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We discuss an extremely rare case of low-grade Schwann cell leptomeningeal neoplasm with no evident intradural primary, presenting with rapid neurological decline leading to death reflecting the aggressive biological behaviour of this entity despite its low-grade morphology. Notwithstanding extensive investigations, the diagnosis was only established on autopsy as clinical presentation is non-specific making diagnosis challenging. This condition could be considered in patients presenting with leptomeningeal disease if initial workup of more common causes is non-revealing.

An 8-year-old girl presented with precocious puberty and an otherwise normal examination. MRI of the pituitary was normal; however, an incidental oval, non-enhancing T2 hyperintense lesion in the right temporal subcortical white matter was identified ([figure 1][1]). The neuro-radiographic

Sacral spinal cord ependymoma is an uncommon pathology. Most of the reported cases are consistent with a myxopapillary ependymoma histopathologic subtype. Non-myxopapillary ependymomas rarely occur in the sacral region. Most lesions are intradural; however, rare extradural cases can occur. We present the case of a 46-year-old female patient diagnosed with a grade II sacral extradural ependymoma, emphasising the importance of an interdepartmental case approach for diagnosis and management. Even though grade II ependymomas are considered low grade, the potential for recurrence and metastatic disease has been reported. There are no treatment guidelines for these rare tumours besides gross total resection.

A 6-year-old boy presented for evaluation after a new onset focal seizure. CT demonstrated a large right hemispheric mass with midline shift ([figure 1A][1]). MRI confirmed a T2 hyperintense tumour with reduced diffusivity, minimal enhancement on postgadolinum sequences, and linear calcifications on

A 16‐year‐old boy with negative past medical history presented to an outside hospital with a first time right‐sided focal seizure. Neurologic examination at presentation was normal. CT performed in the emergency room revealed a large left intra‐axial cerebral mass without haemorrhage ([

A 3-year-old boy presented with acute onset of prolonged right sided focal seizures with secondary generalisation. The investigation findings were suggestive of a neoplastic process more than an inflammatory process. Decision to perform brain biopsy from the lesion to establish the precise nature of lesion was undertaken.

We present a 24-year-old man with a 2-year history of progressive right-sided monocular vision loss with no other symptoms. An MRI showed a meningioma compressing the right optic nerve and the cavernous sinus. The tumour was partially resected. Eight days after discharge the patient was admitted with fever, a severe stabbing headache, insomnia, nausea and vomiting. A FilmArray panel and a cerebral biopsy were performed which were positive for herpes simplex virus 1 (HSV-1). An MRI of the brain showed asymmetric bilateral lesions in the frontobasal region with predominance of the right side. Acyclovir was started and continued until completing 21 days. A month after discharge, he started experiencing insomnia, trichotillomania, limb tremor, persistence of abulia, apathy and emotional lability. An HSV-1 encephalitis relapse was suspected, acyclovir and foscarnet were started. Due to the poor response to antiviral therapy CSF was tested, which was positive for anti-NMDA receptor encephalitis. A treatment course of intravenous immunoglobulin was started with a favourable outcome.

Primary leptomeningeal melanomas are rare, comprising less than one percent of all brain tumours. They are aggressive and radioresistant tumours, with a poor prognosis. The mainstay of treatment is complete surgical resection and chemotherapy with limited success. Distinguishing a primary leptomeningeal melanoma from the more common metastatic disease can be difficult, and often requires the use of ancillary molecular testing. Primary central nervous system melanomas, including uveal melanomas, frequently exhibit mutations in GNAQ and GNA11, rare in the cutaneous and mucosal counterparts.A case of a primary leptomeningeal melanoma of the cerebellopontine angle is described. Molecular studies identified a GNA11 p.Q209L and a KIT p.M541L missense variant, with losses of chromosomes 1p and 3p demonstrated with cytogenetic studies. Complete surgical resection was not possible and leptomeningeal metastatic disease rapidly ensued despite immunotherapy. Further understanding of the molecular signature may translate to improved diagnosis, prognostication and development of targeted therapies.

This article characterizes 2 cases of longitudinally extensive transverse myelitis (LETM) that did not respond to immunotherapy and were diagnosed by biopsy as primary central nervous system (CNS) malignancies. Diffuse H3 K27M-mutant glioma is a recently described entity with very few cases of isolated spinal disease described in adults. Primitive neuroectodermal tumor is similarly uncommon in the spinal cord. Malignancies should be considered in patients who fail to improve with immunomodulatory therapy. We believe the experiences of our center will raise awareness about that point, broaden the existing understanding of the diagnostic approach to LETM, and highlight the need for additional studies.

A 34-year-old pregnant woman at 28 gestational weeks was diagnosed with a brain tumor after experiencing a generalised seizure. After completion of antenatal fetal lung maturation, she underwent an osteoplastic craniotomy parietal on the left side and a microsurgical partial tumor resection under general anaesthesia. With a histology of a diffuse astrocytoma and the postoperative stable amount of residual tumor on follow-up imaging, the pregnancy proceeded until 37 gestational weeks. A healthy baby boy was delivered by elective caesarean section. An awake craniotomy for removal of the residual tumor was planned two weeks later, followed by adjuvant treatment (combined radio-/chemotherapy). A multidisciplinary approach, combined with appropriate timing and a transparent and empathic communication, was able to create the most effective tailored management and optimise maternal and neonatal outcomes.

