Sphenoid plasmacytoma as initial presentation of multiple myeloma-case report.

Plasmacytoma is a rare plasma cell neoplasm. Whether solitary or associated with multiple myeloma (MM), it rarely involves the skull base, particularly the sphenoid bone. We present a unique case of sphenoid bone plasmacytoma secondary to MM, highlighting diagnostic and therapeutic challenges. A 56-year-old female presented with headaches, vomiting, epistaxis, and cranial nerve deficits. Cerebral imaging revealed a 65-mm tumor infiltrating the sphenoid bone and adjacent structures. Subtotal resection was performed using an endoscopic nasal approach. Histopathology revealed plasmacytoma, and diagnostic workup confirmed MM. By the end of biological exploration, relapse of the sphenoid plasmacytoma was observed, and the patient was successfully treated with radiotherapy, immunochemotherapy, and autologous stem cell transplantation. After 18-month follow-up, sustained complete remission was confirmed. Although rare, the diagnosis of plasmacytoma should be considered in cases of skull base tumors. This localization is highly predictive of MM, warranting comprehensive investigations to initiate prompt and adequate management.

Surgical Management and Outcome of the Planum Sphenoidale Meningioma: A Single-Center Strobe Compliant Study.

BACKGROUND: The resection of the jugum (planum) sphenoidale meningioma (JSM) is challenging, and there is a scarcity of available data in the literature. This study aimed to describe the surgical management and long-term outcome of jugum sphenoidale meningioma. METHODS: This was a retrospective, single-center, cohort study of 91 JSM patients treated in our department from 2005 to 2022. All the included patients have undergone surgical treatment. Clinical, radiologic, and surgical data were extracted with specific attention to the age at onset, family history, duration from symptoms to diagnosis, clinical presentation, quality of excision, recurrence, and treatment outcome. RESULTS: WHO grade I and grade II meningioma were 83.5% (n = 76) and 16.5% (n = 15) respectively. Fronto-lateral 42.9% (n = 39) and pterional approaches 57.1% (n = 52) were the 2 major surgical approaches used for the management of planum sphenoidale meningioma. Twelve patients needed emergency surgical treatment at admission: 4 (4.4%) External CSF Shunt and 8 (8.8%) VP-Shunt. We have found a statistically significant (P = 0.032) association between the quality of excision and the risk of recurrence. The GTR (Hazard ratio = 1) of the planum meningioma protects the patient from recurrence within 60 months of follow-up. Postoperative complications encountered were CSF-Leak in 2.2%, Meningitis 4.4%, surgical site infection 2.2%, and surgical site hematoma 3.3%. The overall postoperative favorable outcome was 68.1% (n = 62). CONCLUSIONS: The pterional approach has allowed the achievement of GTR for jugum sphenoidale meningioma with protective odds from recurrence risk.

Dermoid cyst, unusual location of the pterion: About a case and review of literature.

Intracranial dermoid cysts are benign tumors of congenital origin. The intradiploic forms are rare, exceptional at the pterional level. We report the case of a 10-year-old girl who presented with a cutaneous fistula in the left frontotemporal region. The blind end of the fistula was an intradiploic dermoid cyst in the rare location of the pterion confirmed by imaging and histopathology. Once this lesion is suspected, it is important to identify its location and morphology using imaging techniques and to complete excision of the cyst to avoid complications associated with infection and to mitigate the risk of subtotal resection.

Evaluation of unruptured aneurysm scoring systems and ratios in subarachnoid hemorrhage patients with multiple intracranial aneurysms.

