Malignant meningioma of the falx cerebri in a child: case report and literature review.

Meningiomas are common intracranial tumors in adults but rare in pediatric cases, with malignant histological features being even less frequent. Primary pediatric malignant meningioma of the falx cerebri has not been previously reported in the literature. We present a case of a malignant meningioma of the falx cerebri in a child who was admitted to the hospital following head trauma, presenting with mental impairment and limb weakness. CT examination revealed a right parietal epidural hematoma and a large intracranial lesion. The patient underwent drainage of the intracranial hematoma and a two-frontal craniotomy with tumor resection under microscopy. Histopathological analysis confirmed malignant meningioma, and genetic testing identified a YAP1-MAML2 gene fusion. There is no consensus on the clinical, imaging, and pathological features of pediatric meningiomas. In this paper, we discuss the clinical features, diagnostic and treatment protocols, and pathological characteristics of this case, along with a review of the relevant literature.

Simultaneous sampling of both cingulate gyri using a single interhemispheric depth electrode: A technical note.

Background: Simultaneous sampling of the cingulate gyri through a single depth electrode inserted underneath the falx cerebri is clinically useful in certain cases of drug-resistant epilepsy. However, the frequency at which each region of the cingulate gyri - namely, anterior, middle, and posterior - can be simultaneously sampled with a single electrode remains uncertain. Methods: We assessed the anatomical relationship between the falx cerebri and the cingulate gyrus in 50 adults and children. Subsequently, we determined whether an arbitrary line, denoted as A (representing a 5 mm gap between the falx cerebri and corpus callosum necessary for depth electrode insertion), fell within the anterior, middle, or posterior cingulate gyrus. Results: The shape of the falx cerebri and its intersection point with the corpus callosum varied substantially across individuals, with a significant difference between children and adults (P = 0.02). The A line was located in the middle cingulate gyrus in 18 children (72%), while 3 (12%) and 4 (16%) had it located in the posterior and anterior cingulate gyrus, respectively. Among adults, 15 individuals (60%) had the A line in the middle cingulate gyrus, 10 (40%) in the posterior cingulate gyrus, and none in the anterior cingulate gyrus. Conclusion: This study demonstrates the feasibility of simultaneous sampling of both the anterior and middle cingulate gyri in adults and children. Moreover, it represents the first investigation to document the wide interindividual variability in the morphology of the falx cerebri and its association with the cingulate gyrus.

Adrenocorticotropic Hormone Producing Pituitary Carcinoma in the Falx Cerebri, Retroclival Region, Ethmoidal Cells, and Other Locations.

Pituitary carcinoma is a condition defined by metastasis of a pituitary tumor to a distant location, and it is a very rare type of adenohypophyseal tumor. We present a case of a 29-year-old female who was followed up in our Endocrinology Department. Past medical history included the diagnosis of Cushing disease and transsphenoidal tumor resection at 12 years of age, followed by transcranial resection two years later because of persistently elevated adrenocorticotropic hormone (ACTH). Despite the surgical management, the patient persisted with increased ACTH and hypercortisolism, and, thus, bilateral adrenalectomy was performed a year later. Two years after the procedure, the patient presented with a newly diagnosed pituitary macroadenoma, and the diagnosis of Nelson syndrome was made. Linear accelerator radiotherapy was given, which reduced the size of the tumor. Later, several imaging studies showed multiple lesions on the falx cerebri, posterior clinoid process, retroclival region, cerebellopontine angle, pterygopalatine fossa, infratentorial region, and posterior ethmoidal cells. Biopsy and immunohistochemistry of the falx cerebri lesions described ACTH-producing pituitary adenocarcinoma. Treatment was given with intramuscular octreotide, dabrafenib, and trametinib. Despite persistently elevated ACTH levels, the patient has since remained clinically stable, without new development or worsening of symptoms. There are three unique aspects of our case. First, we reported an unusual presentation of this disease, since the patient in our case was a female with an early age of onset. Second, this is the first reported case demonstrating pituitary carcinoma in the falx cerebri. Third, the prognosis of pituitary carcinoma is usually very poor, and mortality is extremely high; however, the patient in our case has been followed up for seven years since the diagnosis of the metastatic lesions and has remained clinically stable.

Dural arteriovenous fistulas in the falx cerebri: case series and literature review.

