Intracranial Myxoid Mesenchymal Tumor With EWSR1-ATF1 Fusion.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that arises primarily in the extremities of young adults. Recurrent gene fusions involving EWSR1 with members of the cAMP response element binding protein (CREB) family have been reported in a diverse group of tumors, including AFH. AFH-like lesions have been reported to occur intracranially and the reported cases show low proliferation indices, frequently have a connection with the dura, and show recurrent EWSR1 rearrangements. These tumors have been termed intracranial myxoid mesenchymal tumor with EWSR1-CREB family gene fusions. A literature search identified 11 reported cases of intracranial AFH-like lesions with an EWSR1 rearrangement. Here, we report a case of intracranial myxoid mesenchymal tumor with an EWSR1-ATF1 fusion in an adult patient, and review the existing literature on this recently described entity.

FGFR1 tyrosine kinase domain duplication in pilocytic astrocytoma with anaplasia.

We report the case of a 27-yr-old male with visual field loss who had a 4.9-cm complex cystic mass in the right occipital lobe. Histologic examination showed pilocytic astrocytoma (PA) with anaplasia, and molecular characterization revealed FGFR1 duplication with additional variants of unknown significance in several genes (ARID1A, ARID1B, CHEK2, EPHA5, and MLL2). This is one of only a very few reported cases of anaplastic PA with characterization of molecular alterations.

Extending the Neuroanatomic Territory of Diffuse Midline Glioma, K27M Mutant: Pineal Region Origin.

Diffuse midline glioma, H3-K27M mutant (DMG-K27M) is a newly described, molecularly distinct infiltrative glioma that almost exclusively arises in midline CNS structures, including the brain stem, especially the pons, as well as the thalamus and spinal cord with rare examples seen in the cerebellum, third ventricle, and hypothalamus. To our knowledge, only 1 case of a molecularly confirmed DMG-K27M arising in the pineal region has been previously reported. We present the second occurrence of a tissue-confirmed DMG-K27M of the pineal region, which, to our knowledge, is the first case reported in a child and the first case with documented preoperative MRI. This case, in addition to a prior report described in an adult, defines the lower end of a broad age range of DMG-K27M onset (12-65 years) and establishes the pineal gland as a bona fide site of origin for this newly codified midline glioma.

Magnetic resonance imaging features of pilocytic astrocytoma of the brain mimicking high-grade gliomas.

OBJECTIVE: The typical magnetic resonance/computed tomographic imaging appearance of pilocytic astrocytoma (PA) is that of a cyst with an intensely enhancing mural nodule. The purpose of this study was to illustrate the aggressive imaging features of PA. METHODS: One hundred patients referred to the cancer center with brain tumors histologically proven to be PA were retrospectively reviewed (95 by magnetic resonance imaging and 5 by computed tomographic imaging) and analyzed. RESULTS: The patient population includes 76 pediatric patients younger than 18 years and 24 adults ranging from 19 to 45 years old. Tumor locations consisted of the following: optic chiasm (22), lateral ventricle (3), thalamus (12), basal ganglia (1), cerebral hemisphere (10), corpus callosum (2), brain stem (26), fourth ventricle (1), and cerebellum (23). The imaging appearance of PA consisted of typical features in 71 cases and aggressive features in 29 cases. CONCLUSIONS: It is important to recognize the aggressive imaging appearance of PA (grade 1 astrocytoma) because it can be mistaken for high-grade gliomas and may thus lead to inappropriate therapy. Despite the aggressive imaging appearance of PA, there is no histopathologic evidence of anaplasia.

Central nervous system lymphoma characterization by diffusion-weighted imaging and MR spectroscopy.

PURPOSE: The characterization and differentiation of central nervous system (CNS) lymphoma has important diagnostic, therapeutic, and prognostic significance. The purpose of this study is to characterize the diffusion-weighted imaging (DWI) and MR spectroscopic (MRS) findings in CNS lymphoma. MATERIALS AND METHODS: Twenty consecutive patients (male [n= 12], female [n= 8]) with histopathologically proven CNS lymphoma were retrospectively evaluated during this study from July 2005 to April 2007. Patients included immunocompromised (n= 9) and immunocompetent (n= 11) individuals. MR Imaging (pretreatment n= 13), pre- and post-treatment (n= 7) included DWI (n= 20) (b = 1000s/mm2) and ADC (apparent diffusion coefficient) maps of all patients. MRS was performed (n= 10) with PRESS (point-resolved spectroscopy) sequence (multivoxel n= 8, single voxel n= 2) with a TE of 144 msec. All patients were histopathologically confirmed to have lymphoma by biopsy. RESULTS: Areas of restricted diffusion were observed in 90 % (n= 18/20) on pretreatment scans. The diffusion restriction was variable on post-treatment scans. Median metabolite ratios in 10 patients were Cho/Cr- 2.12, NAA/Cho - .49, and NAA/Cr - 1.64. Presence of lactate or lipid was noted in 90 % (n= 9/10). Sites of lesion location were subcortical white matter (n= 6), basal ganglia (n= 4), corpus callosum (n= 3), extra-axial space including cavernous sinus (n= 5), cerebellum (n= 1), and lateral ventricle (n= 1). CONCLUSION: Restricted diffusion is a consistent imaging finding in CNS lymphoma in immunocompetent patients. Spectroscopy is helpful in initial imaging diagnosis and post-treatment surveillance. These lesions are usually paraventricular in location. MR imaging appearances differ among immunocompetent and immunosuppressed individuals in most cases.

