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Cerebellar medullomyoblastoma with advanced neuronal differentiation and hamartomatous component
Cerebellar medullomyoblastoma with advanced neuronal differentiation and hamartomatous component
- Source :
- Acta Neuropathologica. 82:408-413
- Publication Year :
- 1991
- Publisher :
- Springer Science and Business Media LLC, 1991.
-
Abstract
- 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.
- Subjects :
- Pathology
medicine.medical_specialty
Cerebellum
Hamartoma
Enolase
Pathology and Forensic Medicine
Lesion
Neoplasms, Muscle Tissue
Cellular and Molecular Neuroscience
medicine
Humans
Child
Neurons
Glial fibrillary acidic protein
biology
Cell Differentiation
medicine.disease
Immunohistochemistry
medicine.anatomical_structure
nervous system
Primitive neuroectodermal tumor
biology.protein
Cerebellar vermis
Synaptophysin
Female
Desmin
Neurology (clinical)
medicine.symptom
Medulloblastoma
Subjects
Details
- ISSN :
- 14320533 and 00016322
- Volume :
- 82
- Database :
- OpenAIRE
- Journal :
- Acta Neuropathologica
- Accession number :
- edsair.doi.dedup.....bf08f887edbe1244b60c90d3cbb55c18
- Full Text :
- https://doi.org/10.1007/bf00296553