Dural arteriovenous fistulas (DAVFs) within the falx cerebri are infrequently documented and may be linked with the falcine sinus/venous plexus. The falcine sinus/venous plexus, often regarded as a normal venous structure, can exhibit pathological characteristics, differing from the persistent fetal falcine sinus. A retrospective analysis was conducted at a single center to identify all cases of DAVFs within the falx cerebri spanning from 2002 to 2022. Demographic data, fistula features, treatment modalities, clinical outcomes, and fistula closure were collected and analyzed. Additionally, relevant literature on DAVFs in this location was reviewed. Ten cases were identified at our center, supplemented by 13 cases reported in the literature. In our cohort, patients had an average age of 49.4 ± 8.1 years, with a male predominance of 90%. Trans-arterial embolization (TAE) alone achieved immediate complete occlusion in eight cases, while conservative treatment was pursued in two cases. No treatment-related complications or fistula recurrences were observed. In the literature, seven patients underwent direct surgery, three underwent TAE, and one underwent both direct surgery and radiosurgery for complete fistula closure. No instances of fistula recurrence or treatment complications were reported. Dural arteriovenous fistulas within the falx cerebri are rare, with limited literature available. They typically present as aggressive lesions. Treatment options include direct surgery or TAE. However, due to a lack of long-term DSA follow-up, the cure and recurrence rates are unknown for endovasdcular therapy. Further investigation is warranted to elucidate the involvement of the falcine sinus/venous plexus in falx cerebri DAVFs.

Further Expanding the Mutational Spectrum of Gorlin Syndrome in Three Unrelated Families.

Introduction: Gorlin syndrome is a rare, autosomal dominant multi-systemic disorder with a predisposition to the development of cancers such as medulloblastoma and nevoid basal cell carcinoma. Heterozygous pathogenic variants in PTCH1 are responsible for 90% of Gorlin syndrome cases. Pathogenic variants in PTCH1 cause overstimulation of the sonic hedgehog signaling pathway, which plays a role in the development of embryonic structures and tumorigenesis. Clinical major and minor diagnostic criteria for Gorlin syndrome have been determined. Odontogenic keratocyst (OKC) is the most common reason for medical admission in Gorlin syndrome. In this article, it is aimed to draw attention to the fact that patients with Gorlin syndrome are not very rare in our country and the variability in phenotypic and dysmorphic findings may be a clue for the diagnosis. Methods: Exome sequencing was performed on the Illumina NextSeq550 System platform by using the Ion Ampliseq exome RDY kit for Illumina. Sanger sequencing was performed accordingly for the other affected individuals in both families. Results: In this study, the clinical and molecular findings of 9 Gorlin syndrome patients from three unrelated families are presented. Macrocephaly, calcification of falx cerebri, palmar-plantar pits, rib anomalies, and OKC were detected in decreasing order in more than half of the patients. A novel heterozygous frameshift PTCH1 variant in family 1, a nonsense previously reported PTCH1 variant in family 2, and a novel heterozygous splice-site PTCH1 variant in family 3 were detected. Conclusion: Gorlin syndrome should be kept in mind in patients presenting with macrocephaly, palmoplantar pits, and OKC history. Careful examination of all family members is essential in the timely diagnosis of other affected individuals with minor phenotypic findings.

Dural composite hemangioendothelioma: The first intracranial case.

Background: Composite hemangioendothelioma (CHE) is a rare, locally aggressive neoplasm of intermediate malignant potential. It is composed of a mixture of vascular tumors with a predilection for the dermis and subcutis of the extremities. Case Description: In this report, we describe a 41-year-old man who presented with a 2-month history of headache, dizziness, and intermittent seizures. Magnetic resonance imaging showed a hemorrhagic, multilobulated, and dural-based mass with extension into the calvarium. The mass measured 10.3 × 4.8 × 4 cm along the interhemispheric fissure and encased the superior sagittal sinus. Excision was performed, and histopathologic examination revealed a heterogeneous mixture of vascular components consisting of epithelioid hemangioendothelioma, retiform hemangioendothelioma, and hemangioma. This is the first report of a primary intracranial CHE. Conclusion: The spectrum of mesenchymal neoplasms within the cranium expands to encompass CHE.

The distance between the falx cerebri and the corpus callosum in patients with bilateral subdural hematomas: A potential measure of disease severity.

