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Clear cell meningiomas are a rare histological subtype of World Health Organization (WHO) grade II meningioma. Despite its relatively low frequency, clear cell meningioma has attracted considerable attention because of its unique pathological characteristics, clinical behavior, and challenging management considerations. The purpose of our systematic review is to provide clinicians with a better understanding of this rare disease. PubMed was searched for articles in the English language published from 1988 to 2023 June. The keywords were as follows: "clear cell meningioma," "clear cell" and "meningioma." We analyzed clinical manifestations, radiological manifestations, pathological features, comprehensive treatment strategies, and prognosis to determine the factors influencing recurrence-free survival (RFS). Recurrence-free survival curves of related factors were calculated by the Kaplan‒Meier method. The log-rank test and Cox univariate analysis were adopted to assess the intergroup differences and seek significant factors influencing prognosis and recurrence. Fifty-seven papers met the eligibility criteria, including 207 cases of clear cell meningioma (CCM), which were confirmed by postoperative pathology. The fifty-seven articles involved 84 (40.6%) males and 123 (59.4%) females. The average age at diagnosis was 27.9 years (range, 14 months to 84 years). Among the symptoms observed, headache, neurologic deficit, and hearing loss were the most commonly reported clinical manifestations. Most tumors (47.8%) were located in the skull base region. Most tumors showed significant enhancement, and homogeneous enhancement was more common. A total of 152 (74.1%) patients underwent gross total resection (GTR), and 53 (25.9%) patients underwent subtotal resection (STR). During the follow-up, the tumor recurred in 80 (39.4%) patients. The log-rank test and the Cox univariate analysis revealed that tumor resection range (GTR vs. STR) and adjuvant treatment (YES vs. NO) were significant predictors of recurrence-free survival (RFS). Clear cell meningioma is a rare type of meningioma with challenging diagnosis and therapy. The prognosis of this disease is different from that of regular meningiomas. Recurrence remains a possibility even after total tumor resection. We found that the surgical resection range and adjuvant treatment affected the recurrence period. This finding provides significant guidance for the treatment of clear cell meningioma. [ABSTRACT FROM AUTHOR]

A group of structures in the human central nervous system (CNS) represents a noteworthy dilemma for the neuroscientist, particularly to the neuroanatomist. In this paper an attempt is made by extensive review of the literature to give an account of the significance of the subcommissural organ (SCO) in humans and its possible relationship with cerebrospinal fluid (CSF) disorders. The subcommissural organ is a gland located in the diencephalic plate caudal to the pineal organ that covers the anterior part of the posterior commissure. Histologically, it is a highly differentiated ependyma. After birth, the SCO undergoes regressive changes, and in the adult human only remnants of the specialized SCO cells can be found. The Reissner's fiber (RF) may be regarded as a pure secretory product of the SCO. Only a few vertebrate species have been reported to lack an RF, namely the bat, camel, chimpanzee, and man. Nonetheless, a successful immunoreaction against a proteinaceous compound of the fetal human SCO has been performed. Recently, new interest was elicited regarding SCO and its possible implication in the pathogenesis of hydrocephalus. The objective of this review is to bring into consideration the relevance of the SCO to the neurosurgical scenario. [ABSTRACT FROM AUTHOR]

Pediatric CNS cavernomas still are diagnostically and therapeutically challenging lesions. With the help of magnetic resonance imaging, the natural history of cavernomas now guiding therapeutic strategies is well documented in adults but remains poorly known in the pediatric age group, since most previous studies dealt with adult and pediatric patients together. This paper focuses on clinical, imaging, and therapeutic features and differential diagnosis of CNS cavernomas with an emphasis on their specificities in the pediatric age group. It is based upon a critical review of the literature and our single-center experience with 36 children (35 with cerebral cavernomas and one with spinal cord cavernoma) operated on during the period of 1985–1999 as well as with seven additional unoperated pediatric cases. Our experience resembles that of other authors regarding the high hemorrhagic risk in children compared to adults. These angiographically occult vascular malformations are often revealed by the sudden onset of intracerebral hematoma with acute focal neurologic deficits, concomitant manifestations, and/or signs of raised intracranial pressure. True epilepsy is less common and may be related to chronic or recurrent microbleeding. Evocative imaging findings are also somewhat different in the two age groups, and we propose here an imaging classification of cerebral cavernomas based on both morphological and signal characteristics that is applicable to the pediatric age group. A sharply demarcated spherical intracerebral hematoma or heterogeneous lesion should always make one consider the hypothesis of a cavernoma. For symptomatic lesions and most rapidly growing asymptomatic lesions, the treatment of choice is complete microsurgical excision preceded by careful anatomical and functional evaluation. Improvements in surgical techniques and anesthesiology over recent years have brought good results in most operated children. The limited role of radiosurgery in the management of pediatric cerebral cavernomas is discussed. There is still a need for well-conducted specific evaluation of the natural history of these lesions in the pediatric age group to aid in systematic research, follow-up, and therapeutic strategies for asymptomatic cavernomas. [ABSTRACT FROM AUTHOR]