An 8-year-old boy with no significant medical history or family history presented with 1–2 weeks of decreased visual acuity and blurry vision. The patient denied any headaches, vomiting, dizziness, seizures or weakness. Physical examination was significant for poorly reactive pupils, constricted

Introduction : Brain tumor is a disease with high morbidity and mortality rates. Treating brain tumors requiring medical providers to have basic understanding of brain tumor diagnosis and management. The most common brain tumors are meningiomas, gliomas, pituitary adenomas and brain metastasis. The treatment of each type of brain tumor is different and multidisciplinary involving other scientific fields besides neurosurgery. Case Series : We reported 131 cases of brain tumors at the Haji Adam Malik Hospital in the period January 2018-December 2019, consisting of 52 cases (40%) of meningioma, 34 cases (26%) of Glioma , 12 cases (9%) of pituitary adenomas and 33 cases (25%) of brain metastasis. Meningiomas were mostly treated with surgery alone in 38% of cases, gliomas with surgery followed by chemo-radiation in 41% of cases, all of pituitary adenomas were treated with endonasal transfenoid surgery, while brain metastasis in 70% of cases required whole brain radiation with controlled primary tumors. Discussion : The modalities for treating brain tumor patients has been increasing recently. Meningiomas are mostly benign and managed by surgical resection only, chemo-radiation reserved for high risk or refractory case of meningiomas. Glioblastoma is the most common glioma and aggressive malignant primary brain tumor, limited response to gross surgical tumor and chemo-radiation. Pituitary adenomas mostly treated with endonasal transfenoid surgery, radiotherapy use in recurrent case. Brain metastasis may require complex multidisciplinary care with neurosurgery, radiation oncology, and medical oncology. Conclusion: The treatment of brain tumors is influenced by various considerations such as age, patient condition,neurological deficits, location of the tumor, comorbidities, experience of the neurosurgeon and available modalities. Treatment methods for brain tumors continue to evolve.

Here we report a challenging case of a 52-year-old man presenting with subacute constipation, urinary retention, impotence, absent Achilles reflexes, and hypoesthesia in S2-S5 dermatomes. We review the clinical decision-making as the symptoms evolved and diagnostic testing changed over time. Once the diagnosis is settled, we discuss the sign and symptoms, additional diagnostic tools, treatment options and prognosis.

Background The coronavirus disease identified in 2019 (COVID-19) pandemic changed neurosurgery protocols to provide ongoing care for patients while ensuring the safety of health care workers. In Brazil, the rapid spread of the disease led to new challenges in the health system. Neurooncology practice was one of the most affected by the pandemic due to restricted elective procedures and new triage protocols. We aim to characterize the impact of the pandemic on neurosurgery in Brazil. Methods We analyzed 112 different types of neurosurgical procedures, with special detail in 11 neurooncology procedures, listed in the Brazilian Hospital Information System records in the DATASUS database between February and July 2019 and the same period in 2020. Linear regression and paired t-test analyses were performed and considered statistically significant at P < 0.05. Results There was an overall decrease of 21.5% (28,858 cases) in all neurosurgical procedures, impacting patients needing elective procedures (−42.46%) more than emergency surgery (−5.93%). Neurooncology procedures decreased by 14.89%. Nonetheless, the mortality rate during hospitalization increased by 21.26%. Linear regression analysis in hospitalizations (Slope = 0.9912 ± 0.07431; CI [95%] = 0.8231−1.159) and total cost (Slope = 1.03 ± 0.03501; CI [95%] = 0.9511−1.109) in the 11 different types of neurooncology procedures showed a P < 0.0001. The mean cost per type of procedure showed an 11.59% increase (P = 0.0172) between 2019 and 2020. Conclusions The COVID-19 pandemic has increased mortality, decreased hospitalizations, and therefore decreased overall costs, despite increased costs per procedure for a variety of neurosurgical procedures. Our study serves as a stark example of the effect of the pandemic on neurosurgical care in settings of limited resources and access to care.

OBJECTIVE Transitional care in surgical neurooncology is poorly studied. However, this period is pivotal, since it allows the patient to be empowered in his or her disease management. Here, the authors describe the experience of the Necker-Enfants Malades and the Sainte-Anne Hospital collaboration. METHODS The mixed transitional consultations started in September 2019 in a dedicated space for transitional care, named the “La Suite” department, located in the Necker-Enfants Malades Hospital, Paris, France. The authors organized planned consultations to schedule the clinical and radiological follow-up in the adult neurosurgical department but also emergency consultations to manage tumor recurrence in young adult patients. Transitional care was performed jointly by pediatric and adult neurosurgeons who have developed clinical and research skills in the field of surgical neurooncology. Neuropathological analysis was performed by a neuropathologist who is specialized in pediatric and adult neurooncology. RESULTS Fourteen patients benefited from a mixed transitional consultation. All of them accepted to start their management in an adult neurosurgical environment. Eleven patients (78.6%) for whom the disease was controlled benefited from a planned consultation. Three patients (21.4%) required rapid neurosurgical management for a tumor recurrence (n = 2) or for a new primary CNS tumor (n = 1) and benefited from an emergency consultation. CONCLUSIONS For adult patients harboring a brain tumor during childhood or adolescence, the authors suggest that neurosurgeons specialized in adult surgical neurooncology with a full knowledge in pediatric neurooncology will combine the required skills to optimize care management for these patients within a dedicated multidisciplinary organization framework.

OBJECTIVE Genomic analysis in neurooncology has underscored the importance of understanding the patterns of survival in different molecular subtypes within gliomas and their responses to treatment. In particular, diffuse gliomas are now principally characterized by their mutation status (IDH1 and 1p/19q codeletion), yet there remains a paucity of information regarding the prognostic value of molecular markers and extent of resection (EOR) on survival. Furthermore, given the modern emphasis on molecular rather than histological diagnosis, it is important to examine the effect of maximal resection on survival in all gliomas with 1p/q19 codeletions, as these will now be classified as oligodendrogliomas under the new WHO guidelines. The objectives of the present study were twofold: 1) to assess the association between EOR and survival for patients with oligodendrogliomas in the National Cancer Database (NCDB), which includes information on mutation status, and 2) to demonstrate the same effect for all patients with 1p/19q codeleted gliomas in the NCDB. METHODS The NCDB was queried for all cases of oligodendroglioma between 2004 and 2014, with follow-up dates through 2016. The authors found 2514 cases of histologically confirmed oligodendrogliomas for the final analysis of the effect of EOR on survival. Upon further query, 1067 1p/19q-codeleted tumors were identified in the NCDB. Patients who received subtotal resection (STR) or gross-total resection (GTR) were compared to those who received no tumor debulking surgery. Univariable and multivariable analyses of both overall survival and cause-specific survival were performed. RESULTS EOR was associated with increased overall survival for both histologically confirmed oligodendrogliomas and all 1p/19q-codeleted–defined tumors (p < 0.001 and p = 0.002, respectively). Tumor grade, location, and size covaried predictably with EOR. When evaluating tumors by each classification system for predictors of overall survival, facility setting, age, comorbidity index, grade, location, chemotherapy, and radiation therapy were all shown to be significantly associated with overall survival. STR and GTR were independent predictors of improved survival in historically classified oligodendrogliomas (HR 0.83, p = 0.18; HR 0.69, p = 0.01, respectively) and in 1p/19q-codeleted tumors (HR 0.49, p < 0.01; HR 0.43, p < 0.01, respectively). CONCLUSIONS By using the NCDB, the authors have demonstrated a side-by-side comparison of the survival benefits of greater EOR in 1p/19q-codeleted gliomas.

