Anatomical study of the mastoid foramina and mastoid emissary veins: classification and application to localizing the sigmoid sinus.

The mastoid foramen (MF) is located on the mastoid process of the temporal bone, adjacent to the occipitomastoid suture or the parietomastoid suture, and contains the mastoid emissary vein (MEV). In retrosigmoid craniotomy, the MEV has been used to localize the position of the sigmoid sinus and, thus, the placement of the initial burr hole. Therefore, this study aimed to examine the exact location and variants of the MF and MEV to determine if their use in localizing the sigmoid sinus is reasonable. The sample in this study comprised 22 adult dried skulls (44 sides). MF were identified and classified into five types based on location, prevalence, whether they communicated with the sigmoid sinus and exact entrance into the groove of the sigmoid sinus. The diameters and relative locations of the MF in the skull were measured and recorded. Finally, the skulls were drilled to investigate the course of the MEV. Additionally, ten latex-injected sides from human cadavers were also dissected to follow the MEV, especially in cases with more than one vein. We found that type I MFs (single foramen) were the most prevalent (50%). These MFs were mainly located on the occipitomastoid suture; only one case on the right side was adjacent to the parietomastoid suture. Type II (paired foramina) was the second most prevalent (22.73%), followed by type III (13.64%), type 0 (9.09%), and type IV (4.55%). The diameter of the external opening in a connecting MF (2.43 ± 0.79) was twice that of a non-connecting MF (1.14 ± 0.56). Interestingly, on one side, two MFs on the external surface shared a single internal opening; the MEV bifurcated. MFs followed three different courses: ascending, almost horizontal, and descending. Regardless of how many external openings there were for the MF, these all ended at a single opening in the groove for the sigmoid sinus. For cadaveric specimens with multiple MEVs, all terminated in the sigmoid sinus as a single vein, with the more medial veins terminating more medially into the sinus. Based on our study, the MF/MEV can guide the surgeon and help localize the deeper-lying sigmoid sinus. Knowledge of this anatomical relationship could be an adjunct to neuronavigational technologies.

An anatomical study of the sigmoid sinus artery: Application to the transmastoid approach.

INTRODUCTION: We aimed to investigate the morphological features of the artery that traverse the sigmoid sinus's lateral surface and to discuss this structure's clinical relevance. METHODS: Ten sides from five cadaveric Caucasian heads were used for gross anatomical dissection to investigate the morphological features of the sigmoid sinus artery (SSA), and additional five sides were used for histological observation. RESULTS: The SSA was found on eight out of ten sides (80%). The mean diameter of the SSA was 0.3 mm. The mean distance from the tip of the mastoid process to the artery was 20.3 mm. Histological observation identified extradural and intradural courses of SSA. The intradural course was further categorized into protruding and non-protruding types. In the protruding type, the SSA traveled within the dura but indented into the bone, making it more or less an intraosseous artery. In the non-protruding type, the SSA traveled within the dura but did not protrude into the bone but rather indented into the lumen of the SS. In all sections, both intradural and extradural courses were identified simultaneously. CONCLUSIONS: When the mastoid foramen is observed, it does not always only carry an emissary vein but also an artery. The SSA could be considered a "warning landmark" during bone drilling for the transmastoid approach.

How I do it - The "drum skin" duraplasty technique after foramen magnum decompression for Chiari malformations.

BACKGROUND: Chiari malformations are a spectrum of posterior cranial fossa anomalies characterized by herniation of the cerebellar tonsils through the foramen magnum. Surgery is the treatment of choice for selected patients with good postoperative outcomes. METHODS: We describe foramen magnum decompression (FMD) with dural opening and a "drum skin" duraplasty technique. CONCLUSIONS: In our experience, FMD with "drum skin" duraplasty is a safe and effective procedure, reducing the risk of CSF leakage and arachnoidal adhesions and yielding better long-term clinical and radiological outcomes than other traditional techniques.

Escalation therapy in severe traumatic brain injury: how long is intracranial pressure monitoring necessary?

