| Abstract: |
We present a case of large cell medulloblastoma with myogenic and melanotic differentiation arising in the cerebellar vermis of a 2-year-old boy and following an aggressive clinical course. Histologic and immunohistochemical features of this tumor include primitive neuronal, rhabdomyoblastic, and pigmented epithelial elements, along with large cell features. Immunohistochemical and molecular data (c-myc gene amplification and the presence of isochromosome 17q) support the contention that this histologically diverse tumor represents a pattern of medulloblastoma with striated muscle and pigmented epithelial differentiation, rather than a teratoma or a cerebellar variant of melanotic neuroectodermal tumor of infancy ('progonoma'). © Springer Science+Business Media, LLC. 2008. |
| Keywords: |
immunohistochemistry; preschool child; child, preschool; disease course; histopathology; case report; postoperative period; cisplatin; drug withdrawal; methotrexate; topotecan; drug megadose; nuclear magnetic resonance imaging; brain tumor; genetic analysis; tumor localization; carboplatin; multiple cycle treatment; gene amplification; melanin; etoposide; vomiting; tumor differentiation; neoplasm proteins; glial fibrillary acidic protein; cyclophosphamide; vincristine; stem cell transplantation; cell differentiation; thiotepa; intensive care unit; antigens, neoplasm; iodine 131; tumor recurrence; medulloblastoma; epithelium cell; seizure; epithelium; keratins; drug substitution; c-myc; oncogene c myc; proto-oncogene proteins c-myc; karyotyping; nerve cell differentiation; cerebellar neoplasms; ataxia; brain surgery; chromosome 17q; skeletal muscle; myogenin; differentiation; meningeal metastasis; neuroectodermal tumors, primitive; cytokeratin ae1; cytokeratin ae3; hospice care; cerebrospinal fluid cytology; muscle development; chromosomes, human, pair 17; molecular; neoplasms, muscle tissue; consciousness disorder; alpha actin; anaplastic; i17q; isochromosome17q; large cell; medullomyoblastoma; melanotic; myogenic; pigmented; large cell medulloblastoma; mydriasis
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