Background: This study aims to appraise aneurysm scores and ratios' ability to discriminate between ruptured aneurysms and unruptured intracranial aneurysms (UIAs) in subarachnoid hemorrhage (SAH) patients harboring multiple intracranial aneurysms (MICAs). We, then, investigate the most frequent risk factors associated with MICAs. Methods: We retrospectively applied unruptured intracranial aneurysm treatment score (UIATS) and population hypertension age size of aneurysm earlier SAH from another aneurysm site of aneurysm (PHASES) score, aspect, and dome-to-neck ratio to the 59 consecutive spontaneous SAH patients with MICAs admitted between January 2000 and December 2015 to the Department of Neurosurgery of the University Hospital Center "Hôpital des Spécialités" of Rabat (Morocco). Patients with at least two intracranial aneurysms (IAs) confirmed on angiography were included in the study. Results: Fifty-nine patients were harboring 128 IAs. The most frequent patient-level risk factors were arterial hypertension (AHT) 30.5 % (n = 18) and smoking status 22.0 % (n = 13). A PHASES score recommended treatment in 52 of 60 ruptured aneurysms and in six of 68 UIAs with a sensitivity of 31.67% and a specificity of 76.47%. UIATS recommended treatment in 26 of 62 ruptured aneurysms and in 35 of 55 UIAs with a sensitivity of 41.9% and a specificity of 63.6%. Aspect ratio recommended treatment in 60 of 60 ruptured aneurysms and in 63 of 68 UIAs with a sensitivity of 100% and a specificity of 88.24%. Dome-to-neck ratio recommended treatment in 45 of 60 ruptured aneurysms and in 48 of 68 UIAs with a sensitivity of 80% and a specificity of 63.24%. The aspect ratio (area under the curve [AUC] = 0.953) AUC > 0.8 has a higher discriminatory power between ruptured aneurysms and UIAs. Conclusion: AHT and smoking status were the most common risk factors for intracranial multiple aneurysms and the aspect ratio and PHASES score were the most powerful discrimination tools between ruptured aneurysms and the UIAs.

Outcome of Brain Metastases Treated with Gamma Knife Stereotactic Radiosurgery: A Retrospective Cohort Study of 205 Cases.

BACKGROUND: Brain metastases (BMs) are the most common expansive intracranial lesions in adults. Approximately 50% of patients diagnosed with new BMs will have >1 BM at the diagnosis. We report our experience with BMs treated with Leksell Gamma Knife stereotactic radiosurgery (GKSR) and evaluate the outcomes. METHODS: Patients treated by GKSR in our institution between 2008 and 2021 for BMs were evaluated retrospectively. RESULTS: A total of 205 patients (56.6% women) were included, with a median age of 59 years (range, 25-83 years). The breast (n = 85; 42.5%) and lung (n = 76; 38%) were the most common original locations for the primary tumors. Of the 205 patients, 103 (50.3%) had a single BM and 102 (49.7%) had ≥2 BMs. The median number of multiple BMs treated was 4 (range, 2-43). The mean overall survival (OS) time was 6.00 months (95% confidence interval [CI], 5.07-6.93 months) for all BMs. The median rate of tumor control after radiosurgery was 65% (range, 20%-99%) during a median follow-up of 6.00 months (95% CI, 3-84 months). In the overall population, the 1-, 2-, and 5-year OS rate was 37.55%, 25.12%, and 18.51%, respectively. The mean OS time was longer for those with multiple BMs than for those with a single BM (mean, 10 months [95% CI, 6.67-13.33 months] vs. mean, 5 months [95% CI, 4.21-5.70 months]; P = 0.03). Retreatment, tumor stability (control), and progression influenced the OS of patients with BMs, whether single or multiple (P < 0.001). CONCLUSIONS: GKSR provides good results in terms of OS and better quality of life for patients with BMs, whether single or multiple. Tumor stability and retreatment influenced the OS of patients with BMs.

Spinal intradural epidermoid cyst: Case report.