Traumatic brain injury frequently causes an elevation of intracranial pressure (ICP) that could lead to reduction of cerebral perfusion pressure and cause brain ischemia. Invasive ICP monitoring is recommended by international guidelines, in order to reduce the incidence of secondary brain injury; although rare, the complications related to ICP probes could be dependent on the duration of monitoring. The aim of this manuscript is to clarify the appropriate timing for removal and management of invasive ICP monitoring, in order to reduce the risk of related complications and guarantee adequate cerebral autoregulatory control. There is no universal consensus concerning the duration of invasive ICP monitoring and its related complications, although the pertinent literature seems to show that the longer is the monitoring maintenance, the higher is the risk of technical issues. Besides, upon 72 h of normal ICP values or less than 72 h if the first computed tomography scan is normal (none or minimal signs of injury) and the neurological exam is available (allowing to observe variations and possible occurrence of new-onset pathological response), the removal of invasive ICP monitoring can be justified. The availability of non-invasive monitoring systems should be considered to follow up patients' clinical course after invasive ICP probe removal or for substituting the invasive monitoring in case of contraindication to its placement. Recently, optic nerve sheath diameter and straight sinus systolic flow velocity evaluation through ultrasound methods showed a good correlation with ICP values, demonstrating their potential role in place of invasive monitoring or in the early weaning phase from the invasive ICP monitoring.

Anatomical Study of the Zygomaticofacial Foramen and Its Related Canal.

The zygomaticofacial branch (ZFb) of the zygomatic nerve passes through the lateral wall of the orbit anterolaterally and traverses the zygomaticofacial foramen (ZFFOUT). However, in terms of intraorbital course, only a few studies have focused on the orbital opening of the ZFb (ZFFIN) and related canal. Therefore, this study aimed to locate the orbital opening and exit of the ZFb of the zygomatic nerve. Twenty sides from 10 fresh frozen cadaveric Caucasian heads were used in this study. The vertical distance between inferior margin of the orbit and ZFFIN (V-ZFFIN), the horizontal distance between the lateral margin of the orbit and ZFFIN (H-ZFFIN), diameter of the ZFFIN (D-ZFFIN), the vertical distance between the inferior margin of the orbit and ZFFOUT (V-ZFFOUT), the horizontal distance between the lateral margin of the orbit and ZFFOUT (H-ZFFOUT), and the diameter of the ZFFOUT (D-ZFFOUT) were measured, respectively. The ZFFIN were located 5.1 ±â€Š2.0 mm superior to the inferior margin of the orbit and 4.3 ±â€Š1.6 mm medial to the lateral margin of the orbit. The ZFFOUT was located 1.2 ±â€Š2.9 mm inferior to the inferior margin of the orbit and 1.1 ±â€Š3.0 mm lateral to the lateral margin of the orbit. The diameter of the ZFFOUT was significantly larger than that of the ZFFIN. Additional knowledge of the zygomatic nerve and its branches might decrease patient morbidity following invasive procedures around the inferolateral orbit.

Spontaneous Intracranial Hypotension and Subdural Hematomas Treatment Management Using MMA Embolization and Target Blood Patch: A Case Report.

We report a patient suffering from spontaneous intracranial hypotension (SIH) who, following a non-selective lumbar blood patch, returned to his healthcare provider with severe symptoms of neurological deficits. It was subsequently discovered that the aforementioned deficits were due to a bilateral subdural hematoma, and an emergency surgical drainage of the hematoma has been performed. However, the hematoma reformed and potential cerebrospinal fluid leakage was consequently investigated through myelography. Following the diagnostic finding of a venous diverticulum, a selective blood patch was executed in the affected area, and in order to stabilize the hematoma, an embolization of the middle meningeal arteries was performed. The combination of such operations allowed for the resorption of the hematoma and the improvement of neurological symptoms.

The Posterior Auricularis Muscle as Superficial Landmark for the Sigmoid Sinus and Transverse-Sigmoid Sinus Junction: An Anatomical Study.