BACKGROUND: We aimed to assess the usefulness of the falx cerebri and the corpus callosum measurements as imaging markers of the evaluation of patients with bilateral subdural hematomas. METHODS: The anterior-posterior and craniocaudal distances between the falx cerebri (FC) and the corpus callosum (CC) were retrospectively measured in 88 head CT scans from 2018 to 2022 from patients with bilateral subdural hematomas and associated with quantitative data and clinical outcomes. Statistical analysis was performed using multivariate regression and receiver operating characteristic curves. RESULTS: Of the 88 patients included, 77.3% were male and the median age of 76.0 years (interquartile range 14.0). The mean craniocaudal and anterior-posterior FC-CC distances were 27.6 ± 6.2 mm and 25.1 ± 6.9 mm, respectively, and showed a positive correlation with hematoma thickness and volume. Both anterior-posterior and craniocaudal FC-CC distances exhibited moderate to good inter-rater reliability. After adjusting for confounders, the craniocaudal FC-CC distance was associated with an increased risk of altered consciousness at admission (OR=1.013; 95% CI 1.001-1.024; p = 0.031), downward displacement of the third ventricle (OR=1.019; 95% CI 1.001-1.038; p = 0.035), and a reduced time to surgery (ß = 0.057; 95% CI 0.007-0.107; p = 0.027). CONCLUSION: This study emphasizes that increased FC-CC distances in patients with bilateral subdural hematomas may aid clinical decision-making and are associated with larger hematoma volumes, evidence of descending transtentorial herniation on imaging, and a heightened risk of altered consciousness at admission.

Falcine meningioma: FEBAIR principles in a "Grade Zero" concept application - 2D operative video.

Background: Falcine meningiomas account for 5% of intracranial meningiomas. They may involve the eloquent cortex as well as vascular structures. Gross-total resection with additional margins has been shown to be beneficial to patients and has been associated with a reduction in disease recurrence. Case Description: A 57-year-old patient presented with recurrent frontal headaches that worsened when lying down. Magnetic resonance imaging showed a large lesion with homogeneous enhancement attached to the anterior third of the falx cerebri at the right frontal lobe, causing significant compression, and suggesting a meningioma. Conclusion: This operative video highlights the application of 1st-time, exposure, bone removal, arachnoid, irrigation, and reconstruction principles for safely and effectively removing a large falcine meningioma using the "grade zero" concept for maximal resection.

Frameshift mutation in exon 17 of PTCH1 gene in Nevoid basal cell carcinoma syndrome: A case report.

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. In majority of the cases, the presence of multiple and recurrent jaw cysts especially during the first two decades of life is one of the first symptoms of this syndrome. We present here a case of 14-year-old female patient who reported with a chief complaint of facial swelling for 3 weeks. The radiographs revealed multiple cysts in maxilla and mandible. Incisional biopsy of the lesions was done and the histopathologic features were suggestive of odontogenic keratocyst. Further investigations revealed the presence of falx cerebri calcifications and multiple nevi on palms and feet. Genetic study was done to confirm the diagnosis of NBCCS, which showed mutations in PTCH gene. This case stresses the importance of genetic study in suspected cases of NBCCS especially in young patients of nonsyndromic parents.

Bulky supernumerary bone of the falx cerebri: case report and literature review.

We present a rare case of ossification of the falx cerebri in a 25-year-old man, discovered incidentally on a craniofacial computed tomodensitometry (CT) scanner. This ossification presented a true cortico-medullary differentiation and pushed aside the right frontal lobe. There was no associated clinical manifestation.

Endovascular Management of Falcine Dural Arteriovenous Fistula-A Case Report and Review of Literature.

Cranial dural arteriovenous (AV) fistulas are abnormal connections between the branches of dural arteries to dural veins or venous sinuses. They are most frequently located at the transverse sinus and cavernous sinus. They can occur at every cranial dural sinus. Dural AV fistula of falx cerebri is rare. A 62-year-old female presented with signs and symptoms of raised intracranial pressure. Radiological imaging revealed a dural AV fistula at the posterior one-third falx cerebri. She underwent transarterial embolization, and complete obliteration of the fistula was achieved. A detailed digital subtraction angiography study is warranted in patients with seemingly benign complaints like recurrent headaches, and falcine dural AV fistula should be identified and treated in the nick of time. We describe a very rare falcine dural AV fistula case and its management.

Distance Between the Corpus Callosum and the Falx Cerebri: A Little Explored Measure with Potential Surgical Implication.