No standard treatment protocol to guide the management of the primary central nervous system atypical teratoid rhabdoid tumors (ATRTs). To evaluate the efficacy of GTR (gross total resection), RT (radiotherapy), CCMT (conventional chemotherapy), or intensified chemotherapy (ICMT) and verify the optimal treatment strategy. A total of 501 cases (18 cases from our center and 483 cases from published literature) were eligible for analysis. Clinical characteristics were reviewed, and overall survival (OS) of each combined treatment modality was compared. These prior publication data were processed according to PRISMA guidelines. This study included 265 (52.9%) males and 216 (43.1%) females. The median age of the cohort was 2.2 years with 295 (58.9%) cases younger than 3 years. GTR was achieved in 217 (43.3%) patients. Initial adjuvant CCMT, CCMT plus intrathecal chemotherapy (ITCMT), CCMT plus high-dose chemotherapy (HDCMT), and CCMT plus ITCMT and HDCMT were administered in 228 (45.5%), 78 (15.6%), 55 (11.0%), and 24 (4.8%) patients, respectively. Radiotherapy (RT) was prescribed in 266 (53.1%) patients. Fewer patients younger than 3 years old received RT (21.9% vs 33.0%, p < 0.001, chi-square test). The OS of the entire cohort at 1, 3, and 5 years were 56.6, 35.9, and 30.8%, respectively. After adjusting for age and sex, GTR (HR 0.630; p < 0.001), RT (HR = 0.295; p < 0.001), CCMT (HR = 0.382; p < 0.001), and ICMT (HR = 0.209; p < 0.001) were independent prognostic factors. The 3-year OS of surgery alone, surgery plus CCMT, surgery plus RT, surgery plus ICMT, surgery plus CCMT and RT, and surgery plus ICMT and RT were 8.9, 13.4, 23.7, 37.4, 48.3, and 68.5%, respectively. When taking into consideration the extent of tumor resection (n = 462), GTR followed by RT, CCMT, intrathecal chemotherapy, and high-dose chemotherapy provided the best OS (5-year OS 88.2%). In younger children, adjuvant ICMT had a greater 3-year OS than adjuvant RT alone (34.0% vs 0%, p =.001). This study identified independent favorable predictors for OS of ATRTs and distinguished significantly different OS following various treatment modalities. If tolerable, intensive treatment with GTR followed by adjuvant RT and ICMT is recommended. Intensified CCMT could be an alternative to avoid radiological radiotoxicity for younger children CRD42018098841. [ABSTRACT FROM AUTHOR]

The incidental diagnosis of both developmental venous anomalies (DVAs) and cavernous malformations (CMs) in the central nervous system is increasing with improved imaging techniques. While classically silent diseases, these cerebrovascular pathologies can follow an aggressive course, particularly when present in the brainstem. In the last decade, substantial research has focussed on KRIT1-mediated tight junction gene expression and their role in CM development. However, our understanding of the physiologic conditions precipitating symptomatic CM development or CM haemorrhage with and without concomitant DVAs, remains lacking. The only established risk factor for CM haemorrhage is a previous history of haemorrhage, and literature currently reports trauma as the only precipitant for symptomatic events. While plausible, this occurs in a minority, with many patients experiencing occult events. This manuscript presents a hypothesis for symptomatic CM events by first discussing the anatomical pathways for intracranial venous outflow via the internal jugular veins (IJV) and vertebral venous plexus (VVP), then exploring the role of venous flow diversion away from the IJVs under physiologic stress during dynamic postural shift. The resultant increase in intracranial venous pressure can exacerbate normal and pre-existing structural DVA pathologies, with repeated exposure causing symptomatic or CM-inducing events. This pathophysiological model is considered in the context of the role of the autonomic nervous system (ANS) in postural intracranial venous outflow diversion, and how this may increase the risk of DVA or CM events. It is hoped that this hypothesis invokes further investigation into precipitants for DVA or CM events and their sequela and, also, furthers the current knowledge on pathophysiological development of DVAs and CMs. [ABSTRACT FROM AUTHOR]