OBJECTIVE: Neuronavigation systems coupled with previously reported external anatomical landmarks assist neurosurgeons during intracranial procedures. We aimed to verify whether the posterior auricularis muscle (PAM) could be used as an external landmark for identifying the sigmoid sinus (SS) and the transverse-sigmoid sinus junction (TSSJ) during posterior cranial fossa surgery. METHODS: The PAM was dissected in 10 adult cadaveric heads and after drilling the underlying bone, the relationships with the underlying SS and TSSJ were noted. The width and length of the PAM, and the distance between the muscle and reference points (asterion, mastoid tip, and midline), were measured. RESULTS: The PAM was identified in 18 sides (9 left, 9 right). The first 20 mm of the muscle length (mean 28.28 mm) consistently overlay the mastoid process anteriorly and the proximal half of the SS slightly posteriorly on all sides. The superior border was a mean of 2.22 mm inferior to the TSSJ and, especially when the muscle length exceeded 20 mm, this border extended closer to the transverse sinus; it was usually found at a mean of 3.11 mm (range 0.0-13.80 mm) inferior to the distal third of the transverse sinus. CONCLUSIONS: Superficial landmarks give surgeons improved surgical access, avoiding overexposure of deep neurovascular structures and reducing brain retraction. On the basis of our cadaveric study, the PAM is a reliable and accurate direct landmark for identifying the SS and TSSJ. The PAM could potentially be used for guiding the retrosigmoid approach.

Posterior Petrous Meningiomas: Surgical Classification and Postoperative Outcomes in a Case Series of 130 Patients Operated via the Retrosigmoid Approach.

OBJECTIVE: A standardized definition and classification of primary posterior petrous meningiomas (PPMs) is lacking, with consequent challenges in comparing different case series. This study aimed to provide an anatomical description and classification of PPMs analyzing a homogeneous series of patients operated via the retrosigmoid approach. METHODS: PPMs originate laterally to the petro-occipital fissure within the venous ring composed of the superior petrosal, sigmoid, inferior petrosal, and cavernous sinuses. We proposed a classification based on tumor site of origin, direction of growth relative to the internal acoustic meatus, and cranial nerves' displacement. Four types of PPMs were defined: retromeatal (type A), meatal (type B), premeatal (type C), and broad-based (type D). We performed a retrospective analysis of 130 consecutive patients with PPMs who underwent surgery as first-line treatment. RESULTS: The PPM classification predicted clinical presentation, postoperative morbidity, and resection rates. Headache, hydrocephalus, and cerebellar deficits were more common in type A (59.0%, 37.7%, 49.2%) and type D (66.7%, 66.7%, 33.3%). Hypoacusia/anacusia was more common in type B (87.5%), while trigeminal hypoesthesia/anesthesia was more common in type C (85.0%). After surgery, patients with type A and D PPMs were at higher risk to develop cerebellar deficits (11.5%-22.2%), whereas patients with type B and C PPMs presented with hypoacusia/anacusia (12.5%) and trigeminal deficits (10.0%), respectively. The near-total resection rate was higher in type A (91.8%), followed by types B (82.5%), C (80.0%), and D (77.8%) PPMs. CONCLUSIONS: The PPM surgical classification has an operative and prognostic relevance. In expert hands, the retrosigmoid approach represents a safe and effective approach to remove PPMs.

The Occipitalis Muscle as an Adjunct Superficial Landmark for the Transverse Sinus and Transverse-Sigmoid Junction: An Anatomical Study With Application to Posterior Cranial Fossa Surgery.

INTRODUCTION: Although neuronavigation systems are widely used for identifying deep intracranial structures, additional superficial anatomical landmarks can be useful when this technology is not available or is not working properly. Herein, we investigate the potential of the occipitalis muscle (OM), rarely mentioned in neurosurgical literature, as a superficial landmark for the transverse sinus (TS) and transverse-sigmoid sinus junction (TSJ). METHODS: Eighteen adult cadaveric heads underwent dissection. The borders of the OM were identified and measured. The muscle was then removed and the bone underlying the muscle was drilled. The relationships between the OM and the underlying dural venous sinuses were then investigated by using a surgical microscope. RESULTS: The OM is a quadrangular-shaped muscle, that invariably crosses the lambdoid suture, showing relationships with the TS inferiorly and the TSJ laterally. The medial border was located a mean of 2.7 cm from the midline and its lower edge was a mean of 1.6 cm above the TS. The inferior border was found between the lambdoid suture and the superior nuchal line in all the specimens. The medial half of the inferior margin was placed on average 1.1 cm superiorly to the TS while the lateral margin ran just above or over the TS. The lateral border was located a mean of 1.1 cm medially to the asterion and approximated the mastoid notch, being within 1-2 cm from it. The TSJ was between 2.1 and 3.4 cm lateral to OM lateral border. CONCLUSION: A combination of superficial anatomical landmarks can be useful for surgical planning. We found that the OM represents a valuable aide for neurosurgeons and is a reliable landmark for the deeper-lying TS and TSJ.

