Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation

¹ Department of Neurosurgery, University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655 Japan
² Department of Neurosurgery, University of Tokyo Hospital, Tokyo, Japan
³ Department of Pathology, University of Tokyo Hospital, Tokyo, Japan
⁴ Department of Pathology, Massachusetts General Hospital, Boston, MA USA
⁵ Department of Neuropathology, Heinrich-Heine-University, Düsseldorf, Germany
⁶ Department of Radiation Therapy and Oncology, International Medical Center of Japan, Tokyo, Japan

^* To whom correspondence should be addressed. E-mail: kouga-tky{at}umin.ac.jp or toudou-nsu@umin.ac.jp.

	Abstract

Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic neoplasm of the brain. Some PXAs are accompanied by anaplastic features and are difficult to manage because of frequent recurrences that lead to early death. No previous reports have demonstrated consistent efficacy of adjuvant radiotherapy or chemotherapy for this disease. We report a case of PXA with anaplastic features treated with stereotactic irradiation (STI) that resulted in a long-term control of repeatedly recurring nodules throughout the neuraxis. A 47-year-old woman presented with an epileptic seizure due to a large tumor in the right frontal lobe. The tumor was resected and diagnosed as PXA with anaplastic features. Sixteen months later, a relapse at the primary site was noted and treated with stereotactic radiosurgery using gamma knife. Two years later, the patient developed a tumor nodule in the cervical spinal cord that histologically corresponded to a small cell glioma with high cellularity and prominent MIB-1 labeling. In the following months, multiple nodular lesions appeared throughout the central nervous system, and STI was performed 6 times for 8 intracranial lesions using gamma knife and twice, using Linac, for 3 spinal cord lesions in total. All lesions treated with STI were well controlled and the patient was free from symptomatic progression for 50 months. However, diffuse dissemination along the craniospinal axis eventually progressed and she died 66 months after initial diagnosis. Autopsy showed that the nodules remained well-demarcated from the surrounding nervous system tissue. STI may be an effective therapeutic tool for controlling nodular dissemination of PXA with anaplastic features.

Key Words: glioma, pleomorphic xanthoastrocytoma, stereotactic irradiation, stereotactic radiosurgery

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