Background The impact on survival of complete resection (CR) in patients with malignant glioma and MGMT promoter methylation on adjuvant therapy strategies has been proven in the past. However, it is not known whether a MGMT promoter methylation can compensate a subtotal resection. Therefore, we analyzed the progress of postoperative residual tumor tissue depending on the molecular tumor status. Methods We included all glioblastoma, IDH-wildtype (WHO grade IV) patients with postoperative residual tumor tissue, who were treated at our neurooncological department between 2010 and 2018. Correlation of molecular patterns with clinical data and survival times was performed. The results were compared to patients following CR. Results 267 patients with glioblastoma, IDH-wildtype (WHO grade IV) received surgery of whom 81 patients with residual tumor were included in the analysis. MGMT promoter was methylated in 31 patients (38.27%). Median OS and PFS were significantly increased in patients with methylated MGMT promoter (mOS: 16 M vs. 13 M, p = 0.009; mPFS: 13 M vs. 5 M, p = 0.003). In comparison to survival of patients following CR, OS was decreased in patients with residual tumor regardless MGMT methylation. Conclusion Our data confirm impact of MGMT promoter methylation in patients with glioblastoma, IDH-wildtype on OS and PFS. However, in comparison to patients after CR, a methylated MGMT promoter cannot compensate the disadvantage due to residual tumor volume. In terms of personalized medicine and quality of life as major goal in oncology, neuro-oncologists have to thoroughly discuss advantages and disadvantages of residual tumor volume versus possible neurological deficits in CR.

A 38-year-old man presented to our centre with subacute cognitive decline and right limb weakness. His memory had been normal until 5 months before, initially having difficulty recalling friends’ names. Over the next 3 months, he became sleepy and lacked interest in social activities. Gradually his right limbs became inflexible, interfering with tooth brushing and walking. An MR scan of brain at another hospital showed asymmetric confluent white matter hyperintensities, mild temporal lobe atrophy and normal intracranial arteries (figure 1). He had been treated elsewhere with intravenous immunoglobulins and corticosteroids for presumed antibody-negative autoimmune encephalitis, but his symptoms had continued to worsen despite regular immunotherapy, such that he could no longer talk or walk. Figure 1 MR scan of brain series (A) FLAIR and (B) enhanced MR scan of brain 3 months after onset showed confluent white matter hyperintensities involving left fronto-temporo-insular lobe, basal ganglia and thalamus, with mild temporal lobe atrophy and faint patchy enhancement. Repeat (C) FLAIR and (D) enhanced MR scan of brain 5 months after onset showed prominent left temporal lobe atrophy. FLAIR, fluid-attenuated inversion recovery. He had been previously well and was immunocompetent. There was no history of substance abuse or toxin exposure and no relevant family history. On examination, he was alert with mixed aphasia. Cognitive assessment identified impaired temporal and spatial orientation and problems with long-term and short-term memory. He could not complete calculation or the Mini-Mental State Examination. There was no meningism. He had a right facial palsy. The strength in his right arm and leg was rated as 3/5 proximally and 0/5 distally; left-sided limb strength was normal. Deep tendon reflexes were 4+ on the right, with inducible ankle clonus, and 3+ on the left. The right plantar response was extensor. Sensation testing was limited by cognitive deficits. His sensation to painful stimuli …

Assessment of disease activity in glioblastoma (GBM) can be challenging due to several clinical and radiological pitfalls. Besides MRI, FET-PET and neurocognitive assessment (NA) are used in several neuro-oncological centers in order to improve the specificity of response assessment. We performed a retrospective study to investigate whether the assessment by RANO (Response Assessment in NeuroOncology) corresponds to FET-PET imaging and NA results. Moreover, the concordance of RANO with a final recommendation of an interdisciplinary neuro-oncological tumor board recommendation (TBR) was analyzed. We enrolled 25 consecutive patients with newly diagnosed histologically confirmed GBM in a pilot study, accounting for 81 multimodal test results. All patients were selected after undergoing consecutive follow-up comprising MRI, FET-PET, and NA with a subsequent TBR. Results were analyzed for correlations between RANO, FET-PET and NA. An additional consistency analysis was performed to elucidate the impact of RANO on decision making. A highly statistically significant correlation was found between RANO and FET-PET and NA results (all P

The article is devoted to the issue of molecular genetic diagnosis of cerebral glioblastomas. Despite significant advances in neurooncology, little progress has been made in prolonging the life of patients with cerebral glioblastoma, and a significant part of the effectiveness of treatment depends on the recognition of two prognostic biomarkers: mutations of the isocitrate dehydrogenase (IDH) promoter and the methylation of the O6-methylguanine methyl transferase (MGMT) promoter. The article summarizes the data of world and domestic clinical studies, allowing to supplement the histological characteristics of primary glioblastomas with genetic markers: the presence of the TERT mutation, EFGR amplification, loss of PTEN function, LOH 10q, and the presence of the BRAF mutation. It should be noted that the amplification of EGFR, causing resistance to apoptotic stimuli and alkylating chemotherapy with Temozolomide, attracts much attention as a therapeutic target. The frequency of occurrence of the TERT mutation is 90% of all tumors of various genesis, most often the TERT mutation is found in oligodendroglioma or primary glioblastoma. Loss of heterozygosity in the region of localization of the PTEN gene is observed in many types of sporadic tumors, including more than 40% of glioblastomas. Mutations in this gene are found in tumors of the brain, endometrium, prostate, kidney, and mammary gland. The presence of a PTEN mutation is a poor prognostic factor. LOH 22q is much more common in secondary glioblastomas (82%) than in primary glioblastomas (41%). Among brain tumors, the BRAF mutation is most common with pleomorphic xanastrocytoma (60-70%).The BRAF V600E mutation was found in epithelioid glioblastoma, which is a rare and aggressive type of glioblastoma, characterized by an unfavorable prognosis (about 6 months) and frequent leptomeningeal spread. Thus, knowledge of the molecular mechanisms of carcinogenesis will enable a personalized approach to treatment with glioblastomas of the brain.