A Newly Discovered Dural Venous Sinus of the Skull Base: The Anterior Petroclinoid Sinus.

INTRODUCTION: Neurosurgeons must master microanatomy and its variants that occur at the skull base. To the best of our knowledge, we describe a previously unreported dural venous sinus (DVS) within the anterior petroclinoid fold and discuss its potential surgical relevance. METHODS: Fifteen latex-injected human cadaveric heads (30 sides) underwent skull base dissection using a surgical microscope. The anterior petroclinoid fold was opened in search of evidence of an embedded DVS. When identified, this sinus was documented, photographed, and measured with microcalipers. RESULTS: An anterior petroclinoid sinus (APCS) was identified in 67% of sides. These DVSs had a mean diameter of 0.65 mm and a length ranging from 8 to 15 mm (mean: 12.5 mm). The APCS ran from the anterior aspect of the cavernous sinus, arising just lateral (60%), posterior (20%), or medial (20%) to the anterior clinoid process, to the superior petrosal sinus (65%) or the posterior aspect of the cavernous sinus (35%), at the level of the posterior clinoid process. Along the course of the APCS, the oculomotor nerve was located medially at the oculomotor porus. Although slightly more common and larger on the right side, there was no statistically significant side difference. CONCLUSIONS: An improved knowledge of variant DVSs can lower the risk of intraoperative complications and increase our understanding of the venous outflow from the cavernous sinus. Future neuroimaging techniques might also seek to identify the APCS. This is the first description of a DVS contained within the anterior petroclinoid fold.

Neuroplasticity Mechanisms in Frontal Brain Gliomas: A Preliminary Study.

Background: Pathological brain processes may induce adaptive cortical reorganization, however, the mechanisms underlying neuroplasticity that occurs in the presence of lesions in eloquent areas are not fully explained. The aim of this study was to evaluate functional compensatory cortical activations in patients with frontal brain gliomas during a phonemic fluency task and to explore correlations with cognitive performance, white matter tracts microstructural alterations, and tumor histopathological and molecular characterization. Methods: Fifteen patients with frontal glioma were preoperatively investigated with an MRI study on a 3T scanner and a subgroup underwent an extensive neuropsychological assessment. The hemispheric laterality index (LI) was calculated through phonemic fluency task functional MRI (fMRI) activations in the frontal, parietal, and temporal lobe parcellations. Diffusion-weighted images were acquired for all patients and for a group of 24 matched healthy volunteers. Arcuate Fasciculus (AF) and Frontal Aslant Tract (FAT) tractography was performed using constrained spherical deconvolution diffusivity modeling and probabilistic fiber tracking. All patients were operated on with a resective aim and underwent adjuvant therapies, depending on the final diagnosis. Results: All patients during the phonemic fluency task fMRI showed left hemispheric dominance in temporal and parietal regions. Regarding frontal regions (i.e., frontal operculum) we found right hemispheric dominance that increases when considering only those patients with tumors located on the left side. These latter activations positively correlate with verbal and visuo-spatial short-term memory, and executive functions. No correlations were found between the left frontal operculum and cognitive performance. Furthermore, patients with IDH-1 mutation and without TERT mutation, showed higher rightward frontal operculum fMRI activations and better cognitive performance in tests measuring general cognitive abilities, semantic fluency, verbal short-term memory, and executive functions. As for white matter tracts, we found left and right AF and FAT microstructural alterations in patients with, respectively, left-sided and right-side glioma compared to controls. Conclusions: Compensatory cortical activation of the corresponding region in the non-dominant hemisphere and its association with better cognitive performance and more favorable histopathological and molecular tumor characteristics shed light on the neuroplasticity mechanisms that occur in the presence of a tumor, helping to predict the rate of post-operative deficit, with the final goal of improving patients'quality of life.

Is There a Role for Surgical Resection of Multifocal Glioblastoma? A Retrospective Analysis of 100 Patients.