Spinal epidermoid cysts are rare benign tumors. The etiology can be acquired or congenital. We present a rare case of an 18-month-old girl presented 4 months ago with spontaneous intergluteal swelling fistulized to the skin and lower limbs weakness. Magnetic resonance imaging of the spine demonstrated an intradural tumor from L3 to L5 levels, isointense on T1, hyperintense on T2-weighted images with contrast enhancement after gadolinium injection without any coexistent spinal dysraphism suggested the diagnosis of the dermal sinus. The patient underwent triple-level laminectomy for biopsy and tumor resection. A pearly white tumor was encountered, with a subsequent biopsy confirming it to be an epidermoid tumor. At 6-month follow-up, the neurologic deficit was improved. Spinal epidermoid cysts are rare tumors that evolve slowly. Complete total removal is the treatment of choice.

Continental Survey of Access to Diagnostic Tools and Endovascular Management of Aneurysmal Subarachnoid Hemorrhage in Africa.

Rationale: Interventional neurovascular procedures are effective in lowering the burden of mortality and complications resulting from aneurysmal subarachnoid hemorrhage (aSAH). Despite the wide uptake of interventional neurovascular procedures in high-income countries, access to care in low- and middle-income countries remains limited, and little is known about accessibility in Africa. In this survey, we decided to assess access to diagnostic tools and treatment of aSAH in Africa. Methodology: A Google form e-survey was distributed to African neurosurgery centers accepting responses from January 4th to March 21st 2021. Data on accessibility to diagnostic tools, treatment methodologies, and interventional neuroradiology personnel in African centers were collected. Ninety five percent confidence intervals were computed for each variable. Results: Data was received from 36 neurosurgical centers in 16 African countries (16/54, 30%). Most centers were public institutions. Ninety four percent of the centers had the necessary resources for a lumbar puncture (LP) and a laboratory for the diagnosis of aSAH. Most centers had at least one computed tomography (CT) scanner, 81% of the centers had access to CT angiography and some had access to conventional angiography. Forty seven percent of the centers could obtain a head CT within 2 h of presentation in an emergency. Sixty one percent of centers provided clipping of intracranial aneurysms whilst only 22% of centers could perform the endovascular treatment. Sixty four percent of centers did not have an endovascular specialist. Conclusion: This survey highlights health inequity in access to endovascular treatment for aSAH. Lack of diagnostic tools to identify an aneurysm and a shortfall of qualified endovascular specialists are prime reasons for this. Our findings can inform health system strengthening policies including the acquisition of equipment and capacity building in Africa.

Bioinformatics Analysis of Differentially Expressed Genes and miRNAs in Low-Grade Gliomas.

Low-grade glioma is the most common type of primary intracranial tumor. In the last 3 years, new observations of molecular precursors in adults with gliomas have led to a modification in the histopathologic classification of these brain tumors. Among the biomarkers that have been highlighted, we have the micro RNAs (miRNAs) which play a crucial role in the regulation of gene expression and the long noncoding RNAs (lncRNAs) controlling various cellular and metabolic pathways. In our study, large-scale data on sequenced RNA and miRNAs from 516 patients were obtained from the Cancer Genome Atlas database by the TCGAbiolinks package. We identified the differential expression of miRNAs and genes using the Limma package and then we used the ClusterProfiler package for annotations of the biological pathways of the expressed genes, the survival package to estimate the survival analysis, and the GDCRNATools package to determine miRNAs-genes and miRNAs-lncRNAs interactions. We obtained a significant correlation between the miRNAs identified and the overall survival of the patients (log-rank P < .05) and we have theoretically proposed a novel network of miRNAs involved in low-grade gliomas, specifically astrocytomas and oligodendrogliomas, which combine both genes and lncRNAs.

Computational Analysis of IDH1, IDH2, and TP53 Mutations in Low-Grade Gliomas Including Oligodendrogliomas and Astrocytomas.

