Currently, the most widely used criteria for assessing response to therapy in high-grade gliomas are based on two-dimensional tumor measurements on computed tomography (CT) or magnetic resonance imaging (MRI), in conjunction with clinical assessment and corticosteroid dose (the Macdonald Criteria). It is increasingly apparent that there are significant limitations to these criteria, which only address the contrast-enhancing component of the tumor. For example, chemoradiotherapy for newly diagnosed glioblastomas results in transient increase in tumor enhancement (pseudoprogression) in 20% to 30% of patients, which is difficult to differentiate from true tumor progression. Antiangiogenic agents produce high radiographic response rates, as defined by a rapid decrease in contrast enhancement on CT/MRI that occurs within days of initiation of treatment and that is partly a result of reduced vascular permeability to contrast agents rather than a true antitumor effect. In addition, a subset of patients treated with antiangiogenic agents develop tumor recurrence characterized by an increase in the nonenhancing component depicted on T2-weighted/fluid-attenuated inversion recovery sequences. The recognition that contrast enhancement is nonspecific and may not always be a true surrogate of tumor response and the need to account for the nonenhancing component of the tumor mandate that new criteria be developed and validated to permit accurate assessment of the efficacy of novel therapies. The Response Assessment in Neuro-Oncology Working Group is an international effort to develop new standardized response criteria for clinical trials in brain tumors. In this proposal, we present the recommendations for updated response criteria for high-grade gliomas.

https://neuroradiologi.dk/onewebmedia/Updated%20RANO.pdf

Updated Response Assessment Criteria for High-Grade Gliomas: Response Assessment in Neuro-Oncology Working Group

We describe the landscape of somatic genomic alterations based on multidimensional and comprehensive characterization of more than 500 glioblastoma tumors (GBMs). We identify several novel mutated genes as well as complex rearrangements of signature receptors, including EGFR and PDGFRA. TERT promoter mutations are shown to correlate with elevated mRNA expression, supporting a role in telomerase reactivation. Correlative analyses confirm that the survival advantage of the proneural subtype is conferred by the G-CIMP phenotype, and MGMT DNA methylation may be a predictive biomarker for treatment response only in classical subtype GBM. Integrative analysis of genomic and proteomic profiles challenges the notion of therapeutic inhibition of a pathway as an alternative to inhibition of the target itself. These data will facilitate the discovery of therapeutic and diagnostic target candidates, the validation of research and clinical observations and the generation of unanticipated hypotheses that can advance our molecular understanding of this lethal cancer.

The somatic genomic landscape of glioblastoma

Objective
To report the autoantigens of a new category of treatment-responsive paraneoplastic encephalitis.

/pdf/paraneoplastic-anti-n-methyl-d-aspartate-receptor-1k25le1vk3.pdf

Paraneoplastic anti–N‐methyl‐D‐aspartate receptor encephalitis associated with ovarian teratoma

Introduction Histological Classification of CNS Tumours Definitions and Explanatory Notes Tumours of Neuroepithelial Tissue Astrocytic Tumours Ependymal Tumours Mixed Gliomas Choroid Plexus Tumours Neuroepithelial Tumours of Uncertain Origin Neuronal and Mixed Neuronal-Glial Tumours Pineal Parenchymal Tumours Embryonal Tumours Tumours of Cranial and Spinal Nerves Schwannoma Neurofibroma Malignant Peripheral Nerve Sheath Tumor Tumours of the Meninges Tumours of Menigothelial Cells Mesenchymal, Non-menigothelial Tumours Primary Melanocytic Lesions Lymphomas and Haemopoietic Neoplasms Germ Cell Tumours Cysts and Tumour-like Lesions Tumours of the Sellar Region Local Extensions from Regional Tumours Metastatic Tumours Unclassified Tumours Illustrations Subject Index Metat Cy Tu

Histological Typing of tumours of the Central Nervous System

Major advances in the management of paraneoplastic neurologic disorders (PND) include the detection of new antineuronal antibodies, the improved characterisation of known syndromes, the discovery of new syndromes, and the use of CT and PET to reveal the associated tumours at an early stage. In addition, the definition of useful clinical criteria has facilitated the early recognition and treatment of these disorders. In this article, we review some classic concepts about PND and recent clinical and immunological developments, focusing on paraneoplastic cerebellar degeneration, opsoclonus-myoclonus, and encephalitides affecting the limbic system.

/pdf/paraneoplastic-syndromes-of-the-cns-3c4xxc3vah.pdf

Paraneoplastic syndromes of the CNS.

In this Second Edition of the bestselling "Diagnostic Imaging: Brain", each 'original' diagnosis has been revised to include the most recent information, updated references, and new image galleries. Moreover, the book features more than 100 new diagnoses. You'll find thousands of new images, all crisply annotated to reinforce the most important points. Richly colored graphics pop off the page, and both typical and variant findings are lavishly illustrated in more than 300 diagnoses. This updated volume will surely become the new standard reference textbook for neuroradiologists, general radiologists, neurologists, and neurosurgeons. A new companion eBook offers the fully searchable expanded text, hundreds of additional images, and extensive linked references.