We conducted a systematic review of 72 studies to characterize trilateral retinoblastomas. Kaplan-Meier analysis was used to estimate survival, and statistical significance was assessed by using a log-rank test. We analyzed 211 cases of trilateral retinoblastomas. The average age of onset of retinoblastoma was 0.79 ± 1.38 years, and the average latency period between the onset of retinoblastomas and trilateral retinoblastomas was 1.49 ± 1.76 years. The brain tumors were found before the retinoblastoma diagnosis in 6 cases (3.1%), concurrently in 61 cases (32.1%), and after the retinoblastoma diagnosis in 123 cases (64.7%). Pineal tumors were found in 155 cases (73.4%) and sellar tumors in 46 cases (21.8%). The overall median survival was 10.3 months (95% CI, 8.5-13) and the 5-year survival rate was 15.7%. Central nervous system symptoms were variable and associated with shorter survival in univariate and multivariate analyses. The survival time in patients who received high-dose chemotherapy with stem cell transplant was significantly longer (p = 0.0067) than that of with or without conventional chemotherapy. Twelve long-term survivors were reported, and of these, six patients were treated with high-dose chemotherapy with stem cell transplant and six patients were treated with conventional chemotherapy. It is important that survivors continue to undergo regular medical surveillance in order to detect trilateral retinoblastoma at a potentially curative stage. Trilateral retinoblastoma patients with an irradiation history had shorter survival than those without irradiation history for retinoblastoma. High-dose chemotherapy should be considered as a potential treatment option for trilateral retinoblastomas. [ABSTRACT FROM AUTHOR]

Glioblastoma (GBM) are high-grade gliomas that severely impact on overall survival (OS). GBM cell motility and the breakdown of the blood-brain barrier could favor GBM cell communication with the systemic circulation. In spite of this, extracranial GBM metastases are rare. Here, we describe two YKL-40-positive GBM patients with extra-CNS (central nervous system) metastases, and we present a meta-analysis of 94 cases. The analysis concluded that extra-CNS metastases occurred 8.5 months after first GBM diagnosis and OS was 12 months; surgical GBM excision was associated at a longer interval to extra-CNS metastasis than biopsy only, and even longer if followed by radiotherapy and chemotherapy. Both our case reports were adult males who developed extra-CNS, YKL-40-positive metastases at lymph nodes, lung and subcutaneous sites, after 86 and 24 months from initial diagnosis of GBM. At first GBM local recurrence, they were treated with bevacizumab (BV), an anti-vascular endothelial growth factor antibody. They died after 4 and 1 month from the occurrence of metastases. Both cases expressed YKL-40 and lacked EGFR amplification, suggesting a mesenchymal phenotype, and maintained such profile at extra-CNS recurrence; they did not show MGMT promoter methylation, IDH1/2 mutations, or c-Met upregulation. Our two cases and the meta-analysis support the idea that prolonged survival of GBM patients increases the probability of GBM cells shedding to lymphatic and hematic system. Interestingly, the present two cases showed the features of mesenchymal profile, usually related with worst prognosis that was maintained in extracranial metastases. [ABSTRACT FROM AUTHOR]

Gliomas are the most common primary brain tumors in adults and, despite advances in the understandings of glioma pathogenesis in the genetic era, they are still ineradicable, justifying the need to develop more reliable diagnostic and prognostic biomarkers for this malignancy. Because changes in cerebrospinal fluid (CSF) are suggested to be capable of sensitively reflecting pathological processes, e.g., neoplastic conditions, in the central nervous system, CSF has been deemed a valuable source for potential biomarkers screening in this era of proteomics. This systematic review focused on the proteomic analysis of glioma CSF that has been published to date and identified a total of 19 differentially expressed proteins. Further functional and protein-protein interaction assessments were performed by using Protein Analysis Through Evolutionary Relationships (PANTHER) website and Ingenuity Pathway Analysis (IPA) software, which revealed several important protein networks (e.g., IL-6/STAT-3) and four novel focus proteins (IL-6, galanin (GAL), HSPA5, and WNT4) that might be involved in glioma pathogenesis. The concentrations of these focus proteins were subsequently determined by enzyme-linked immunosorbent assay (ELISA) in an independent set of CSF and tumor cyst fluid (CF) samples. Specifically, glioblastoma (GBM) CF had significantly lower GAL, HSPA5, and WNT4 levels than CSF from different grades of glioma. In contrast, IL-6 level was significantly higher in GBM CF when compared with CSF and, among different CSF groups, was highest in GBM CSF. Therefore, these candidate protein biomarkers, identified from both the literatures and in silico analysis, may have potentials in clinical diagnosis, prognosis evaluation, treatment response monitoring, and novel therapeutic targets identification for patients with glioma. [ABSTRACT FROM AUTHOR]