OBJECTIVE: To perform measurements in normal brain imaging studies from the free edge of the falx cerebri to the genu and the body of the corpus callosum and perform a statistical analysis based on age, type of study, and sex highlighting potential surgical implication of these measurements. METHODS: In 193 normal brain imaging studies, 3 anatomical points of the corpus callosum were used to measure the distance to the falx cerebri. Horos and RadiAnt DICOM Viewer software were used to perform the measurements. Statistical analysis of data was performed with Minitab18 software. RESULTS: The results obtained in computed tomography (CT) studies were: distance A, mean 2.1065 cm; distance B, mean 2.2677 cm; distance C, mean 1.765 cm. The results obtained in magnetic resonance imaging studies were: distance A, mean 1.7148 cm; distance B, mean 2.1197 cm; distance C, mean 1.5321 cm. Statistically significant differences were obtained in measurements related to the type of study and in measurements made in CT studies related to age. CONCLUSIONS: There is a distance from the free edge of the falx cerebri to the genu and body of the corpus callosum of at least 1 cm in both CT and magnetic resonance imaging studies of normal brains. Statistically significant differences were found in the measurements in relation to the type of study and in relation to age in the measurements made in CT studies. These measurements could be important in determining the extent of bone resection in certain types of decompressive craniectomies.

A Rare Occurrence of Multiple Intracranial Osteomas in the Cranial Cavity of a Cadaver With a Short Review on Subdural Osteoma.

Osteomas are most common among all primary bone tumors of skull bones. They are usually asymptomatic due to their small size and slow growth. They are found incidentally on imaging studies for other neurologic symptoms. Osteoma may be single or multiple when present. They should be differentiated from meningiomas, chordomas, schwannomas, and parosteal osteosarcoma by using different diagnostic methods, including histopathologic study. During routine dissection for MBBS students in an 87 years old female cadaver, we found multiple (seven in number) irregular, lobulated bony masses/structures. Their positions were different with respect to the layers of meninges. Some were present between the dura mater and arachnoid mater compressing the adjacent brain tissues forming impressions on them, and some were outside the dura mater. So, into the previously existing classification, we want to add a new variety under the type b category, i.e., mixed type (intraparenchymal, dural, skull vault) as pointed under the subtype V, which is found in our case.

Giant chondrosarcoma of the falx in an adolescent: A case report.

BACKGROUND: Intracranial chondrosarcomas are slowly growing malignant cartilaginous tumors that are especially rare in adolescents. CASE DESCRIPTION: A 19-year-old woman with no medical history presented with symptoms of intermittent facial twitching and progressive generalized weakness for 6 months. The patient's physical examination was unremarkable. Imaging revealed a large bifrontal mass arising from the falx cerebri, with significant compression of both cerebral hemispheres and downward displacement of the corpus callosum. The patient underwent a bifrontal craniotomy for gross total resection of tumor. Neuropathologic examination revealed a bland cartilaginous lesion most consistent with low-grade chondrosarcoma. Her postoperative course was uneventful, and she was discharged to home on postoperative day 3. CONCLUSION: This is an unusual case of an extra-axial, non-skull base, low-grade chondrosarcoma presenting as facial spasm in an adolescent patient.

Symmetrical Interhemispheric Subdural Hematoma.

Interhemispheric subdural hematoma (ISDH) is a rare subtype of subdural hematoma. We report the case of an 81-year-old woman on hemodialysis with sudden nausea and vomiting. A computed tomography (CT) scan of the brain showed a bilaterally symmetrical increase in the thickness and density of the falx cerebri. At first, the findings were overlooked, but were later identified as an acute ISDH. The patient was treated conservatively and the symptoms completely resolved. The possibility of ISDH should be considered even if CT images of the brain are symmetrical.

Inferior sagittal sinus: magnetic resonance imaging study.

PURPOSE: To date, no study has explored the inferior sagittal sinus (ISS) using neuroimaging modalities. This investigation aimed to characterize it using magnetic resonance imaging (MRI). METHODS: A total of 77 patients with intact cerebral hemispheres and covering meninges underwent thin-sliced, contrast-enhanced MRI. RESULTS: The ISS was well delineated as a linear structure with a constant diameter in 97% of the patients. The maximum intensity projection (MIP) images well delineated the three-dimensional architecture of the ISS and relevant veins. The identified ISSs could be classified into three different types, with the underdeveloped type being the most frequent at 47%. In addition, the ISSs showed considerable variability both in the original site and course along the lower margin of the falx cerebri. Furthermore, in 22% of the cases, fenestrations were identified in the falx cerebri adjacent to or near the ISS. More than 70% of them were located in the middle third of the falx, followed by the anterior and middle thirds of the falx. CONCLUSIONS: The ISS is a constant venous structure characterized by morphological variability and may function as an adjunctive or assistive venous drainage route. Thin-sliced, post-contrast-enhanced sagittal MRI combined with MIP imaging is useful for exploring the ISS.

