Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy.
METHODS
A 4-year-old girl had a large midline cerebellar solid and cystic mass partially attached to the meninges. The original diagnosis was glioblastoma multiforme and she was treated by a gross-total surgical resection followed by chemotherapy and radiation therapy to the posterior fossa during the ensuing 14 months. She has received no further therapy and appears to be doing well 12 years later. This unusual favorable clinical outcome prompted our review of this case.


METHODS
Additional special stains and immunocytochemistry were performed on the paraffin embedded tumor sections.


RESULTS
We have confirmed the original histopathological observations of hypercellularity and focal nuclear pleomorphism, atypical mitoses, vascular hyperplasia, as well as focal necrosis. However, the additional stains revealed that the tumor is a relatively well-circumscribed meningeal-based astrocytic tumor (positive for GFAP) with extensive reticulin deposit and focal neuronal differentiation (positive for synaptophysin). A Ki67 labeling index is generally very low, but is positive in up to 5-10% of tumor cells focally. In the light of the favorable clinical outcome and the overall histological features, this tumor may be best reclassified as a rare example of cerebellar pleomorphic xanthoastrocytoma with foci of anaplasia.
Department of Pathology, SUNY Upstate Medical University, 750 E. Adams St., Syracuse, NY 13210, USA. changh@upstate.edu
Howard T
HT
Chang
Julius Gene S
JG
Latorre
Seung
S
Hahn
Ronald
R
Dubowy
Robert L
RL
Schelper
D001932:Brain Neoplasms / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D002531:Cerebellum / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D015972:Gene Expression Regulation, Neoplastic / Q000502:physiology; D005909:Glioblastoma / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging / Q000379:methods; D009419:Nerve Tissue Proteins / Q000378:metabolism
D009419:Nerve Tissue Proteins