Progression of atypical extraventricular neurocytoma to anaplastic ganglioglioma.
We report a childhood case of thalamic atypical extraventricular neurocytoma that progressed to highly anaplastic ganglioglioma after 8 years of dormancy after subtotal resection and chemotherapy. The neurocytoma displayed immunoreactivity only for synaptophysin, β-catenin, S100, and CD56. The ganglioglioma acquired strong immunoreactivity for chromogranin, glial fibrillary acidic protein, neuron-specific enolase, and p53 and showed a very high proliferation rate approaching 50% in some areas. Tumor transformation was associated with overexpression of components of the sonic hedgehog and Wnt developmental signaling pathways, which are known to regulate tumor-initiating cells in malignant brain neoplasms.
School of Interdisciplinary Sciences, Faculty of Science, McMaster University, Hamilton, Ontario, Canada L8S-4L8. Electronic address: rusiecdr@mcmaster.ca.
Daniel
D
Rusiecki
Department of Pathology and Molecular Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, Canada L8S-4L8. Electronic address: lach@hhsc.ca.
Boleslaw
B
Lach
McMaster Stem Cell and Cancer Research Institute, McMaster University, Hamilton, Ontario, Canada L8S-4L8; Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada L8S-4L8; Department of Biochemistry and Biomedical Sciences, McMaster University, Hamilton, Ontario, Canada L8S-4L8. Electronic address: branavan.manoranjan@medportal.ca.
Branavan
B
Manoranjan
Department of Pediatrics, McMaster University, Hamilton, Ontario, Canada L8S-4L8.
Adam
A
Fleming
Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada L8S-4L8; Department of Surgery, McMaster University, Hamilton, Ontario, Canada L8S-4L8. Electronic address: ajanio@mcmaster.ca.
Olufemi
O
Ajani
McMaster Stem Cell and Cancer Research Institute, McMaster University, Hamilton, Ontario, Canada L8S-4L8; Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada L8S-4L8; Department of Biochemistry and Biomedical Sciences, McMaster University, Hamilton, Ontario, Canada L8S-4L8; Department of Surgery, McMaster University, Hamilton, Ontario, Canada L8S-4L8. Electronic address: ssingh@mcmaster.ca.
Sheila K
SK
Singh
D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001706:Biopsy; D020295:Brain Stem Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D049109:Cell Proliferation; D002471:Cell Transformation, Neoplastic / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D002648:Child; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D018303:Ganglioglioma / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008279:Magnetic Resonance Imaging; D018365:Neoplasm, Residual; D018306:Neurocytoma / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D013788:Thalamus / Q000737:chemistry / Q000473:pathology*; D013997:Time Factors
D014408:Biomarkers, Tumor
Anaplastic ganglioglioma; Anaplastic ganglioneurocytoma; Brain tumor; Epithelial-mesenchymal transition; Extraventricular neurocytoma; STAT3; p53