illustrating the progression of a secondary aPXA with extracranial bone metastasis in a 27-year-old female previously treated for PXA.

To describe the first case of secondary anaplastic pleomorphic xanthoastrocytoma with extracranial metastasis in a patient previously treated for pleomorphic xanthoastrocytoma.  

Although aPXA is a rare diagnosis, recognizing and understanding the tumor can assuredly expand a physician’s diagnostic arsenal and improve patient care. Our patient was treated surgically for PXA at the age of 15 and had no evidence of disease progression for 12 years. However, upon recurrence, her aPXA was extremely aggressive, necessitating multiple resections, radiation therapy, and chemotherapy. Despite the treatments, the tumor progressed rapidly and metastasized to the bones. This case adds to the paucity of literature on secondary aPXA and extracranial metastasis. It also highlights the importance of long-term follow-up for early detection and management of malignant transformation in patients with a history of PXA.