Malignant teratoid/rhabdoid tumour: long-term survival.

摘要: The patient is a previously healthy 19-month-old child who became symptomatic in October 1998 with right hand weakness and cessation of new words progression sided decreased sensation over the next month. computed tomography (CT) magnetic resonance imaging (MRI) scans demonstrated large, deep, left frontal periventricular enhancing mass several cystic areas. A frontoparietal craniotomy was performed tumour grossly resected. pathological diagnosis AT/RT. Grossly, composed firm, pink-tan hemorrhagic tissue. Sections showed densely cellular, infiltrating neoplasm areas necrosis (some calcified), fascicular focal sheets collagen, variable, often prominent reticulin network. There no endothelial proliferation. Light microscopy hematoxylin eosin stain (Figure 1) revealed varied appearance predominantly larger cells, randomly dispersed nests small undifferentiated cells close to interface normal brain had growth patterns: some were loosely arranged bi- or multi-polar forming cords acinar structures against faintly basophilic mucinous background, elongated coarse follicles other compact bundles, while others prominent, sometimes glassy eosinophilic cytoplasmic inclusions eccentric nuclei (rhabdoid cells). Larger vesicular chromatin nucleoli, smaller hyperchromatic nuclei. Mitoses focally numerous atypical form, glycogen inconspicuous. Immunohistochemical studies widespread staining for epithelial membrane antigen (EMA), vimentin 2), glial fibrillary acid protein (GFAP), groups membranous patterns CD99, regional neuron-specific enolase (NSE), scattered transthyretin, rare actin, cytokeratin, synaptophysin. positive chromosome 22 monosomy. Electron polygonal ill-defined acini that formed inconspicuous, entwined short microvillus processes. Variable numbers intermediate filaments identified rudimentary basal lamina. Desmosomes not seen, there evidence neural differentiation.