INTRODUCTION: The emergence of new omics approaches, such as genomic algorithms to identify tumor mutations and molecular modeling tools to predict the three-dimensional structure of proteins, has facilitated the understanding of the dynamic mechanisms involved in the pathogenesis of low-grade gliomas including oligodendrogliomas and astrocytomas. METHODS: In this study, we targeted known mutations involved in low-grade gliomas, starting with the sequencing of genomic regions encompassing exon 4 of isocitrate dehydrogenase 1 (IDH1) and isocitrate dehydrogenase 2 (IDH2) and the four exons (5-6 and 7-8) of TP53 from 32 samples, followed by computational analysis to study the impact of these mutations on the structure and function of 3 proteins IDH1, IDH2, and p53. RESULTS: We obtain a mutation that has an effect on the catalytic site of the protein IDH1 as R132H and on the catalytic site of the protein IDH2 as R172M. Other mutations at p53 have been identified as K305N, which is a pathogenic mutation; R175 H, which is a benign mutation; and R158G, which disrupts the structural conformation of the tumor suppressor protein. CONCLUSION: In low-grade gliomas, mutations in IDH1, IDH2, and TP53 may be the key to tumor progression because they have an effect on the function of the protein such as mutations R132H in IDH1 and R172M in IDH2, which change the function of the enzyme alpha-ketoglutarate, or R158G in TP53, which affects the structure of the generated protein, thus their importance in understanding gliomagenesis and for more accurate diagnosis complementary to the anatomical pathology tests.

Endoscope-assisted keyhole surgery via an eyebrow incision for removal of large meningiomas of the anterior and middle cranial fossa.

BACKGROUND: Conventional open surgery of large meningiomas has proven to be challenging even in experienced hands. Intense retraction and dissection around neurovascular structures increase morbidity and mortality. In the present study, we retrospectively analyzed the surgical technique, and outcome in 40 patients with large anterior cranial fossa meningiomas extending to the middle fossa. All patients were approached via a supraorbital mini craniotomy. METHODS: It is a retrospective study of 40 patients (12 males, 28 females) who underwent surgery for large anterior cranial fossa meningiomas (diameter >5 cm) extending to the middle fossa in four different neurosurgical centers within 6 years. Depending on the localization of the tumor, the skin incision was between 2.5 and 3 cm long and was made without shaving the patient's eyebrow hair. Subsequently, a keyhole craniotomy was performed of approximately 0.8×1.2-1.4 cm in diameter. Preoperative and postoperative clinical and radiological data were analyzed and discussed. RESULTS: Headache and psycho-organic syndrome were the most common presenting symptom in all patients. Presenting symptoms were associated with psychological changes in 23 cases, visual impairment in 19 patients, and anosmia in 17 patients. In overall, 36 of 40 patients (90%) showed a good outcome and returned at long-term follow-up to their previous occupations. The elderly patients returned to their daily routine. CONCLUSION: With the appropriate keyhole approach as a refinement of the classic keyhole craniotomy to a smaller key"burr"hole, and with use of modern and new designed equipment, it is possible to perform complete resection of large anterior and middle fossa meningiomas with the same safety, efficiency and with less complication rates as described in the literature for large meningiomas even performed with classic keyhole craniotomies.

A sacral hydatid cyst mimicking an anterior sacral meningocele.

Hydatid disease is a zoonosis caused by Echinococcus granulosus. It is a progressive disease with serious morbidity risks. Sacral hydatid disease is very uncommon, accounting for < 11% of spinal hydatidosis cases. The diagnosis of a sacral hydatid cyst is sometimes difficult because hydatidosis can simulate other cystic pathologies. The authors report on 9-year-old boy admitted to their service with a paraparesis that allowed walking without aid. The boy presented with a 2-year history of an evolving incomplete cauda equina syndrome as well as a soft cystic mass in the abdomen extending from the pelvis. Radiological examination revealed an anterior meningocele. A posterior approach with laminectomy from L-5 to S-3 was performed. Three lesions with classic features of a hydatid cyst were observed and removed. The diagnosis of hydatid cyst was confirmed histopathologically. Antihelmintic treatment with albendazole (15 mg/kg/day) was included in the postoperative treatment. The patient's condition improved after surgery, and he recovered normal mobility. The unusual site and presentation of hydatid disease in this patient clearly supports the consideration of spinal hydatid disease in the differential diagnosis for any mass in the body, especially in endemic areas.

