Cerebellar medullomyoblastoma with advanced neuronal differentiation and hamartomatous component
| Autor: | Paul Kleihues, T. Höll, M. G. Yasargil, O. D. Wiestler |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
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 |
| Zdroj: | Acta Neuropathologica. 82:408-413 |
| ISSN: | 1432-0533 0001-6322 |
| DOI: | 10.1007/bf00296553 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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