BACKGROUND: Glioblastoma with multiple localizations (mGBMs) can be defined as multifocal, where enhancing lesions present a connection visible on magnetic resonance imaging fluid-attenuated inversion recovery imaging, or multicentric, in the absence of a clear dissemination pathway. OBJECTIVE: To evaluate the role of the extent of resection (EOR) in the treatment of mGBMs and its correlation with overall survival (OS) and progression free survival (PFS). METHODS: One hundred patients with mGBMs were treated at our Institution between 2009 and 2019. Clinical, radiological, and follow-up data were collected. EOR of the contrast-enhancing part of lesions was classified as gross total resection (GTR, absence of tumor remnant), subtotal resection (STR, residual tumor < 30% of the initial mass), partial resection (PR, residual tumor > 30% of the initial mass), and needle or open biopsy (residual tumor > 75% of the initial mass). RESULTS: Approximately 15% of patients underwent GTR, 14% STR, 32% PR, and 39% biopsy. Median OS was 17 mo for GTR, 11 mo for STR, 7 mo for PR, and 5 mo for biopsy. Greater EOR was associated with a significantly longer OS than biopsy. GTR and STR were associated with a longer PFS in Kaplan-Meier survival analyses. After adjusting for age, Karnofsky performance status (KPS), number of lesions, and adjunctive therapy in multivariable Cox regression analyses, GTR, STR, and PR were still associated with OS, but only GTR remained associated with PFS. CONCLUSION: Our study suggests that EOR may positively influence survival of patients with mGBM. Surgical resection can be a reasonable option when performance and access to adjuvant treatment can be preserved.

From Neurosurgical Planning to Histopathological Brain Tumor Characterization: Potentialities of Arcuate Fasciculus Along-Tract Diffusion Tensor Imaging Tractography Measures.

Background: Tractography has been widely adopted to improve brain gliomas' surgical planning and guide their resection. This study aimed to evaluate state-of-the-art of arcuate fasciculus (AF) tractography for surgical planning and explore the role of along-tract analyses in vivo for characterizing tumor histopathology. Methods: High angular resolution diffusion imaging (HARDI) images were acquired for nine patients with tumors located in or near language areas (age: 41 ± 14 years, mean ± standard deviation; five males) and 32 healthy volunteers (age: 39 ± 16 years; 16 males). Phonemic fluency task fMRI was acquired preoperatively for patients. AF tractography was performed using constrained spherical deconvolution diffusivity modeling and probabilistic fiber tracking. Along-tract analyses were performed, dividing the AF into 15 segments along the length of the tract defined using the Laplacian operator. For each AF segment, diffusion tensor imaging (DTI) measures were compared with those obtained in healthy controls (HCs). The hemispheric laterality index (LI) was calculated from language task fMRI activations in the frontal, parietal, and temporal lobe parcellations. Tumors were grouped into low/high grade (LG/HG). Results: Four tumors were LG gliomas (one dysembryoplastic neuroepithelial tumor and three glioma grade II) and five HG gliomas (two grade III and three grade IV). For LG tumors, gross total removal was achieved in all but one case, for HG in two patients. Tractography identified the AF trajectory in all cases. Four along-tract DTI measures potentially discriminated LG and HG tumor patients (false discovery rate < 0.1): the number of abnormal MD and RD segments, median AD, and MD measures. Both a higher number of abnormal AF segments and a higher AD and MD measures were associated with HG tumor patients. Moreover, correlations (unadjusted p < 0.05) were found between the parietal lobe LI and the DTI measures, which discriminated between LG and HG tumor patients. In particular, a more rightward parietal lobe activation (LI < 0) correlated with a higher number of abnormal MD segments (R = -0.732) and RD segments (R = -0.724). Conclusions: AF tractography allows to detect the course of the tract, favoring the safer-as-possible tumor resection. Our preliminary study shows that along-tract DTI metrics can provide useful information for differentiating LG and HG tumors during pre-surgical tumor characterization.

Cavernous malformations of the central nervous system in the pediatric age group.

OBJECTIVE: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. MATERIALS: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. RESULTS: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. CONCLUSION: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.

Spinal Dural Arteriovenous Fistulas: Clinical Results and Quality of Life Assessment with Surgical Treatment as a Crucial Therapy. The Joint Experience of Two Centers.