Gliomatosis cerebri (GC) is a rare fatal glial neoplasm of the central nervous system. Neuroimaging, histological, and clinical outcome data were reviewed of 17 consecutive patients, 8 males and 9 females aged 15-68 years (median, 37 years), treated for GC between April 1992 and October 2007. All patients received cranial irradiation to include the hyperintense area on T2-weighted magnetic resonance imaging. The total dose of the radiotherapy was 50-72 Gy (median, 60). Intravenous nimustine hydrochloride was administered in all patients, combined with temozolomide in four patients. The median survival time was 23.3 months, with a median follow-up of 23.3 months. Kaplan-Meier analysis demonstrated the overall survival rate which was 70.6% for 1 year, 23.5% for 3 years, and 17.7% for 5 years. Spinal enhanced lesions and nodular malignant transformation to glioblastoma were observed during follow-up in two patients each. Poor survival showed correlation with higher Ki-67 labeling index, higher choline/ N-acetylaspartate ratio on magnetic resonance spectroscopy, tumor volumes, lower Karnofsky performance status on admission, cognitive/behavioral deterioration, poor response to the initial radiochemotherapy, and emergence of paraventricular enhanced lesions during the clinical course. The prognosis for patients with GC is unfavorable, but radiochemotherapy may prolong survival. [ABSTRACT FROM AUTHOR]

A retrospective study on solid central nervous system haemangioblastomas was performed to characterize clinical features, treatment strategies and outcome in these rare lesions. Between 1993 and 2006 23, solid haemangioblastomas were surgically removed in 17 patients. Eight lesions were located within pons Varolii and medulla oblongata, six within the cerebellar hemispheres and three in the cerebellopontine angle. Three haemangioblastomas were located supratentorially and three within the spinal cord. All patients except two underwent pre-operative magnetic resonance imaging (MRI). Post-operative digital subtraction angiography and/or MRI was performed in all surviving patients. Except for spinal cord lesions, rather unsystematic clinical symptoms were observed. Twenty-two tumours could be resected completely. Two patients with brainstem lesions died within 10 weeks after surgery from infectious complications. Persistent new neurological deficits occurred in two patients. Three patients underwent radiosurgery prior to or following the surgical procedure. Solid haemangioblastomas represent a surgical challenge due to their arteriovenous malformation-like vascularisation and their frequent location in eloquent areas. Surgery is the therapy of choice. Circumferential dissection with devascularization and en bloc removal yields good functional results. A location within the brainstem carries the most unfavourable prognosis. [ABSTRACT FROM AUTHOR]

Central nervous system (CNS) germ cell tumors constitute a unique class of rare tumors that mainly affect children and adolescents. These tumors are believed to originate from displaced primordial germ cells. Recently, results of treatment of germ cell tumors have improved with use of radiotherapy and combination chemotherapy. However, some tumors have proven refractory to intensive treatment with surgery, radiation, and combination chemotherapy. Nestin is an intermediate filament protein expressed in undifferentiated cells during CNS development and in CNS tumors and is used as a marker of immature elements of tumors, including brain tumor stem cells. In this study, we examined for the first time nestin expression in 19 CNS germ cell tumors (nine pure germinomas, five germinomas with syncytiotrophoblastic giant cells, one yolk sac tumor, one choriocarcinoma, one embryonal carcinoma, and two mature teratomas). Nestin was expressed in 14 cases but was not expressed in three pure germinomas and two mature teratomas. Clinically, nestin-negative tumors did not exhibit dissemination, while all tumors that exhibited dissemination also strongly expressed nestin protein. These findings suggest that the detection of nestin expression could be useful in the management of CNS germ cell tumors, as an auxiliary predictor of dissemination and/or progression. [ABSTRACT FROM AUTHOR]

Pyogenic infections of the central nervous system of dental origin are quite uncommon in industrialized countries. We report six cases with intracerebral ( n=4) and intraspinal ( n=2) infections treated in our hospital. The microbial pathogen was successfully isolated in all patients. Fusobacterium nucleatum as well as Streptococcus species were found in three cases. Bacillus species were identified in two patients. Actinomyces was the etiologic agent in one case. All patients suffered from dental pathologies, so that after clinical and radiological exclusion of other sources an oral focus was presumed. Therapeutic management consisted of an operative procedure in order to obtain decompression, as well as evacuation of the pus on the one hand, followed by targeted antibiotics on the other. Clinical improvement was achieved in all patients, with one patient lost to follow-up. On magnetic resonance tomography, the inflammatory changes also disappeared in all cases. We recommend that oral infection with recurrent bacteraemia should always be considered in the pathogenesis of the so-called “cryptic” intracerebral and intraspinal infections. [ABSTRACT FROM AUTHOR]