Origin of chordoid glioma of the third ventricle.

CONTEXT: Chordoid glioma is a relatively recently described unique glial neoplasm that has been formally codified by the World Health Organization in Pathology and Genetics of Tumours of the Nervous System, in which it is included along with astroblastoma and gliomatosis cerebri under the rubric "Tumors of Uncertain Origin." Many examples of chordoid glioma come to clinical attention only at a relatively large size and occupy a large portion of the third ventricle. Accordingly, the anatomic origin of chordoid glioma has been unclear and debated. OBJECTIVE: To examine the regional anatomic origin of chordoid glioma. DATA SOURCES: The clinical, imaging, histologic, immunophenotypic, and ultrastructural data in previously published case series and individual case reports of chordoid glioma were reviewed in conjunction with the study of a new case of chordoid glioma that presented at a relatively small size, thereby facilitating neuroanatomic localization. CONCLUSIONS: Chordoid glioma exhibits features of specialized ependymal differentiation on ultrastructural examination, and all examples reported in the literature to date have displayed a highly stereotypical suprasellar anatomic localization and an ovoid shape, as seen on neuroimaging studies and gross anatomy. Neuroanatomic, radiologic, and clinical evidence supports an anatomic origin for chordoid glioma from the vicinity of the lamina terminalis.

T2-weighted spine imaging with a fast three-point dixon technique: comparison with chemical shift selective fat suppression.

PURPOSE: To develop a phased-array coil-compatible, fast three-point Dixon (TPD) technique, and compare its performance in T2-weighted spine imaging with that of the standard chemical shift selective (CHESS) fat suppression technique. MATERIALS AND METHODS: We acquired T2-weighted spine images of 27 patients using essentially identical scanning parameters with the fast TPD technique and standard fast spin echo (FSE) with CHESS fat suppression. A phased-array coil-compatible image reconstruction algorithm was developed to generate separate water and fat images from the data acquired with the fast TPD technique. Three neuroradiologists independently scored the images from the two different techniques for uniformity of fat suppression and lesion conspicuity using a four-point system (1 = poor, 2 = fair, 3 = good, 4 = best). RESULTS: The reviewers' mean scores were 3.2 and 2.1 for the uniformity of fat suppression, and 3.0 and 2.0 for the lesion conspicuity for the fast TPD and the CHESS fat suppression techniques, respectively. The fast TPD technique was statistically superior to the CHESS technique at P < 0.0005. CONCLUSION: The fast TPD technique provides superior fat suppression and lesion conspicuity, and potentially can be used as an alternative to T2-weighted imaging of the spine.

Textiloma (gossypiboma) mimicking recurrent intracranial tumor.

CONTEXT: Resorbable substances used to achieve hemostasis during neurosurgical procedures comprise 3 principal classes based on chemical composition: (1) gelatin sponge, (2) oxidized cellulose, and (3) microfibrillar collagen. Nonresorbable hemostatic aides include various forms of cotton and rayon-based hemostats (cottonoids and kites). Resorbable and nonresorbable hemostatic agents have been reported to cause symptomatic mass lesions, most commonly following intra-abdominal surgery. Histologic examination typically shows a core of degenerating hemostatic agent surrounded by an inflammatory reaction. Each agent exhibits distinctive morphologic features that often permit specific identification. Hemostat-associated mass lesions have been variously referred to as textilomas, gossypibomas, gauzomas, or muslinomas. OBJECTIVES: The aims of this study were to (1) identify cases of histologically proven cases of textiloma in neurosurgical operations, (2) characterize the specific hemostatic agent associated with textiloma formation, and (3) characterize the preoperative magnetic resonance imaging appearance of textiloma. DESIGN: Cases in which a textiloma constituted the sole finding on repeat surgery for recurrent brain tumor, or was a clinically significant component of a radiologically identified mass lesion together with residual tumor, constituted the study set. RESULTS: Five textilomas were identified and evaluated. The primary neoplasm was different in each case and included pituitary adenoma, tanycytic ependymoma, anaplastic astrocytoma, gliosarcoma, and oligodendroglioma. In all cases, preoperative magnetic resonance imaging suggested recurrent tumor. Textilomas included all categories of resorbable hemostatic agent. Other foreign bodies were present in some cases; the origin of these foreign bodies was traced to fibers shed from nonresorbable hemostatic material placed temporarily during surgery and removed before closure (cottonoids and kites). Inflammatory reactions included giant cells, granulomas, and fibroblastic proliferation. Microfibrillar collagen (Avitene) textilomas were associated with a striking eosinophil infiltration that was not seen with any other hemostatic agent. CONCLUSIONS: Hemostatic agents may produce clinically symptomatic, radiologically apparent mass lesions. When considering a mass lesion arising after intracranial surgery, the differential diagnosis should include textiloma along with recurrent tumor and radiation necrosis.