OBJECTIVE: Dorsal intradural arteriovenous fistulas (AVFs) consist of a direct connection between a radicular feeding artery and the coronal venous plexus; this direct connection leads to arterialization of the venous plexus, venous congestion, and myelopathy. Controversy still exists regarding the best treatment modality of spinal dural AVFs. Surgical disconnection of spinal dural AVFs is a straightforward procedure with a high success rate and virtually no risk of recurrence or incomplete treatment. To identify factors associated with the clinical progression of dorsal intradural AVFs and quantify the range of surgical outcomes in terms of neurologic improvement as well as patients' perception of quality of life (QOL). METHODS: A retrospective observational study of 19 consecutive patients treated with surgery over a 10-year period was carried out. We analyzed surgical results and clinical outcomes. We also evaluated the impact of this disease and its sequelae on the patients' postoperative health-related QOL. RESULTS: The surgical procedure showed good results in terms of neurologic improvement as well as patients' perception of QOL. CONCLUSIONS: Our series confirmed that surgical obliteration of dorsal intradural AVFs is an effective and safe procedure. The results of this retrospective analysis make us believe that surgery, given its low morbidity and high success rate, represents a safe and effective first therapeutic option for these spinal vascular malformations. It could be considered to avoid unsuccessful endovascular attempts that could delay the definitive treatment of this disease. The surgical procedure showed good results in terms of neurologic improvement as well as patients' perception of QOL.

Anatomic Study of Extracranial Needle Trajectory Using Hartel Technique for Percutaneous Treatment of Trigeminal Neuralgia.

OBJECTIVE: The aim of this study was to describe the anatomic trajectory of the extracranial needle for percutaneous rhizotomy and correlate this with structures at risk during such a procedure. METHODS: Six sides from 3 frozen fresh Caucasian heads were used in this study. Hartel anatomic landmarks for percutaneous trigeminal rhizotomy procedures were used. A free hand technique was then used, and intraprocedural visualization of the needle was performed with fluoroscopy. When the procedure was completed, the face was dissected along with the needle pathway up to the foramen ovale in order to evaluate for any damaged structures. RESULTS: On all sides, the needle passed lateral to the buccinator muscle and near the parotid duct to then pierce the superior head of the lateral pterygoid muscle to enter the infratemporal fossa. This placed the needle near the buccal nerve on all sides, although no direct injury was noted. Although very near, no branches of the facial nerve or artery were damaged. On 1 side, the maxillary artery was pierced. CONCLUSIONS: To our knowledge, this is the first study to describe the detailed extracranial anatomic needle pathway using the Hartel approach. Such data might help surgeons better recognize potential complications from such procedures.

Disseminated Oligodendroglial-like Leptomeningeal Tumor in the Adult: Case Report and Review of the Literature.

BACKGROUND: Diffuse leptomeningeal glioneuronal tumor (DLGNT) was recently added to the World Health Organization classification of central nervous system tumors. DLGNT is a rare entity that occurs more commonly in pediatric patients, but occasional cases have been reported in adults. This tumor has been recognized as a distinct pathologic entity; however, its biologic behavior remains unclear. It is considered an indolent neoplasm, although considerable morbidity has been reported. For this reason, further characterization and collection of evidence are crucial. METHODS: In this article, we reported a case of a 36-year-old woman with a DLGNT characterized by rapid, aggressive behavior. We also performed a review of the literature for reported cases of low-grade and high-grade forms involving adults and children. RESULTS: DLGNTs should no longer be considered only as low-grade tumors affecting pediatric patients. The spectrum of presentations also includes aggressive tumors affecting adults. CONCLUSIONS: Further clinical and pathologic data supported by cytogenetic and molecular investigations are mandatory to better characterize DLGNTs.

Primary Cardiac High-grade Myxofibrosarcoma Presenting with Multiple Brain Metastases: A Case Report.

Herein we describe the case of a young patient who presented with a recent history of epilepsy due to multiple brain lesions; he did not complain about any cardiopulmonary impairments. The patient died as a consequence of hemorrhagic progression of brain metastatic disease. Regardless of a thorough investigation, the heart tumor remained occult. Primary cardiac tumors are very rare entities. Most of these are benign, but approximately 25% are malignant, and the majority of these are sarcomas. Myxofibrosarcoma and osteosarcoma are exceptionally rare. To date, we find only small series of cardiac myxofibrosarcoma, and to our knowledge, this one exceptionally presented with multiple brain metastatic lesions without cardiopulmonary symptoms.