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Pineal Parenchymal Tumor of Intermediate Differentiation: Clinicopathological Report and Analysis of Epidermal Growth Factor Receptor Variant III Expression

Li, Gordon MD*; Mitra, Siddhartha PhD*; Karamchandani, Jason MD; Edwards, Michael S.B. MD; Wong, Albert J. MD

doi: 10.1227/01.NEU.0000367726.49003.F1
Clinicopathological Study

OBJECTIVE: Epidermal growth factor receptor (EGF) receptor gene amplification is commonly seen in cancer and is the target of many therapies. EGF receptor variant III (EGFRvIII) is the most common variant of the EGF receptor and has been detected in a large percentage of patients with glioblastoma multiforme but not in normal brain. Therapies targeting EGFRvIII are currently being investigated in clinical and preclinical trials.

METHODS: A 14-year-old girl who presented with headaches was found to have a pineal parenchymal tumor of intermediate differentiation. We review the histopathological properties that led to the diagnosis of this tumor. EGF receptor gene amplification and EGFRvIII expression have not been analyzed in pineal tumors. We investigated EGF receptor gene status and EGFRvIII expression in this patient's tumor.

RESULTS: Tumor tissue was obtained and analyzed with flow cytometry, reverse-transcriptase polymerase chain reaction, and Western blot analysis. EGFRvIII was detected by all 3 methods. The tumor was further analyzed by fluorescence in situ hybridization, which did not reveal EGF receptor gene amplification.

CONCLUSION: This is the first report of EGFRvIII expression in a pineal tumor. It is interesting that this variant is detected in the absence of EGF receptor gene amplification. A larger study evaluating the presence of EGFRvIII in pineal tumors is needed.

Department of Neurosurgery, Stanford University School of Medicine, Stanford, California. (Li) (Mitra) (Edwards)

Department of Neuropathology, Stanford University School of Medicine, Stanford, California. (Karamchandani)

Division of Pediatric Neurosurgery Center for Children's Brain Tumor, Lucile Packard Children's Hospital, Stanford, California. (Edwards)

The Cancer Biology Program, Stanford University School of Medicine, Stanford, California. (Wong)

Reprint requests: Gordon Li, MD, Department of Neurosurgery, Stanford University Hospital, 300 Pasteur Drive, Edwards Bldg, Room 200, Stanford, CA 94305. E-mail:

*These authors contributed equally to this work.

Received, May 18, 2009.

Accepted, November 22, 2009.

Copyright © by the Congress of Neurological Surgeons