West Nile encephalitis in 2 hematopoietic stem cell transplant recipients: case series and literature review.

Most human cases of West Nile virus infection are acquired via bites from an infected mosquito. In some cases, infection may also be transmitted by infected blood products or transplanted organs. There have been recent publications suggesting that chemotherapy and immunosuppression may increase a person's risks of developing central nervous system disease if the person is infected with the West Nile virus. Because patients undergoing hematopoietic stem cell transplantation not only are immunocompromised, but also receive multiple blood products, they are at a particularly high risk for acquiring symptomatic disease if exposed to the West Nile Virus. We describe here 2 patients who underwent hematopoietic transplantation at our institution and subsequently developed fatal West Nile virus infections.

Pathologically proven cavernous angiomas of the brain following radiation therapy for pediatric brain tumors.

Lesions consistent with cavernous angiomas (CAs) of the brain are sometimes seen on MRI scans of the brains of patients who received radiation therapy for brain tumors as children. The lesions appear years later within brain tissue that was included in radiation fields. It is unclear whether these MRI-detected lesions are true CAs or a pathological variant. This study reports the clinical, radiographical, and pathological findings in 3 cases of radiation-induced CAs of the brain. From 1995 to 1997, 3 patients previously treated with radiation therapy (45-55 Gy) for pediatric brain tumors (medulloblastoma, ependymoma, and a presumed midbrain astrocytoma) underwent resections of symptomatic and enlarging lesions that were consistent with a CA of the brain. All of the lesions occurred within fields of prior irradiation. None of the patients had received chemotherapy as part of their cancer treatment. CA-presenting symptoms included seizures, cranial nerve deficits, and headaches. The lesions appeared 7-19 years after radiation therapy and slowly enlarged on subsequent imaging studies. MRI scans of the lesions revealed characteristics typical of CA. The lesions became symptomatic 1-5 years after they were initially noted. Surgical resection was performed 1-2 years after symptoms began. The age at resection ranged from 15 to 23 years (10-21 years after radiation therapy). Pathological analysis of the three lesions showed typical CA characteristics. Some CAs may be caused by radiation therapy for pediatric brain tumors. They are radiologically and pathologically similar to sporadically occurring CAs of the brain and may enlarge over time and become symptomatic. CAs can be safely resected using standard microsurgical techniques.

Melanoma metastatic to central neurocytoma: a novel case of tumor-to-tumor metastasis.

We report a case of a 43-year-old man with known metastatic melanoma and two intracranial tumors, one of which was resected and confirmed to be melanoma. At autopsy, the second lesion was found to be a central neurocytoma harboring metastatic melanoma. To our knowledge, this represents the first reported case of tumor-to-tumor metastasis to a central neurocytoma. The most common pattern of tumor-to-tumor metastasis for intracranial neoplasms, in which an aggressive high-grade malignancy serves as the source tumor and a more indolent neoplasm serves as the recipient tumor, is affirmed by the present novel example.

Method for efficient fast spin echo Dixon imaging.

In order to satisfy the Carr-Purcell-Meiboom-Gill (CPMG) condition, echo shift as dictated in fast-spin-echo (FSE)-based Dixon imaging was previously achieved by applying a time shift to the readout gradient and the data acquisition window. Accordingly, interecho spacing is increased, which entails increased image blurring and, in multislice imaging, a significant reduction in the slice coverage for a given imaging time. In this work, a new method is developed by which the echo shift is induced by "sandwiching" in time the readout gradient with a pair of small gradients of equal area and of opposite polarity. While data with non-zero phase shifts between water and fat signals are collected as fractional echoes, no increase in echo spacing is necessary with the modified acquisition strategy, and increased time efficiency is therefore achieved. In order to generate separate water-only and fat-only images in data processing, a set of low-resolution images are first reconstructed from the central symmetric portion (either 128 x 128 or 64 x 64) of the acquired multipoint Dixon data. High-resolution images using all the acquired data, including some partial Fourier-reconstructed images, are then phase demodulated using the phase errors determined from the low-resolution images. The feasibility of the technique is demonstrated using a water and fat phantom as well as in clinical patient imaging.