Purpose The introduction of hypofractionated stereotactic radiosurgery (hSRS) extended the treatment modalities beyond the well-established single-fraction stereotactic radiosurgery and fractionated radiotherapy. Here, we report the efficacy and side effects of hSRS using Cyberknife® (CK-hSRS) for the treatment of patients with critical brain metastases (BM) and a very poor prognosis. We discuss our experience in light of current literature. Methods All patients who underwent CK-hSRS over 3 years were retrospectively included. We applied a surface dose of 27 Gy in 3 fractions. Rates of local control (LC), systemic progression-free survival (PFS), and overall survival (OS) were estimated using Kaplan–Meier method. Treatment-related complications were rated using the Common Terminology Criteria for Adverse Events (CTCAE). Results We analyzed 34 patients with 75 BM. 53% of the patients had a large tumor, tumor location was eloquent in 32%, and deep seated in 15%. 36% of tumors were recurrent after previous irradiation. The median Karnofsky Performance Status was 65%. The actuarial rates of LC at 3, 6, and 12 months were 98%, 98%, and 78.6%, respectively. Three, 6, and 12 months PFS was 38%, 32%, and 15%, and OS was 65%, 47%, and 28%, respectively. Median OS was significantly associated with higher KPS, which was the only significant factor for survival. Complications CTCAE grade 1–3 were observed in 12%. Conclusion Our radiation schedule showed a reasonable treatment effectiveness and tolerance. Representing an optimal salvage treatment for critical BM in patients with a very poor prognosis and clinical performance state, CK-hSRS may close the gap between surgery, stereotactic radiosurgery, conventional radiotherapy, and palliative care.

Background Quality of life is essential for oncologic patients. Several tools are available to improve microsurgery and reduce morbidity. Diode laser is a precise and useful technology for microsurgery. The goal of this pioneer case series is to describe the oncologic use of the 980nm diode laser and the qualitative variables analyzed. Besides, review the current literature about lasers in neurosurgery. Methods A longitudinal prospective study described patients with meningioma or glioma submitted to neurosurgical laser-assisted procedures. Also, we performed a review in medical databases using the terms “diode laser” and “neurosurgery.” Results No paper described the use of a diode laser in neurooncology. The 980nm diode laser was used in 15 patients. The device is thin, silent, and easy to handle. Excellent hemostasis was observed, especially in skull base meningiomas. Also, it was easy and fast to delimit tumor from normal brain tissue without damage to surrounding parenchyma. No postoperative complications occurred. Conclusions The diode laser is a useful tool for brain tumor surgery, particularly concerning hemostasis. Surgical site coagulation is effective without damage to adjacent structures, especially in gliomas near eloquent regions. We consider this technique a suitable adjuvant resource for brain tumor surgeries to provide an excellent hemostasis and help cut and vaporize a lesion.

Leukemia-associated myelitis is a rare but underestimated complication. It has a different etiology associated with both, the main disease and its treatment methods. It requires differential diagnosis with funicular myelosis, polyradiculoneuropathy, tumor and hemorrhagic formation, stroke, dysmetabolic manifestations, as well as with the consequences of treatment of the underlying disease using radiation, cytostatic, targeted therapy.It should also be differentiated from paraneoplastic myelopathy and progression of the underlying disease. However,with the help of neuroimaging methods, it can be detected more recently than a detailed clinical picture appears. A case report of myelopathy in a 31 year old patient with acute lymphoblastic leukemia is presented. Treatment of the underlying disease was carried out with the use of chemotherapy, radiation therapy, allogeneic hematopoietic stem cell transplantation and targeted therapy. The nature of the disease,i.e. recurrent course of acute lymphoblastic leukemia, the variety of treatment methods, and the absence of focal changes in neuroimaging in the zone that determines clinical manifestations, made it necessary to consider a wide range of possible etiological factors for the development of myelopathy. Myelopathy was confirmed by MRI 2.5 months after the debut of neurological symptoms, which corresponds to modern concepts and time criteria for visualization in neurooncology. The article presents the criteria for diagnosing myelopathy, a complication of acute lymphoblastic leukemia. It should also be differentiated from However, with the help of neuroimaging methods, it can be detected more recently than a detailed clinical picture appears.

The diagnostic potential of PET using the amino acid analogue O-(2-[F-18]fluoroethyl)-L-tyrosine ([F-18]FET) in brain tumor diagnostics has been proven in many studies during the last two decades and is still the subject of multiple studies every year. In addition to standard magnetic resonance imaging (MRI), positron emission tomography (PET) using [F-18]FET provides important diagnostic data concerning brain tumor delineation, therapy planning, treatment monitoring, and improved differentiation between treatment-related changes and tumor recurrence. The pharmacokinetics, uptake mechanisms and metabolism have been well described in various preclinical studies. The accumulation of [F-18]FET in most benign lesions and healthy brain tissue has been shown to be low, thus providing a high contrast between tumor tissue and benign tissue alterations. Based on logistic advantages of F-18 labelling and convincing clinical results, [F-18]FET has widely replaced short lived amino acid tracers such as L-[C-11]methyl-methionine ([C-11]MET) in many centers across Western Europe. This review summarizes the basic knowledge on [F-18]FET and its contribution to the care of patients with brain tumors. In particular, recent studies about specificity, possible pitfalls, and the utility of [F-18]FET PET in tumor grading and prognostication regarding the revised WHO classification of brain tumors are addressed. (C) 2020 Elsevier Inc. All rights reserved.

