Non-anaplastic pleomorphic xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination.

CASE REPORT: A case of a non-anaplastic pleomorphic xanthoastrocytoma (PXA) presenting with leptomeningeal dissemination (LMD) affecting a 9-year-old girl is presented.

DISCUSSION: The neoplasia in this young girl had the otherwise classical clinical features of PXA: the relatively advanced paediatric age of the patient, the seizures as presenting sign; the primary site in the temporal lobe; and the MRI findings of the partially solid and cystic superficial lesion. Only the tumour involvement of the chiasma and the infundibulus was a relatively unusual finding. In a 5-year period, the tumour underwent malignant transformation, bringing the child to death because of the primary tumour progression. However, the leptomeningeal deposits remained unchanged throughout the clinical course.

CONCLUSION

To our knowledge, this is the first case of a non-anaplastic PXA presenting with disseminated disease. Thus, it was thought important to describe this case in order to add further information regarding the spectrum of the presenting clinical features of this rare neoplasm and the phenomenon of LMD of non-malignant glioma.