MR imaging of leptomeningeal metastases: comparison of three sequences.

BACKGROUND AND PURPOSE: Recent work has shown that fluid-attenuated inversion recovery (FLAIR) imaging with contrast enhancement is highly sensitive for detecting subarachnoid space disease. We hypothesized that contrast-enhanced FLAIR imaging has superior sensitivity to contrast-enhanced T1-weighted MR imaging in detecting leptomeningeal metastases. METHODS: Sixty-eight patients referred for suspected leptomeningeal metastases underwent 74 MR imaging studies. The patients had either temporally related cytologic proof of leptomeningeal metastases or negative results of clinical follow-up confirming absence of leptomeningeal metastases. The MR imaging examinations included unenhanced and contrast-enhanced FLAIR images and contrast-enhanced T1-weighted MR images that were independently reviewed by two neuroradiologists blinded to the results of cytology. Each of the three sequences was reviewed individually and separately and was assigned a score of positive or negative for leptomeningeal metastases. Discrepancies were settled by consensus. RESULTS: Of the 17 studies of patients with cytology-proven leptomeningeal metastases, two were positive based on unenhanced FLAIR images, seven were positive based on contrast-enhanced FLAIR images, and 10 were positive based on contrast-enhanced T1-weighted MR images. Of the 57 studies of patients without leptomeningeal metastases, 53 were negative based on unenhanced FLAIR images, 50 were negative based on contrast-enhanced FLAIR images, and 53 were negative based on contrast-enhanced T1-weighted MR images. The sensitivity and specificity of unenhanced FLAIR images for detecting leptomeningeal metastases were 12% (two of 17) and 93% (53 of 57), respectively. The sensitivity and specificity for contrast-enhanced FLAIR images for detecting leptomeningeal metastases were 41% (seven of 17) and 88% (50 of 57), respectively. The sensitivity and specificity of contrast-enhanced T1-weighted MR images for detecting leptomeningeal metastases were 59% (10 of 17) and 93% (53 of 57), respectively. CONCLUSION: Contrast-enhanced fast FLAIR sequences are less sensitive than standard contrast-enhanced T1-weighted MR sequences in detecting intracranial neoplastic leptomeningeal disease.

Unusual small choroid plexus cyst obstructing the foramen of monroe: case report.

This is a case report of unusual case of choroid plexus cyst at the right foramen of Monro in the anterior third ventricle that caused unilateral obstructive hydrocephalus. The value of small-FOV thin-section MR imaging in the diagnosis of small lesions of the foramen of Monroe is demonstrated. The immunohistochemical findings in choroid epithelial cysts in comparison with those of other types of cysts at this location are discussed.

Characterization of benign and metastatic vertebral compression fractures with quantitative diffusion MR imaging.

BACKGROUND AND PURPOSE: Conventional imaging techniques cannot be used to unambiguously and reliably differentiate malignant from benign vertebral compression fractures. Our hypothesis is that these malignant and benign vertebral lesions can be better distinguished on the basis of tissue apparent diffusion coefficients (ADCs). The purpose of this study was to test this hypothesis by using a quantitative diffusion imaging technique. METHODS: Twenty-seven patients with known cancer and suspected metastatic vertebral lesions underwent 1.5-T conventional T1-weighted, T2-weighted, and contrast-enhanced T1-weighted imaging to identify the lesions. Diffusion-weighted images of the areas of interest were acquired by using a fast spin-echo diffusion pulse sequence with b values of 0-250 s/mm(2). The abnormal regions on the diffusion-weighted images were outlined by using the conventional images as guides, and the ADC values were calculated. On the basis of pathologic results and clinical findings, the cases were divided into two categories: benign compression fractures and metastatic lesions. The ADC values for each category were combined and plotted as histograms; this procedure was followed by statistical analysis. RESULTS: The patient group had 12 benign fractures and 15 metastases. The mean ADC values, as obtained from the histograms, were (1.9 +/- 0.3) x 10(-4) mm(2)/s and (3.2 +/- 0.5) x 10(-4) mm(2)/s for metastases and benign fractures, respectively. CONCLUSION: Our results indicate that quantitative ADC mapping, instead of qualitative diffusion-weighted imaging, can provide valuable information in differentiating benign vertebral fractures from metastatic lesions.