Multiple cerebral and spinal cord cavernomas in Klippel-Trenaunay-Weber syndrome.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome in which patients harbor cutaneous hemangiomas, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. The clinical presentation of this syndrome is variable and the etiopathogenesis is presumably genetic in view of recent discoveries of RASA1 gene mutations in KTWS patients. Similarly, the KRIT1 gene is involved in pathogenesis of cavernous angiomas. Both RASA1 and KRIT1 genes interact with Rap1a protein, a member of the Ras family of guanosine triphosphatases (GTPases) signalling cellular adhesion. We report a 55-year-old male with KTWS harboring multiple cavernous angiomas in the thoracic spinal cord and the brainstem, as revealed by MRI. Angiography ruled out arteriovenous malformation. The patient was managed conservatively. The rarity of cavernous angiomas in KTWS and the possibilities of shared genetic pathways between KTWS and cavernous angiomas are discussed.

Intradural cervical inflammatory pseudotumor mimicking epidural hematoma in a pregnant woman: case report and review of the literature.

BACKGROUND: Inflammatory pseudotumors usually affect the lung and the orbit. They occur extremely rarely in the spine. We encountered a case of intradural extramedullary IPT of the cervical spine in a pregnant woman that initially presented as an epidural hematoma in MRI. CASE DESCRIPTION: This is a case of a 30-year-old, HIV-negative, full-term pregnant lady presenting with 2-month history of progressive quadriparesis with rapid worsening of power in all 4 limbs for 3 days. Magnetic resonance imaging scanning revealed cervical extramedullary compressive lesion likely to be epidural bleed from its imaging characteristics. Intraoperatively, intradural extramedullary granulomatous lesion was found with dural thickening. Gross total excision was accomplished; histopathologic study assessed an IPT. The patient improved after surgery excision and is doing well at 6-month follow-up with remarkable neurological recovery. CONCLUSION: Cervical intradural IPT is a very rare issue in the spine. Diagnosis can be confused with a neoplastic lesion like an "en plaque meningioma" or as epidural bleed like in the present case.

Management of intracranial arachnoid cysts: institutional experience with initial 32 cases and review of the literature.

Surgical indications and modalities in treatment of intracranial arachnoid cysts still remain controversial owing to limited understanding of the pathophysiologic mechanisms and natural history of this pathology. Current literature favours endoscopic interventions for arachnoid cysts. We retrospectively reviewed 32 intracranial arachnoid cysts managed over 11-year period in our institution. Post-therapeutic results were clinically and radiologically assessed. Supratentorial location of cysts was noted in 75% cases (n=24) while 25% cysts were located infratentorially (n=8). The mean cyst size was 54mm in largest dimension (range 10-100mm; median 50mm). Hemiparesis was noted in 37% cases, raised intracranial pressure and seizures in 34% cases each, while cranial nerve dysfunction was noted in 16% cases. Seventy five percent cases were surgically managed: excision and marsupialization was done in 53% cases (n=17), stereotactic aspiration in 12.5% cases (n=4), endoscopic fenestration in 6.25% cases (n=2) and cystoperitoneal shunting initially in 1 case (3%) and after recurrence of primarily excised cysts in 2 cases. Conservative treatment with regular clinical and imaging control was done in 25% cases (n=8). The mean follow-up was 72 months (range: 12-108 months). Good outcome was noted in 72% cases, 16% cases remained unchanged while only one case with giant suprasellar cyst worsened. With excision and marsupialization, 65% of cysts reduced in size (n=11/17), 17% cysts resolved completely (n=3/17). The overall recurrence rate was 29%. Surgery excision and marsupialization of symptomatic cases provided good results.