Dural-based Lesions Research Articles

Magnetic Resonance–Guided Laser Ablation for the Treatment of Recurrent Dural-Based Lesions: A Series of Five Cases

Magnetic resonance-guided laser-induced thermotherapy (MR-LITT) is a minimally invasive technique that shows promise in neuro-oncology because of its superiority in delivering precise minimally invasive thermal energy with minimal collateral damage. In this analysis, we investigate initial data on the effect of MR-LITT on dural-based lesions. Five patients were identified with dural-based lesions (4 meningiomas, 1 solitary fibrous tumor) with clear evidence of radiologic progression. In all 5 cases, the tumors were localized to the lateral convexity or paramedian locations in the supratentorial space. All patients received MR-LITT and then a follow-up magnetic resonance imaging scan at 24 hours after treatment, at 1 month, and at each subsequent follow-up visit. Local control of the ablated tumor was evaluated with radiographic follow-up and symptomatic progression-free survival was recorded. Five LITT treatments were performed on 5 patients with an average age of 65.2 years. The average tumor volume was 29.7 cm3 and ablation dosage was 12.4 W. On average, 80% of the pretreatment lesion volume was ablated. The mean follow-up time was 59.3 weeks. In total, 2 patients (1 with an anaplastic meningioma and 1 with a solitary fibrous tumor) had radiographic evidence of disease progression. In the observed time of the 3 patients with no progression, there was a 52% reduction in tumor volume. There were no major perioperative complications. MR-LITT is a promising technology for dural-based lesion treatment. This initial study demonstrates that MR-LITT is safe and offers several advantages over open surgical treatment. Randomized studies are needed to evaluate its role as a treatment adjunct.

Tuberculosis of the Clivus

AbstractThe rarity of the tubercular involvement of the clivus makes the preoperative diagnosis difficult. We report a case of a 40-year-old man who presented with right-sided trigeminal neuralgia without any other neurological symptoms or deficits. Postcontrast images showed a dural-based lesion in the region of the clivus which was isointense on T1 images and hypointense on T2 images and the lesion was enhancing after contrast administration suggestive of a tumor. The patient underwent surgical excision of the lesion and the histopathological examination was suggestive of tuberculosis. The patient completed the course of antitubercular therapy and recovered well. Tuberculosis of the clivus region runs an indolent course and following appropriate treatment these patient do well.

Intracranial Rosai–Dorfman disease

Rosai–Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system. We present the diagnosis and treatment of five patients with intracranial RDD. The patients were preoperatively misdiagnosed as meningioma or eosinophilic granuloma. All five patients were treated by total or subtotal surgical resection and none of them experienced recurrence. Histopathological examination showed a characteristic emperipolesis, the lymphocytes were engulfed in the S-100 protein and CD68 positive histiocytes, with negative expression of CD1a. Preoperative diagnosis of intracranial RDD is still challenging because the lesion is usually a dural-based lesion that mimics a meningioma. Surgical resection is an effective treatment and radiotherapy, steroid and chemotherapy has not demonstrated reliable therapeutic efficiency.

Extended Pericranial Flap for Anterolateral Skull Base Reconstruction after Frontotemporal Orbitozygomatic Transcavernous Approaches: Operative Technique and Nuances

Introduction: Technical advances in neurosurgery over the past several decades have allowed for the safe resection of deep and invasive skull base lesions previously deemed inoperable. Frontotemporal orbitozygomatic approaches are useful for resecting parasellar and cavernous sinus lesions. Meningiomas and other dural-based lesions arising from this region often require extensive dural resection and bone removal leading to a significant risk for cerebrospinal fluid leakage. Pericranial flap reconstruction has become a popular method for reconstructing skull base defects due to its autologous nature, ease of harvesting, and natural vascular pedicle. However, the lateral extent of the pericranium ends at the superior temporal line (STL) and the surface area is somewhat limited when using a frontotemporal curvilinear incision.

Metastatic thymoma – A rare presentation as a dural-based spindle cell lesion in the cranial cavity

Metastatic thymoma – A rare presentation as a dural-based spindle cell lesion in the cranial cavity

Neurosyphilis.