Intravascular papillary endothelial hyperplasia (IPEH), commonly known as Masson’s tumor, is a benign lesion that manifests as an excessive proliferation of endothelial cells within a vessel wall. IPEH is extremely rare in the brain, with only 36 intracranial cases previously described in the literature. It is commonly mistaken for more malignant pathologies, such as angiosarcoma. Careful histopathological examination is required for diagnosis, as no clinical or radiographic features are characteristic of this lesion. In this first published case of intracranial IPEH presenting during pregnancy, the authors describe a 32-year-old female with a left frontal intraparenchymal hemorrhage resulting in complete expressive aphasia at 28 weeks 6 days’ gestation. An MRI scan obtained at a local hospital demonstrated an area of enhancement within the hemorrhage. The patient underwent a left frontoparietal craniotomy for hematoma evacuation and gross-total resection (GTR) of an underlying hemorrhagic mass at 29 weeks’ gestation. This case illustrates the importance of multidisciplinary patient care and the feasibility of intervention in the early third trimester with subsequent term delivery. While GTR of IPEH is typically curative, the decision to proceed with surgical treatment of any intracranial lesion in pregnancy must balance maternal stability, gestational age, and suspected pathology.

Purpose Psychosocial screening for glioma patients is challenging because many patients suffer from neurocognitive deficits, which may impair assessment. This study’s aim was to exploratively develop three screening questions for unmet needs to prospectively be applicable in patient–doctor consultation. Methods Patient interviews, a survey for health-care professionals and a weighted scoring procedure were developed for this study. Six main areas were defined according to main areas of validated questionnaires (psyche, cognition, body, role functioning, social support, unmet needs). Patients and health-care professionals rated the importance of these areas and corresponding items, patients additionally stated whether the issues addressed affected them. Results A total of 50 patients were included, and 36 health-care professionals participated in the online survey. The three areas (psyche, body and cognition) considered to be most relevant by both, health-care professionals and patients, generated three screening questions. If the patient was affected by the issue addressed with a screening question, a subordinate question from that area that our patient sample considered most important could additionally be asked. The elaborated screening questions are the following: (1) main area psyche: “Has your mood worsened?”, (2) main area body: “Do physical changes put a strain on you?”, and (3) main area cognition: “Has your memory capacity worsened?” Conclusion These questions represent a basis for further research regarding their application in neuro-oncological clinical routine.

Background: Medulloblastoma is the most common central nervous system tumor in childhood. This study aims to evaluate the clinical features, treatments, and outcomes of pediatric patients diagnosed with medulloblastoma Materials and Method:Between 2006 and 2019, the medical records of children with medulloblastoma were reviewed retrospectively. Patients who died after surgery, before chemotherapy or radiotherapy were not included in the survival analysis Results: During the study period, 38 children were diagnosed with medulloblastoma. Twenty-one of the patients were male (55.3%) and 17 were female (44.7%). The ages of the children ranged from 3 months to 17 years (median age 8 years). The ages of five patients were under 3 years (13.1%). The most common complaints were headache (n: 26, 68.4%), imbalance (n: 21, 55.3%), and vomiting (n: 20, 52.6%). The mass sizes ranged between 3 and 6 cm in 32 patients (84.2%). At the time of diagnosis, 5 patients had seeding metastasis (13.1%). The most commonlyused chemotherapy protocol included vincristine, cisplatin, etoposide (60.5%). Five patients died after surgery without any chemotherapy or radiotherapy. Of the 33 patients included in the life analysis, 12 died (36.4%). Follow-up times ranged from 2 months to 14 years (median, 44 months). The overall survival rate was 59.1%. Eight patients had relapsed (24.2%). Late relapse was detected in 3 of the relapsed patients (relapse times were the 91st, 69th, and 72nd months). Conclusions: It is possible to achieve satisfactory treatment results in children with medulloblastoma using international treatment guidelines and recommendations, with an experienced professional team dedicated to pediatric neurooncology.

Introduction: Meningiomas of the sphenoid wing make up approximately 15–20% of total cranial meningiomas.Giant medial sphenoid involve the dura of the greater and lesser wings of the sphenoid, the anterior clinoid process,the spheno-orbital bone and the middle cranial fossa, hyperostosis bone was common to found. Surgicalmanagement of giant medial sphenoid is extremely challenging due to their intimate relationship with vital neuralstructures.Case Report : A 40-year-old woman came to the emergency room with complaints of decreased consciousness thathad been experienced since 1 week , recurrent headaches, weakness of the right limb and history of blurred vision. Anon-contrast head ct scan and MRI brain contrast revealed an extra-axial giant mass meningioma in left medialsphenoid wing with hyperostosis bone Craniectomy tumor removal was performed with a subtemporal approachcombined with cranio-orbytozygotomy.Discussion : Hyperostosis is a common phenomenon occuring in different meningiomas with incidence rangingfrom 25 to 49% of meningiomas.it is most commonly seen in sphenoid wing and convexity meningioma . The bonyhyperostosis is of neoplastic nature and is responsible for many of the clinical manifestation of such tumors andhence should be totally drilled to achieve cure and avoid recurrence.Conclusion : Sphenoid wing meningioma en plaque, mainly meningothelial meningiomas, are characterized by theassociated bony hyperostosis that gives them a distinct radiological appearance. Extensive tumor removal is crucialfor correction of proptosis and adequate visual decompression to achieve satisfactory cosmetic and functionaloutcome.

Surgery of the base of the skull requires knowledge of the topography of the internal carotid artery, cavernous sinus, cranial nerves of the sellyar and paraclinoid regions. Equally important is knowledge of the topography and structure of the dura mater (CSF) at the base of the skull, CSF duplicates, cerebellar ligaments, meningeal membranes (tank membranes of the brain base, carotid oculomotor membrane of the proximal carotid ring) and carotid rings in neurooncology and vascular neurosurgery. The article presents the results of the study of the structure of TMO in the selvary and paraselar regions.

