Acta Neuropathologica

, Volume 82, Issue 5, pp 408–413

Cerebellar medullomyoblastoma with advanced neuronal differentiation and hamartomatous component

  • T. Höll
  • P. Kleihues
  • M. G. Yasargil
  • O. D. Wiestler
Case Reports

DOI: 10.1007/BF00296553

Cite this article as:
Höll, T., Kleihues, P., Yasargil, M.G. et al. Acta Neuropathol (1991) 82: 408. doi:10.1007/BF00296553

Summary

This report describes an unusual medullo-myoblastoma which developed in the cerebellar vermis of a 6-year-old girl. Histological investigation showed a highly cellular and predominantly undifferentiated tumor. Myogenic differentiation was prominent in clusters of large tumor cells with eosinophilic cytoplasm and immunoreactivity for desmin and myoglobin. Electron microscopy revealed the presence of immature Z-bands. Immunohistochemically, numerous cells showed incipient expression of myoblastic marker antigens, supporting the view that medulloblastomas and related primitive neuroectodermal tumors possess the potential for non-neural differentiation. In addition, there was evidence of advanced neuronal differentiation, with expression of neuron-specific enolase, synaptophysin, retinal S-antigen, and the formation of ganglioid tumor cells. Occassional neoplastic cells expressed glial fibrillary acidic protein without morphologically detectable astrocytic differentiation. Associated with the neoplasm was brain tissue containing clusters of neuronal cells and focal accumulations of immature oligodendroglia-like cells which expressed neuronal marker antigens. This unusual component resembled a hamartomatous lesion and would support the hypothesis that the cerebellar medullomyoblastoma originated from a teratomatous or malformative lesion. Alternatively, this component may constitute the end stage of advanced neuronal differentiation of a primitive neuroectodermal tumor.

Key words

Medullomyoblastoma Neuronal differentiation Hamartoma 

Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • T. Höll
    • 1
  • P. Kleihues
    • 1
  • M. G. Yasargil
    • 2
  • O. D. Wiestler
    • 1
  1. 1.Laboratory of Neuropathology, Institute of PathologyUniversity of ZürichZürichSwitzeland
  2. 2.Department of Neurosurgery, University HospitalUniversity of ZürichZürichSwitzerland

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