This article reviews the etiology, clinical manifestations, diagnosis, and treatment of neurosyphilis, with a focus on issues of particular relevance to neurologists. The number of cases of infectious syphilis in the United States has steadily increased since 2000. The highest rates are among men who have sex with men, and approximately half of these individuals are infected with human immunodeficiency virus (HIV). Neurosyphilis is a serious complication of syphilis that can develop at any time in the course of syphilis. Two neuroimaging patterns should alert the neurologist to a diagnosis of neurosyphilis: cerebral gummas, which are dural-based lesions that can mimic meningiomas, and medial temporal lobe abnormalities that can mimic herpes encephalitis. Penicillin G is the recommended treatment for neurosyphilis, but ceftriaxone may be an acceptable alternative. The diagnosis of neurosyphilis can be challenging. A sound understanding of the clinical manifestations and the strengths and limitations of diagnostic tests are essential tools for the neurologist.

Cerebral cavernous malformations associated to meningioma: High penetrance in a novel family mutated in the PDCD10 gene.

Multiple familial meningiomas occur in rare genetic syndromes, particularly neurofibromatosis type 2. The association of meningiomas and cerebral cavernous malformations (CCMs) has been reported in few patients in the medical literature. The purpose of our study is to corroborate a preferential association of CCMs and multiple meningiomas in subjects harbouring mutations in the PDCD10 gene (also known as CCM3). Three members of an Italian family affected by seizures underwent conventional brain Magnetic Resonance Imaging (MRI) with gadolinium contrast agent including gradient echo (GRE) imaging. The three CCM-causative genes were sequenced by Sanger method. Literature data reporting patients with coexistence of CCMs and meningiomas were reviewed. MRI demonstrated dural-based meningioma-like lesions associated to multiple parenchymal CCMs in all affected individuals. A disease-causative mutation in the PDCD10 gene (p.Gln112PhefsX13) was identified. Based on neuroradiological and molecular data as well as on literature review, we outline a consistent association between PDCD10 mutations and a syndrome of CCMs with multiple meningiomas. This condition should be considered in the differential diagnosis of multiple/familial meningioma syndromes. In case of multiple/familial meningioma the use of appropriate MRI technique may include GRE and/or susceptibility-weighted imaging (SWI) to rule out CCM. By contrast, proper post-gadolinium scans may aid defining dural lesions in CCM patients and are indicated in PDCD10-mutated individuals.

Abstract P6-16-03: Intrinsic subtypes and MRI patterns in brain metastasis associated with breast cancer

Abstract Background: Breast cancer is the 2nd most common cancer to metastasize to the brain. The development of brain metastasis (BM) is associated with a lower median survival compared with other locations of metastasis and carries with it high morbidity and reduced quality of life. There are limited treatment options and virtually no approved targeted therapies for this disease. We have previously reported specific pathways enriched in BM compared to primary tumors and now further this study to examine pathways by intrinsic subtype and location of and number of BM. Methods: Archival FFPE material was obtained from BM using pathology records; clinical data, including MRI images, was retrieved from medical records and institutional tumor registry under an IRB protocol. Tumor DNA/RNA was extracted from 2 mm cores macrodissected from the FFPE tissue blocks using the Qiagen AllPrep DNA/RNA FFPE kit. Gene expression profiling was performed using Affymetrix Human Transcriptome Array 2.0 microarray on BM samples for which sufficient RNA was available. Gene-level expression quantification was derived after RMA normalization using the Affymetrix Transcriptome Analysis Console. PAM50 subtypes were assigned by clustering samples using median-subtracted PAM50 gene expression levels. Differential gene expression was estimated using the non-parametric Mann-Whitney test, followed by assessment of false discovery rate using the Banjamini-Hochberg FDR methodology. Pathway enrichment analysis was performed using the NCI Pathway Interaction Database. Results: Gene expression profiling showed the following intrinsic subtype distribution among all BM: luminal A 32% (19/59), luminal B 31% (18/59), HER2 enriched 7% (4/59), and basal subtype 31% (18/59). At time of development of BM 64% (38/59) of patients presented with a single lesion compared to 36% (21/59) of patients who presented with multiple lesions (p=0.12). Thirty-nine percent (7/18) of patients with basal subtypes were observed to present with multiple BM compared to 61% (11/18) non-basal subtypes (p=0.25). In addition, 12% (13/59) of BM were found to be exclusively dural-based lesions. They appeared more frequently in the luminal subtypes [11/13 vs 2/13; p=0.06]. A total of 314 genes were differentially expressed (Wilcox pval < 0.05; FDR < 0.01) between the basal and luminal subtypes. As expected, we found that the FOXA transcription factor network was up-regulated in luminal when compared to the basal subtype, whereas the FOXM1 transcription factor network was up-regulated in the basals. We also found a total of 28 genes to be significantly differentially expressed (Wilcox pval < 0.05; FDR < 0.01) between the dural and non-dural BM. The beta1 integrin and syndecan-1 pathways were significantly enriched, along with angiogenesis and lymphatic endothelium pathways. Key genes in these pathways (COL1A1, COL1A2, COL3A1, CDH11, were found to be at least 2-fold up-regulated in the dural BM compared to non-dural BM. Conclusion: Identifying pathways that are differentially expressed between intrinsic subtypes may help us develop new targeted therapies to provide more treatment options for breast cancer patients with brain metastasis. Citation Format: Nicole Williams, Vinay Varadan, Aditi Vadodkar, Kristy Miskimen, Stephanie Kim, Shaveta Vinayak, Paula Silverman, Jill Barnholtz-Sloan, Andrew Sloan, Cheryl Thompson, Lisa Rogers, Hannah Gilmore, Lyndsay Harris. Intrinsic subtypes and MRI patterns in brain metastasis associated with breast cancer [abstract]. In: Proceedings of the Thirty-Seventh Annual CTRC-AACR San Antonio Breast Cancer Symposium: 2014 Dec 9-13; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2015;75(9 Suppl):Abstract nr P6-16-03.