Neuro-oncology is a rapidly evolving subspecialty that involves the management of patients with primary or metastatic central and peripheral nervous system neoplasms, as well as any other disorders or complications affecting the nervous system that result either directly or indirectly from central nervous system or systemic malignancies and related treatment. Neurologists serve a critical role in the multidisciplinary management of these complex patients. As leaders of the Child Neurology Society Special Interest Group in NeuroOncology, we propose ways to provide sufficient exposure, minimize knowledge gaps, and optimize training experiences in neuro-oncology for child neurology residency programs.

Emerging evidence suggests that STK11 mutations may influence clinical outcome and response to immunotherapy in cancer. Next-generation targeted sequencing of STK11 mutation status in a large cohort of 188 meningiomas. STK11 loss-of-function mutations were identified in 3.7% of meningiomas. STK11 mutations were found in both low- and high-grade lesions and samples from primary and recurrent disease. There was a 2.8-fold increased risk of death for patients whose meningioma harbored an STK11 mutation, after controlling for lesion grade and occurrence status. The median overall survival for patients with STK11-mutated meningiomas was 4.4 years compared with 16.8 years. These data identify recurrent STK11 mutations in a subset of meningiomas. Genotyping of STK11 is encouraged for meningioma patients undergoing immunotherapy-based therapy.

Los gliomas del tallo son el 10-20% de los tumores cerebrales pediátricos, el glioma pontino intrínseco difuso es el glioma del tallo más común en la infancia, se trata de un grupo heterogéneo de tumores biológicamente distinto de los demás gliomas de alto grado pediátricos y del adulto. Los pacientes con un glioma pontino intrínseco difuso presentan un rápido déficit de pares craneales, paresia o ataxia, la apariencia de estas lesiones en resonancia magnética nuclear es característica, constituyen un desafío terapéutico en la neurooncología. La quimioterapia no ha demostrado ser un tratamiento que detenga o retrase el crecimiento del tumor. La radioterapia es el único tratamiento válido, pero su eficiencia es solo transitoria. Este artículo describe dos casos clínicos que involucran a un paciente pediátrico y a un paciente adulto, diagnosticados con un glioma pontino intrínseco difuso, el propósito es hacer un paralelo entre los casos.

Introduction: The extent of meningioma resection is the most fundamental risk factor for recurrence, and exact knowledge of extent of resection is necessary for prognostication and for planning of adjuvant treatment. Currently used classifications are the EANO-grading and the Simpson grading. The former comprises radiological imaging with contrast-enhanced MRI and differentiation between “gross total removal” and “subtotal removal,” while the latter comprises a five-tiered differentiation of the surgeon’s impression of the extent of resection. The extent of resection of tumors is usually defined via analyses of resection margins but has until now not been implemented for meningiomas. PET/MRI imaging with 68Ga-DOTATOC allows more sensitive and specific imaging than MRI following surgery of meningiomas. Objective: To develop an objective grading system based on microscopic analyses of resection margins and sensitive radiological analyses to improve management of follow-up, adjuvant therapy, and prognostication of meningiomas. Based on the rationale of resection-margin analyses as gold standard and superior imaging performance of 68Ga DOTATOC PET, we propose “Copenhagen Grading” for meningiomas. Results: Copenhagen Grading was described for six pilot patients with examples of positive and negative findings on histopathology and DOTATOC PET scanning. The grading could be traceably implemented and parameters of grading appeared complementary. Copenhagen Grading is prospectively implemented as a clinical standard at Rigshospitalet, Copenhagen. Conclusion: Copenhagen Grading provided a comprehensive, logical, and reproducible definition of the extent of resection. It offers promise to be the most sensitive and specific imaging modality available for meningiomas. Clinical and cost-efficacy remain to be established during prospective implementation.

Purpose: Pediatric low-grade glioma (pLGG) is the most prevalent childhood brain tumor. Patients with BRAF V600 mutation–positive pLGG may benefit from treatment with dabrafenib. Part 2 of a phase I/IIa study, open-label study (NCT01677741) explores the activity and safety of dabrafenib treatment in these patients. Patients and Methods: Patients ages 1 to Results: Overall, 32 patients with pLGG were enrolled (part 1, n = 15; part 2, n = 17). Minimum follow-up was 26.2 months. Among all patients, the ORR was 44% [95% confidence interval (CI), 26–62] by independent review. The 1-year progression-free survival rate was 85% (95% CI, 64–94). Treatment-related adverse events (AE) were reported in 29 patients (91%); the most common was fatigue (34%). Grade 3/4 treatment-related AEs were reported in 9 patients (28%). Conclusions: Dabrafenib demonstrated meaningful clinical activity and acceptable tolerability in patients with BRAF V600–mutant pLGG.

Annually, the incidence of brain tumors has slightly increased and also the patient prognosis is still disappointing, especially for high-grade neoplasms. So, researchers seek methods to improve therapeutic index as a critical aim of treatment. One of these new challenging methods is radioimmunotherapy (RIT) that involves recruiting a coupling of radionuclide component with monoclonal antibody (mAb) which are targeted against cell surface tumor–related antigens or antigens of cells within the tumor microenvironment. In the context of cancer care, precision medicine is exemplified by RIT; precision medicine can offer a tailored treatment to meet the needs for treatment of brain tumors. This review aims to discuss the molecular targets used in radioimmunotherapy of brain tumors, available and future radioimmunopharmaceutics, clinical trials of radioimmunotherapy in brain neoplasms, and eventually, conclusion and future perspective of application of radioimmunotherapy in neurooncology cancer care.