Topographical anatomy of the tentorium cerebelli and venous confluences in human midterm fetuses.

Early development of veins and sinuses at and around the posterior cranial fossa seemed not to be shown by photographs except for our recent study (Ann Anat, 2020). Examination of histological sections of 38 fetuses at 10-16 weeks gestational age (GA) demonstrated that: (1) the superior petrosal sinus passed posterosuperiorly through the tentorium cerebelli and, distant and lateral to both the cerebellum and internal ear, drained into the transverse sinus; (2) the superior sagittal sinus was underdeveloped, and the inferior sagittal sinus was not yet evident; (3) the straight sinus (STS) originated from a joining of the bilateral pial veins from the lateral ventricular choroid plexus, passed through the inferoposterior part of the falx cerebri, reached the initial confluens sinuum, and then divided into the bilateral transverse sinuses. The STS origin was immediately behind the pineal body, and near the inferoposterior end of the third ventricle. The falx had a thick attachment to the tentorium below the entire course of the STS and was behind other parts of the brain. Therefore, the development and growth of the posterior dural system seemed to be independent from brain growth, and occurred well before the cerebellum grew to fill the posterior cranial fossa. A basic configuration of intracranial veins and sinuses, including embryonic transient veins (such as the vena capitis prima) seemed to be established by venous return from the choroid plexus and cranial wall, without greatly increasing the abundance of neuronal or glial cells in the brain.

Mechanical Properties of the Cranial Meninges: A Systematic Review.

The meninges are membranous tissues that are pivotal in maintaining homeostasis of the central nervous system. Despite the importance of the cranial meninges in nervous system physiology and in head injury mechanics, our knowledge of the tissues' mechanical behavior and structural composition is limited. This systematic review analyzes the existing literature on the mechanical properties of the meningeal tissues. Publications were identified from a search of Scopus, Academic Search Complete, and Web of Science and screened for eligibility according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The review details the wide range of testing techniques employed to date and the significant variability in the observed experimental findings. Our findings identify many gaps in the current literature that can serve as a guide for future work for meningeal mechanics investigators. The review identifies no peer-reviewed mechanical data on the falx and tentorium tissues, both of which have been identified as key structures in influencing brain injury mechanics. A dearth of mechanical data for the pia-arachnoid complex also was identified (no experimental mechanics studies on the human pia-arachnoid complex were identified), which is desirable for biofidelic modeling of human head injuries. Finally, this review provides recommendations on how experiments can be conducted to allow for standardization of test methodologies, enabling simplified comparisons and conclusions on meningeal mechanics.

[Cystic meningioma of falx cerebri: About one case and literature review]. / Meningiome kystique de la faux du cerveau : A propos d'un cas clinique et revue de litterature.

Cystic meningioma of the falx is a rare extra-axial tumor, representing 4 to 7% of all intracranial meningioma. We report the case of a 23 years old patient with relative personal health history who has presented for nearly two years generalized headache associated with left hemiparesis. The head CT scan and MRI showed a right posterior parietal tumor with a cystic and fleshy component which was interpreted as glioma. The patient was operated and the intraoperative aspect was an extra axial lesion inserted to the middle tier of the falx cerebri with a clear cleavage plan with the cerebrum. A total ablation of the lesion was down and the histological study confirm the diagnosis. The cystic meningioma is a rare variant of intracerebral meningioma of which only histology makes it possible give the diagnosis of certainty.

Le méningiome kystique de la faux est une tumeur extra-axiale rare, représentant 4 à 7% des méningiomes intracrâniens. Nous rapportons le cas d'une patiente de 23 ans aux antécédents personnels de santé relative qui présentait depuis près de deux ans des céphalées généralisées associées à un déficit de l'hémicorps gauche. Le scanner et l'IRM préopératoire ont montré un processus tumoral pariétal postérieur droit avec une double composante kystique et charnue qui a été interprétée en préopératoire comme gliome. La patiente a été opérée et l'aspect peropératoire était celui d'une lésion extra-axiale insérée aux tiers moyen de la faux du cerveau avec un plan de clivage franc avec le cerveau adjacent. Une exérèse totale de la lésion est réalisée et l'étude histologique a confirmé le diagnostic. Le méningiome kystique est une variante rare de méningiome intracrânien dont seule l'histologie permet de donner le diagnostic de certitude.