Recurrent Pleomorphic Xanthoastrocytoma Presenting With Diffuse Leptomeningeal Spread

Abstract

We report a case highlighting key clinical, CSF, and imaging findings of recurrent pleomorphic xanthoastrocytoma with leptomeningeal spread.

A 60-year-old woman presented to the hospital with subacute, progressive aphasia, paraparesis, and urinary retention. Her history was notable for left temporal pleomorphic xanthoastrocytoma (PXA) treated with gross total resection 17 years prior. The tumor recurred 10 months before her current presentation and was re-resected, revealing anaplastic transformation (WHO grade 3, BRAF V600E mutation positive, and homozygous CDKN2A/B deletion). She was treated with targeted photon radiotherapy and temozolomide for 8 months before developing her current symptomatology. CSF analysis revealed total nucleated cell count of 10/μL (monocyte predominant), protein >1250 mg/dL, glucose <20 mg/dL, xanthochromia, and malignant cells consistent with PXA. MRI showed local recurrence with new diffuse leptomeningeal involvement of the spine and nerve roots (Figure 1). At this point in time, the patient and her family elected to pursue a comfort-based approach to her care moving forward and subsequently discharged home with home hospice.

Figure 1.

T1-post gadolinium MRI brain (axial (A), sagittal (B)) and lumbar spine (sagittal (C), axial (D)) revealing enhancement in the inferior margin of the left cerebellum ((A), arrow), anterior margin of the left temporal lobe ((B), arrow), and diffuse zuckerguss leptomeningeal enhancement throughout the spine and cauda equina ((C, D), arrows).

Although PXA is often associated with a favorable prognosis, recurrence with anaplastic transformation has been associated with leptomeningeal dissemination and worse outcomes.^1,2 Among gliomas, both delayed recurrence and leptomeningeal spread of disease are phenomena to be aware of, with recent estimates for the incidence of leptomeningeal metastases to be as high as 4.7% among patients with glioma. ³ This case highlights key clinical, CSF, and imaging findings of recurrent PXA with leptomeningeal spread.