Objective Diffuse Midline Gliomas (DMG), H3 K27M-mutant have the poorest prognosis among all pediatric high-grade gliomas, with a median survival of 9-11 months. Although radiotherapy (RT) is standard treatment for these tumors, unfortunately there has been no approved and effective treatment which completely diminishes the tumor yet. In our clinic, we started an up-to-date approach to manage DMG, which is adjuvant fractionated external beam radiotherapy along with ONC 201 after tissue diagnosis. Methodology Between January 2016 and June 2021, a total of 11 patients with H3 K27M-mutant diffuse midline glioma, diagnosis confirmed by Next-Generation Sequencing (NGS) were enrolled in study. All patients received ONC201 orally once a week following radiotherapy. Safety, and radiological evaluations were regularly assessed every 12 weeks. Results Among the 11 patients, the median age of diagnosis was 5. Seven (63.6%) patients were male and 4 (36.4%) were female. Primary lesions were localized in the pons in 5 (45.5%) patients, unilateral thalamus (2 on the left, 1 on the right) in 3 (27.3%) patients, bilateral thalamus in 2 (18.2%) patients, and temporo-insular in 1 patient (9.1%). Median progression-free interval was 10 months and median overall survival was 16 months. Conclusion Diffuse midline glioma has dismal prognosis. None of the treatment options made any dramatic changes in disease course during last 30 years. In our series, diffuse midline glioma patients who had ONC201 tend to have few months more progression free and overall survival (16 vs 11 months) in comparison to patients who had classical treatment in literature. As a neurooncology team, we strongly advocate to obtain tissue samples from diffuse tumors, to establish definite diagnosis and to perform NGS

PURPOSE Medulloblastoma is composed of four clinically and prognostically distinct molecular subgroups (WNT, SHH, group 3, and group 4). However, the clinical implications of these subgroups in the context of the unique challenges of low- to middle-income countries are rarely reported. METHODS We assembled an institutional cohort of children (3-18 years) diagnosed with medulloblastoma and treated in Jordan between 2003 and 2016. Tumors were subgrouped by NanoString and correlated with clinical and radiologic characteristics. RESULTS Eighty-eight patients were identified (63% male); median age was 6.9 years (interquartile range 4.8-9.2) and median symptom duration was 6 weeks (interquartile range 4-11). Radiotherapy was implemented as standard-risk in 41 patients (47%) and high-risk in 47 patients (53%). Subgrouping revealed 17 WNT (19%), 22 SHH (25%), 21 group 3 (24%), and 28 group 4 tumors (32%). Median time between craniotomy and radiotherapy was 45 days (17-155); 44% of them > 49 days. Median duration of radiotherapy was 44 days (36-74). Seventy-two patients (82%) received chemotherapy afterward. With a median follow-up of 4.6 years (0.2-14.9), 5-year progression-free survival (PFS) and overall survival were 73.5% and 69.4%, respectively, with no statistically significant survival difference between standard-risk and high-risk patients. Metastasis was significant for overall survival (P = .011). Patients with SHH and group 4 tumors had very good PFS (83.4% and 87.0%, respectively) and those with group 3 tumors had dismal outcomes (PFS 44.9%), whereas WNT tumors had less-than expected PFS (70.5%). PFS was statistically significant in patients with nonmetastatic tumors receiving radiotherapy ≤ 49 days (P = .011), particularly group 3 tumors. CONCLUSION Patients with SHH and group 4 medulloblastoma had excellent survival comparable with high-income countries. Compliance with treatment protocols and avoiding radiotherapy delays are important in achieving adequate survival in low- to middle-income country settings. Subgroup-driven treatment protocols should be considered in countries with limited resources., Molecular diagnostics improves prognostication of medulloblastoma in resource limited settings.

A 21-year-old man developed progressive and bilateral lower limb numbness, gait impairment and urinary incontinence over 10 days. He had received intrathecal methotrexate 20 days previously for acute lymphoblastic B-cell leukaemia, following 7 months of systemic chemotherapy. MR scan of the spinal cord showed bilateral symmetric and extensive T2/fluid attenuated inversion recovery (FLAIR) increased signal involving the dorsal columns in the thoracic cord. His serum folate concentration was at the lower end of the normal range. We stopped the intrathecal chemotherapy and gave folate; after a few days, he progressively improved. Myelopathy is an important adverse effect of intrathecal methotrexate, which may cause clinical and imaging features resembling subacute combined degeneration of the spinal cord. CNS infiltration must be excluded, intrathecal chemotherapy stopped and deficiency of folate or vitamin B12 treated as appropriate.

Glioblastoma (GB) is the most common and most malignant primary brain tumour in adults. Despite much effort, gold standard therapy has not changed since the introduction of adjuvant temozolomide in 2005 and prognosis remains poor. Despite this, there has been significant improvement in the surgical technology and technique, that has allowed for increased rates of safe maximal resection of the tumour. In addition, our increased knowledge of the biology of GB has revealed more potential targets, especially in the field of immunotherapy, which has been successful in revolutionising treatment of other cancers. We review the current best practice for the treatment of GB and explore some of the more recent advances in GB management from both a surgical and molecular therapeutic perspective.

Intramedullary tumors of the spinal cord account for 2%–4% of all central nervous system tumors. Surgical resection with intraoperative neuromonitoring, although challenging, remains the mainstay of treatment with the goal to prevent neurologic decline. We present a case of an unusual anaplastic glioma of the thoracic spinal cord in a 42-year-old male that spanned 7 vertebral levels and could not be definitively characterized using the 2021 World Health Organization Classification of Central Nervous System Tumors.

Medulloblastoma is one of the most common malignant brain tumors in the pediatric population. Recent studies identified four distinct medulloblastoma subgroups with different molecular alterations and pathways, and natural courses and outcomes. To evaluate the results of surgical and medical treatments of patients with medulloblastoma and compare them among the medulloblastoma subgroups. The clinical and radiological features, medical and surgical management and treatment outcomes and their correlation with molecular subgroups of 58 patients treated for medulloblastoma in the last 20 years were evaluated. Fifty-eight patients, of whom 35 were male and 23 were female, were evaluated. The median age was 6 years (range, 1–19 years). The most common symptoms were nausea and vomiting (60%). Forty-three percent of the patients had headache and 40% had ataxia. Previous pathology reports revealed that 43 (74%), eight (14%), five (8%), and two (3%) had classic, desmoplastic, desmoplastic/nodular, and anaplastic morphologies, respectively. After the subgroup analyses, five patients (12%) were attributed to the wingless subgroup (WNT) group; 14 (32.5%), to the sonic hedgehog subgroup (SHH) group; and 24 (56%), to the non-WNT non-SHH group. On the basis of immunohistochemical analysis results, 15 patients could not be attributed to any subgroups. The clinical risk groups (average vs high-risk) and age at diagnosis (≥ 3 years vs

