Medullomyoblastoma. A histologic, immunohistochemical, and ultrastructural study

The light and electron microscopic features of a medullomyoblastoma arising in the cerebellar vermis of a 3-year-old boy are described. Differentiation along both glial and neuronal lines was present in the medulloblastoma component of the tumor. Astrocytic differentiation was confirmed by the observation of compact bundles of 8 to 10 nm glial filaments in cellular processes on ultrastructural examination, and by positive immunostaining for glial fibrillary acidic protein (GFAP). Neuroblastic differentiation was suggested by the demonstration of axon-like processes on silver impregnation, and ultrastructurally by the observation of microtubules, dense-core and clear vesicles, and rare synapse-like structures within cytoplasmic processes. The presence of both primitive and well-differentiated striated muscle fibers in the tumor was confirmed by the demonstration of thick and thin myofilaments and Z bands on electron microscopy, and by positive immunostaining for myoglobin. These findings clearly establish the presence of both neuroectodermal and rhabdomyoblastic components in this neoplasm, and thus set it apart from the pure rhabdomyosarcomas, which may also occur in the cerebellar vermis in children. This case also illustrates the usefulness of electron microscopy and immunohistochemistry in the diagnosis and histogenetic evaluation of primitive or poorly differentiated small cell tumors of the central nervous system.