MS-30 * MENINGEAL MELANOCYTOMA WITH MULTIFOCALITY, RAPID CNS SEEDING, AND SYSTEMIC METASTASES

OBJECTIVE: Meningeal melanocytomas are rare entities that range from benign and well-differentiated lesions with a favorable prognosis to higher-grade lesions that may undergo malignant transformation with subsequent CNS dissemination. Transition from a meningeal melanocytoma to primary CNS melanoma is particularly rare with only a handful of cases reported. Systemic spread of a transformed meningeal melanocytoma, an exquisitely rare occurrence, is, to the best of our knowledge, reported in only three cases. Here we report the case of a woman who initially presented with multifocal meningeal melanocytomas with subsequent rapid transformation, diffuse CNS seeding, and systemic metastases. CLINICAL PRESENTATION AND MANAGEMENT: A 43 year-old woman presented with headaches, nausea, and vomiting for two months with negative GI work-up. She experienced a syncopal episode with CT and MRI brain scans showing 3 dural-based lesions involving the foramen magnum, fourth ventricle, and right CPA. The patient underwent STR of the lesions; the anterior lesion was inaccessible. Pathology reported a melanocytoma with malignant features. A negative dermatology and ophthalmology work-up were performed. The patient received post-operative radiotherapy (50.4 Gy) to the posterior fossa. Six months following surgery she presented with new neurological symptoms, and MRI of the neuroaxis showed new leptomeningeal enhancement within the cerebellum, thoracic spine, and supratentorially. With neuro-oncology input, she was started on Temodar and underwent palliative spinal RT. Unfortunately she continued to experience pain, and follow-up MRI showed new intrathecal C-spine nodules. Abdominal imaging revealed multiple lung and liver metastases, with biopsy of a 7-cm liver lesion revealing malignant melanoma. The patient unfortunately continued to deteriorate and succumbed to her disease approximately 9 months following diagnosis. CONCLUSION: Meningeal melanocytomas are rare tumors that are not only challenging to diagnose, but challenging to clinically predict and manage. This case illustrates these challenges and the multidisciplinary response needed to optimize outcomes.

