A 7-year-old boy with midline cerebellar mass

Brain Pathol. 2005 Jul;15(3):261-2, 267. doi: 10.1111/j.1750-3639.2005.tb00529.x.


January 2005. A boy aged 7 years was found to have a tumor arising from the roof of the fourth ventricle. Histopathologically, part of the tumor appeared as a PNET, while neuroglial tissue, striated and smooth muscle, cartilage and small glandular structures were present in other regions. Tumor cells in both primitive and mature elements showed a loss of chromosome 17p accompanied by a gain of 17q, a pattern consistent with the presence of an isochromosome 17q. This abnormality is not characteristic of intracranial germ cell tumors, but is present in over 30% of medulloblastomas. On the basis of the histologic and genetic abnormalities, we propose a diagnosis of PNET with multilineal differentiation.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Cerebellar Neoplasms / genetics
  • Cerebellar Neoplasms / metabolism
  • Cerebellar Neoplasms / pathology*
  • Child
  • Chromosomes, Human, Pair 17 / genetics
  • Diagnosis, Differential
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Magnetic Resonance Imaging
  • Male
  • Neuroectodermal Tumors, Primitive / genetics
  • Neuroectodermal Tumors, Primitive / metabolism
  • Neuroectodermal Tumors, Primitive / pathology*