Dysembryoplastic neuroepithelial tumors (DNTs) mostly display typical clinical, neuroimaging and histopathological features, but sometimes they reveal heterogeneous or non-specific morphology, which results in diagnostic dilemmas. We present a case of a young adult with longstanding, intractable epilepsy associated with a multinodular cystic lesion in the temporal lobe. The lesion consisted of morphologically different components. In particular, a few cortical nodules displayed a specific glioneuronal element with floating neurons typically found in DNT. Two large, well-circumscribed nodules were entirely composed of biphasic, piloid, astroglial patterns that corresponded strictly to a pilocytic astrocytoma. The well-defined areas, which contained numerous distinct neurocytic-like rosettes, were identical with rosette-forming glioneuronal tumors (RGNTs). This type of neurocytic rosette was widespread within the surrounding piloid background. Some solid nodules exhibited increased cellularity, oligodendroglioma-like elements and a focal ribbon cell arrangement. The lesion was associated with advanced reactive gliosis and foci of dysplastic changes in the adjacent cortex. The clinico-radiological and main histopathological features were consistent with a diagnosis of a complex variant of DNT composed of pilocytic and rosette-forming glioneuronal components. Although both piloid tissue and rosette-like formations have been occasionally mentioned in DNT lesions, the present case of DNT was unique in its well-circumscribed, separate pilocytic and RGNT nodules. We concluded that it represented an unusual, mixed pilocytic/RGNT variant of DNT.
Keywords: DNT components; dysembryoplastic neuroepithelial tumor; neurocytic rosettes; pilocytic astrocytoma; rosette forming glioneuronal tumor.
© 2014 Japanese Society of Neuropathology.