P08.06 * CEREBRAL DURAL METASTASES MIMICKING MENINGIOMAS. DIAGNOSTIC AND SURGICAL FEATURES

Cerebral metastases are the most frequent brain tumors in adults and they are found in 15-30% of patients with a primary tumor. They develop mainly in the brain or cerebellum and less frequently in the meninges. Dural metastases are rare entities and are found in 9% of patients with terminal systemic cancer and in about 5% they represent the only intra-cranial manifestation. However their real incidence is unknown because of the lack of significant numerical series. Clinical presentation with dural-based metastasis mimicking meningioma is very rare and described mostly as case reports from diverse primary locations. The most common primary sites in surgically resected dural metastases have been found to be breast and prostate, but may also be from a primary melanoma, sarcoma, or lymphoma. These often presented as single, cranial and subdural lesions. They can present dural tail sign and often are dural-based or intra-parenchymal. The determination of extra-axial origin of a tumor has a considerable clinical implication. The location of the lesion affects treatment planning and it is predictive for prognosis. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) can show a well defined, lobulated, extra-axial, contrast enhancing lesion. Previously, dural tail sign was reported to be specific for meningiomas, but may be associated with other dural-based lesions. Furthermore, their macroscopic appearance during surgery may even be taken for a meningioma. Because of the prognostic relevance in discriminating both tumors, the definitive diagnosis relies on the histopathological findings. Nowadays MR spectroscopy (MRs) can aid in the differential diagnosis. We report ten cases of dural metastasis from different neoplasms, which on both preoperative CT and MRI and at surgery had the typical appearance of a meningioma. Aim of our study was to investigate the neuroradiological findings and neuropathological aspects of these lesions in order to facilitate the differential diagnosis between meningiomas and other neoplasms. The neuroradiological data were compared with intra-operative characteristics and histological and specific immunohistochemical markers. In our patients the neuroradiological diagnosis of meningiomas was not confirmed by histology. MRs provided interesting complementary data, able to increase the specificity of the neuroradiological diagnosis but in many cases was unconvincing. It is certainly mandatory to resect these lesions, often they have a clear interfaces, much like a meningioma, which allows what appears to be a gross total excision. We still plan to obtain an intra-operative pathologic confirmation for suspected lesions that involve the dura. We also would like to emphasize the precise role of pre-operative diagnosis, which in many cases may be a challenge, to avoid the delay in surgery, with consequent deleterious impact on patient care.

“Brain on fire”: A new imaging sign

Primary central nervous system (CNS) marginal zone B cell lymphoma is a rare condition. It has an indolent disease course and usually presents as a dural-based lesion. We present a patient with non-dural-based, primary CNS marginal zone B cell lymphoma with an unusual imaging appearance, not previously described to our knowledge.

From the Radiologic Pathology Archives: Mass Lesions of the Dura: Beyond Meningioma—Radiologic-Pathologic Correlation

Meningioma is the most common mass involving the dura, making it number one in the differential diagnosis for any dural-based mass; however, a variety of other neoplastic and nonneoplastic lesions also involve the dura. Knowledge of the dural anatomy can provide clues to the various processes that may involve this location. The neoplastic processes include both benign and malignant lesions such as hemangiopericytoma, lymphoma, solitary fibrous tumor, melanocytic lesions, Epstein-Barr virus-associated smooth muscle tumors, Rosai-Dorfman disease, and metastatic lesions. The nonneoplastic processes include infectious and inflammatory entities such as tuberculosis and sarcoid, which may mimic mass lesions. In some cases, neoplasms such as gliosarcoma may arise peripherally from the brain parenchyma, appearing dural-based and even inciting a dural tail. Many of these share similar computed tomographic, magnetic resonance imaging, and angiographic characteristics with meningiomas, such as a dural tail, increased vascularity, avid enhancement, and similar signal characteristics; however, knowledge of the patient's age, gender, and underlying conditions and certain imaging characteristics may provide valuable clues to recognizing these lesions. For example, in the population with human immunodeficiency virus infection, Epstein-Barr virus-associated smooth muscle tumors should be included in the differential diagnosis for dural-based lesions. The surgical course and prognosis for these lesions vary, and knowledge of the variety of lesions that involve the dura, their imaging appearances, and their clinical features assists in narrowing the radiologic differential diagnosis and optimizing patient treatment.

Subdural B Cell Lymphoma

Dural-based B cell lymphomas are rare and have a female preponderance. A 60-year-old Asian man with a history of trivial trauma presented with generalised tonic clonic seizures and headache. Imaging and clinical work-up was done. A temporoparietal subdural lesion with no evidence of systemic lymphoma was detected. Intraoperatively, a dural-based mass lesion was seen with thickened dura and biopsy-proven B cell lymphoma, and the patient was then kept on chemotherapy. A suspicion of this rare entity should be considered in imaging of dural-based lesions.

