Multinodular and vacuolating neuronal tumor of the cerebrum is a recently reported benign, mixed glial neuronal lesion that is included in the 2016 updated World Health Organization classification of brain neoplasms as a unique cytoarchitectural pattern of gangliocytoma. We report 33 cases of presumed multinodular and vacuolating neuronal tumor of the cerebrum that exhibit a remarkably similar pattern of imaging findings consisting of a subcortical cluster of nodular lesions located on the inner surface of an otherwise normal-appearing cortex, principally within the deep cortical ribbon and superficial subcortical white matter, which is hyperintense on FLAIR. Only 4 of our cases are biopsy-proven because most were asymptomatic and incidentally discovered. The remaining were followed for a minimum of 24 months (mean, 3 years) without interval change. We demonstrate that these are benign, nonaggressive lesions that do not require biopsy in asymptomatic patients and behave more like a malformative process than a true neoplasm.
The Response Assessment in Neuro-Oncology criteria were developed as an objective tool for radiologic assessment of treatment response in high-grade gliomas. Imaging plays a critical role in the management of the patient with glioma, from initial diagnosis to posttreatment follow-up, which can be particularly challenging for radiologists. Interpreting findings after surgery, radiation, and chemotherapy requires profound knowledge about the tumor biology, as well as the peculiar changes expected to ensue as a consequence of each treatment technique. In this article, we discuss the imaging findings associated with tumor progression, tumor response, pseudoprogression, and pseudoresponse according to the Response Assessment in Neuro-Oncology criteria for high-grade and lower-grade gliomas. We describe relevant practical issues when evaluating patients with glioma, such as the need for imaging in the first 48 hours, the radiation therapy planning and isodose curves, the significance of T2/FLAIR hyperintense lesions, the impact of the timing for the evaluation after radiation therapy, and the definition of progressive disease on the histologic specimen. We also illustrate the correlation among the findings on conventional MR imaging with advanced techniques, such as perfusion, diffusion-weighted imaging, spectroscopy, and amino acid PET. Because many of the new lesions represent a mixture of tumor cells and tissue with radiation injury, the radiologist aims to identify the predominant component of the lesion and categorize the findings according to Response Assessment in Neuro-Oncology criteria so that the patient can receive the best treatment. ABBREVIATIONS: DCE 4 dynamic contrast-enhanced; GBM 4 glioblastoma; RANO 4 Response Assessment in Neuro-Oncology; rCBV 4 relative CBV; VEGF 4 vascular endothelial growth factor G lial tumors are the most common intra-axial primary tumors of the CNS, with an age-adjusted estimated incidence varying from 0.8 to 5.5 per 100,000, according to the reporting country/organization. 1,2 The most common and aggressive histologic type is glioblastoma (GBM), which accounts for about 60% of the cases. 1 Despite decades of substantial advances in diagnostic radiology, surgery, radiation therapy, chemotherapy, and clinical management of oncology patients, the fatality rate for gliomas remains relentlessly high, especially for GBM. A recent study reported a 1-year survival rate after diagnosis of 41.4% and a 5-year survival rate of only
Toxic and metabolic brain disorders are relatively uncommon diseases that affect the central nervous system, but they are important to recognize as they can lead to catastrophic outcomes if not rapidly and properly managed. Imaging plays a key role in determining the most probable diagnosis, pointing to the next steps of investigation, and providing prognostic information. The majority of cases demonstrate bilateral and symmetric involvement of structures at imaging, affecting the deep gray nuclei, cortical gray matter, and/or periventricular white matter, and some cases show specific imaging manifestations. When an appropriate clinical situation suggests exogenous or endogenous toxic effects, the associated imaging pattern usually indicates a restricted group of diagnostic possibilities. Nonetheless, toxic and metabolic brain disorders in the literature are usually approached in the literature by starting with common causal agents and then reaching imaging abnormalities, frequently mixing many different possible manifestations. Conversely, this article proposes a systematic approach to address this group of diseases based on the most important imaging patterns encountered in clinical practice. Each pattern is suggestive of a most likely differential diagnosis, which more closely resembles real-world scenarios faced by radiologists. Basic pathophysiologic concepts regarding cerebral edemas and their relation to imaging are introduced-an important topic for overall understanding. The most important imaging patterns are presented, and the main differential diagnosis for each pattern is discussed.
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