Diagnostic Imaging: Brain

Metastases to the spine can involve the bone, epidural space, leptomeninges, and spinal cord. The spine is the third most common site for metastatic disease, following the lung and the liver. Approximately 60–70% of patients with systemic cancer will have spinal metastasis. Materials/Methods. This is a review of the imaging techniques and typical imaging appearances of spinal metastatic disease. Conclusions. Awareness of the different manifestations of spinal metastatic disease is essential as the spine is the most common site of osseous metastatic disease. Imaging modalities have complimentary roles in the evaluation of spinal metastatic disease. CT best delineates osseous integrity, while MRI is better at assessing soft tissue involvement. Physiologic properties, particularly in treated disease, can be evaluated with other imaging modalities such as FDG PET and advanced MRI sequences. Imaging plays a fundamental role in not only diagnosis but also treatment planning of spinal metastatic disease.

/pdf/imaging-of-spinal-metastatic-disease-39ostlfhf4.pdf

Imaging of spinal metastatic disease.

Objective:To outline the diagnosis and management of intralabyrinthine schwannomas and to propose a new classification system to further define them.Study Design:Retrospective case review.Setting:Tertiary referral center at a university hospital.Patients:Twenty-eight patients seen at the institution

Intralabyrinthine schwannomas: diagnosis, management, and a new classification system.

BACKGROUND AND PURPOSE: The brain perivascular spaces (PVSs) are pial-lined, interstitial fluid-filled structures that accompany penetrating arteries. When enlarged, they may cause mass effect and can be mistaken for more ominous pathologic processes. The purpose of this study was to delineate the broad clinical and imaging spectrum of this unusual condition. METHODS: Thirty-seven cases of giant PVSs were identified from 1988 to 2004 and were retrospectively reviewed. Clinical data collected included patient demographics, presenting symptoms, and follow-up. Histopathologic data were reviewed when available. Images were evaluated for size and location of the giant PVSs, associated mass effect, hydrocephalus, adjacent white matter changes, and contrast enhancement. RESULTS: There were 24 men and 13 women with an age range of 6–86 years, (mean 46 years). The most common presenting feature was headache (15 patients). Thirty-two cases had multilocular clusters of variably sized cysts. Five lesions were unilocular. All lesions had signal intensity comparable to CSF and did not enhance. The most common location for the giant PVSs was the mesencephalothalamic region (21/36). Fourteen were located in the cerebral white matter; two were in the dentate nuclei. Nine giant mesencephalothalamic PVSs had associated hydrocephalus, which required surgical intervention. CONCLUSION: Giant tumefactive PVSs most often appear as clusters of variably sized cysts that are isointense relative to CSF and do not enhance. They are most common in the mesencephalothalamic region and may cause hydrocephalus. Although they may have striking mass effect, giant PVSs should not be mistaken for neoplasm or other diseases.

http://www.ajnr.org/content/ajnr/26/2/298.full.pdf

Giant Tumefactive Perivascular Spaces

BACKGROUND AND PURPOSE: The imaging appearance of facial nerve schwannomas (FNSs) has been described as an enhancing tubular mass (using T1-enhanced MR) within an enlarged facial nerve canal (using CT). The purpose of this study is to identify how often the FNS imaging findings conform to this description and determine whether there are underlying anatomic explanations for the discrepant imaging appearances identified. MATERIALS AND METHODS: The clinical, pathologic, and radiologic records of 24 FNS in 23 patients were retrospectively reviewed. Each FNS was evaluated for location along the facial nerve. The lesions were cataloged by facial nerve segment with the imaging characteristics of each segment described. RESULTS: The average age at time of first imaging was 39 years (age range, 10–70 years). Eighteen (71%) of the 24 FNSs were pathologically confirmed, while the others were determined intraoperatively or diagnostically by the presence of both enlargement of the facial nerve canal and enhancement on contrast-enhanced T1 MR examination. The most common location was in the geniculate fossa (83%), followed by the labyrinthine and tympanic segments of the facial nerve (both 54%). The most common clinical presentation was facial neuropathy (42%). CONCLUSION: The classic description of FNS on enhanced T1 MR is that of a well-circumscribed fusiform enhancing mass along the course of the intratemporal facial nerve with bone algorithm CT showing sharply defined bony canal enlargement. Modern imaging techniques, however, demonstrate the importance of the surrounding anatomic landscape, leading to various imaging appearances. Lesions traversing the labyrinthine segment can demonstrate a dumbbell appearance. When FNSs track along the greater superficial petrosal nerve, they may present as a round mass projecting up into the middle cranial fossa. FNS of the tympanic segment of the facial nerve preferentially pedunculate into the middle ear cavity, clinically presenting as a middle ear mass. When the mastoid segment of the facial nerve is involved, irregular and “invasive“ tumor margins seen on MR can be explained on CT as tumor breaking into surrounding mastoid air cells.

http://www.ajnr.org/content/ajnr/27/3/694.full.pdf

Karen L. Salzman

Papers

Diagnostic Imaging: Brain

Imaging of spinal metastatic disease.

Intralabyrinthine schwannomas: diagnosis, management, and a new classification system.

Giant Tumefactive Perivascular Spaces

The many faces of facial nerve schwannoma.