Hematoidin (Crystallized Bilirubin) Crystals in an Atypical Meningioma

Hematoidin, first described by Virchow, results from histiocytic breakdown of extravasated red blood cells into hemosiderin, which is converted to biliverdin, then reduced to iron free crystallized bilirubin, or hematoidin, in a hypoxic environment. Crystalline structures, typically sunburst or rhomboid, may be found extracellularly or intracellularly. An 82-year-old male presented with a slowly growing mass on the vertex of the head. Neuroimaging demonstrated a dural-based lesion, with lytic bone destruction of the skull and extension into the scalp (Figure 1A). Histology showed an atypical meningioma. In the subcutaneous tumor, adjacent to hemorrhage, there were intrahistiocytic and extracellular golden crystalline structures in starburst 510028 IJSXXX10.1177/1066896913510028International Journal of Surgical PathologyStueck and Easton research-article2013

Intracranial pseudolymphoma presenting with grand mal seizures

Primary central nervous system lymphoproliferative disorders comprise a heterogenous group of intracranial disease, predominantly of the high-grade non-Hodgkin’s lymphoma type. We report a 56-year-old woman who developed new-onset grand mal seizures and was found to have two small uniformly enhancing dural-based lesions, which were radiologically concerning for meningiomas. Biopsy demonstrated findings consistent with benign, reactive lymphoid tissue. The patient’s seizures resolved post-operatively. To our knowledge, this is the first reported patient with intracranial pseudolymphoma presenting as grand mal seizures. This case highlights this rare differential consideration in a patient with symptomatic dural-based lesion.

Case report from the NIH Clinical Center: CNS nocardiosis

Case report from the NIH Clinical Center: CNS nocardiosis

CCM3 Mutations Are Associated with Early-Onset Cerebral Hemorrhage and Multiple Meningiomas

Mutations of CCM3/PDCD10 cause 10-15% of hereditary cerebral cavernous malformations. The phenotypic characterization of CCM3-mutated patients has been hampered by the limited number of patients harboring a mutation in this gene. This is the first report on molecular and clinical features of a large cohort of CCM3 patients. Molecular screening for point mutations and deletions was used to identify 54 CCM3-mutated index patients. Age at referral and clinical onset, type of inaugural events and presence of extra-axial lesions were investigated in these 54 index patients and 22 of their mutated relatives. Mean age at clinical onset was 23.0 ± 16 years. Clinical onset occurred before 10 years in 26% of the patients, and cerebral hemorrhage was the initial presentation in 72% of these patients. Multiple extra-axial, dural-based lesions were detected in 7 unrelated patients. These lesions proved to be meningiomas in 3 patients who underwent neurosurgery and pathological examination. This ‘multiple meningiomas' phenotype is not associated with a specific CCM3 mutation. Hence, CCM3 mutations are associated with a high risk of early-onset cerebral hemorrhage and with the presence of multiple meningiomas.

Fourth ventricular meningioma in an adult: Case report and review of the literature

Abstract Meningiomas are one of the most common clinical entities in everyday neurosurgical practice. Most meningiomas are extra-axial dural-based lesions. They typically occur along intradural venous sinuses, at the confluences of multiple cranial sutures and also others sites where arachnoid granulations and arachnoid cell rests occur. Most of these tumors also do occur within the lateral ventricles than third ventricle but they are exceptional in fourth ventricle thought to arise from the choroid plexus without dural attachment. Here we present two cases of fourth ventricle meningiomas.

Transient cranial neuropathies as sequelae of Onyx embolization of arteriovenous shunt lesions near the skull base: possible axonotmetic traction injuries

IntroductionTransarterial embolization with Onyx is a relatively safe and increasingly common method of treating cranial dural arteriovenous fistulas (DAVF) and arteriovenous malformations (AVM). Cranial neuropathy resulting from this procedure has...

IMAGING DIAGNOSIS—MAGNETIC RESONANCE IMAGING FINDINGS OF AN INTRACRANIAL EPIDURAL TUBERCULOMA IN A DOG

Magnetic resonance (MR) imaging is highly sensitive for detecting tuberculomas in human patients but the specificity of the MR imaging features is low. Misdiagnosis with intracranial neoplasia is common, especially with dural-based lesions or lesions located in the epidural space. We describe the MR imaging characteristics of an intracranial epidural tuberculoma caused by Mycobacterium tuberculosis infection in a dog. The intracranial mass and skull flat bone lysis and erosion are similar to those described in human caseating tuberculomas and can mimic intracranial neoplastic disease.