Spinal cord cavernous malformations (SCCM), once thought to be extremely rare, have been diagnosed more frequently since the advent of MRI. In addition to the six personal cases surgically treated between 1992 and 1993 and already described in the literature, the authors report here a further ten cases operated on between April 1993 and January 2001. These involved five males and five females whose ages at operation ranged from 12 to 69 years. The SCCM were thoracic in five patients and cervical in five. In seven cases, the malformations bulged on the surface of the pia mater, while in the other three there was subpial discoloration due to the presence of hemosiderin. Removal was total in all cases. After surgery, two patients presented transient worsening of preoperative paraparesis. At follow-up ranging from 2.2 to 9.2 years (mean 5.7), nine patients had made complete motor recovery while one, in whom preoperative paraparesis had been severe, remained stationary. At least one follow-up MRI investigation was performed in each patient 6 to 12 months after operation. As previously reported, the authors confirm that the treatment of choice for symptomatic SCCM is total surgical excision in order to avoid recurrences and the possibility of further hemorrhage. Surgical outcome combines low mortality with a high probability of functional recovery, especially when paraparesis is not severe and of relatively recent onset. [ABSTRACT FROM AUTHOR]

Tuberculosis (TB) of the spine (Pott’s disease) is both the most common and most dangerous form of TB infection. Delay in establishing diagnosis and management cause spinal cord compression and spinal deformity. This study investigated the data on all cases of Pott’s disease reported in Turkey from 1985 to 1996. A total of 694 cases were included. Out of the patients evaluated, 19% were reported in the first half of the period (1985–1990) and 81% in the second half (1991–1996). Tuberculosis affecting the spine was commonly localized in the thoracic region and involved the vertebral body. The presenting symptoms were leg weakness (69%), gibbus (46%), pain (21%), and palpable mass (10%). Decompressive surgery plus anti-TB chemotherapy remains the best mode of therapy for Pott’s disease. Follow-up information was available in 414 of the 694 patients and there were ten deaths (2%), one occurring intraoperatively and the other nine postoperatively. This meta-analysis demonstrates that in Turkey Pott’s disease remains a serious problem, causing paraplegia. It should be considered when patients present with neurological findings suggesting spinal cord compression and spinal deformity. In the present study, it was concluded that the neurological involvement due to Pott’s disease is relatively benign if urgent decompression is performed at the onset of the disease. [ABSTRACT FROM AUTHOR]

Von Hippel-Lindau disease (VHL) is a hereditary cancer syndrome caused by germline mutations of the VHL tumor suppressor gene. Major progress has been made in the last decade in both clinical and fundamental aspects of VHL. The VHL gene product, pVHL, has major and multiple functions: pVHL regulates not only first angiogenesis but also extracellular matrix formation and the cell cycle. A molecular diagnosis of VHL is now available, leading to a transformation in clinical management of patients and their families. Diagnosis of VHL has to be suspected in patients with a VHL-related tumor without familial history and especially in case of hemangioblastoma or endolymphatic sac tumors. Such patients should be systematically investigated for clinical and molecular evidence of VHL disease. Treatment of symptomatic hemangioblastomas remains mainly neurosurgical, often in emergency, but stereotactic radiosurgery is emerging as an alternative therapeutic procedure. In the future, antiangiogenic drugs could represent a potential medical treatment of CNS hemangioblastomas in view of their highly vascular structure. Lastly, visceral manifestations of VHL disease are also of critical importance and require early detection for effective treatment. [ABSTRACT FROM AUTHOR]

Intraparenchymal location of schwannomas in the central nervous system (CNS) is rare. Occasional cases involving the cerebrum, cerebellum, brain stem, and spinal cord have been reported. We report here an additional case of thoracic intramedullary schwannoma in a 42 year old woman. The literature concerning intraparenchymal schwannomas in the CNS is reviewed and discussed. [ABSTRACT FROM AUTHOR]

Extra-axial cavernous malformations (cavernomas) of the central nervous system are rare. Although occasional cases located in different parts of the central nervous system have been reported, only five cases of extra-axial cavernous malformation in the cerebellopontine angle are to be found. We describe here two additional cases of cavernomas in the cerebellopontine angle causing hearing loss and tinnitus presenting as vestibular schwannoma. [ABSTRACT FROM AUTHOR]