We describe a rare case of large, fully cystic spinal schwannoma in a young adult from The Gambia. The initial clinical suspicion was spinal cystic echinococcosis. He came to our attention reporting progressive walking impairment and neurological symptoms in the lower limbs. An expansive lesion extending from L2 to S1 was shown by imaging (ie, CT scan and MRI). Differential diagnoses included aneurysmal bone cyst and spinal tuberculosis and abscess; the initial suggested diagnosis of spinal cystic echinococcosis was discarded based on contrast enhancement results. The final diagnosis of cystic schwannoma was obtained by histopathology of the excised mass. Cystic spinal lesions are rare and their differential diagnosis is challenging. Awareness of autochthonous and tropical infectious diseases is important, especially in countries experiencing consistent migration flow; however, it must be kept in mind that migrants may also present with ‘non-tropical’ pathologies.

International audience; Neurooncology is a young specialty which initially dealt mostly with glioblastoma patients with a short overall survival (OS). Yet, recently the scope gradually expanded by taking care of lower-grade glioma (LGG) patients with a longer OS (1). Historically, these patients were managed with a “wait and see attitude” claiming “benignity” despite 6 to 7 years OS (2, 3). Therefore, quality of care was mainly based on physician’s subjectivity and not on the natural history, leading to beliefs that early surgery was not adapted due to “normal neurological examination”.Twenty years later, it is now admitted that (i) beyond seizures, LGG patients suffer from cognitive and behavioral deficits at diagnosis even in incidental cases (4) (ii) this tumor will inescapably transform in higher grades, explaining the use of “lower-grade glioma” (mixing II/III) expression (5) (iii) early surgery is a main therapeutic factor (significant correlation between extent of resection and OS) (6, 7) (iv) early radiotherapy, at least given alone, is not associated with decreased mortality (6, 8). These changes resulted in a longer life expectancy now over 15 to 16 years (9–11).Moreover, neurooncologists had to pay more attention to quality of life (QoL) for patients who must learn to live with a chronic neoplastic disease.On the other hand, because LGG will systematically recur, further adapted treatments have to be administrated (12). However, heterogeneity of progression patterns (13) makes the prediction of timescales of proliferation, migration, and degeneration at the individual level impossible.To provide more reliable prognostic factors, advances in molecular biology led to a new classification designed for more appropriate decisions (14). Surprisingly, although genetics was initially a tool to better dissociate types of LGG with distinct prognosis, molecular biology rapidly became the first parameter in guidelines (15). Although useful, by taking mostly account of genetics criteria and extrapolating a correlation to specific OS based upon statistical analysis, there is a risk to neglect tumor-host interactions, patient’s wishes, and long-term QoL.Here, the main purpose is to redefine what “best quality of care” means by considering both tumor characteristics and patient’s personal criteria. The ultimate goal is to give the choice of therapeutic orientation at each step thanks to honest although complex and time-consuming information highlighting oncofunctional balance and various strategies individually adapted over time in parallel with changes in tumor behavior and patient’s expectations.

Background: Despite the aggressiveness of multimodal treatment, glioblastoma (GBM) is still a challenge for neurosurgeons, neurooncologists, and radiotherapists. A surgical approach is still a cornerstone in GBM therapeutic management, as the extent of resection is strongly related both to overall survival and progression-free survival. From this perspective, the use of photodynamic molecules could represent an interesting tool to achieve maximal and safe resection. Being able to trace the lesion’s edges, indeed, could allow to improve the extent of resection and to minimize residual tumor while sparing normal tissue. The use of 5-aminolevulinic acid (5-ALA) as a photodynamic tracer is well established due to its strict correlation both with cellularity and metabolic activity of the GBM cell clones. Objective: Our study aims to define whether a different molecular asset of GBM (especially investigating IDH 1/2 mutation, proliferation index, and MGMT promoter methylation) results in different fluorescence expression, possibly because of differences in metabolic pathways due to different genotypes. Methods: Patients undergoing surgery for GBM removal at our Institute (Dep. Of Neurosurgery, Ospedale Città della Salute e della Scienza, University of Turin, Italy) were retrospectively reviewed. Patients with histological diagnosis confirmation and to whom 5-ALA was given before surgery were included. The whole surgical procedure was recorded and then analyzed by three different people (a medical student, a resident, and a senior surgeon with an interest in neurooncology and experience in using 5-ALA) and a score was assigned to the different degrees of intraoperative fluorescence. The degree of fluorescence was then matched with the genotype. Results: A trend of grade 2 fluorescence (i.e., ”strong”) was observed in the IDH 1/2 wild-type (WT) genotype, suggesting a more intense metabolic activity in this particular subgroup, while, no or weak fluorescence was observed more often in the IDH 1/2 mutated tumors, suggesting a lower metabolic activity. No relations were found between fluorescence grade and MGMT promoter methylation or, interestingly, cellularity. As a secondary analysis, more epileptogenicity of the IDH 1/2 mutated GBM was noticed, similarly to other recent literature. Conclusion: Our results do not support the use of 5-ALA as a diagnostic tool, or a way to substitute the molecular profiling, but confirm 5-ALA as a powerful metabolic tracer, able to easily detect the pathological cells, especially in the IDH WT genotype, and in this perspective, further studies will be necessary to better describe the metabolic activity of GBM cells.

Effective cancer therapeutics for brain tumors must be able to cross the blood-brain barrier (BBB) to reach the tumor in adequate quantities and overcome the resistance conferred by the local tumor microenvironment. Clinically approved chemotherapeutic agents have been investigated for brain neoplasms, but despite their effectiveness in peripheral cancers, failed to show therapeutic success in brain tumors. This is largely due to their poor bioavailability and specificity towards brain tumors. A targeted delivery system might improve the efficacy of the candidate compounds by increasing the retention time in the tumor tissue and minimizing the numerous side effects associated with the non-specific distribution of the chemotherapy agent. Heptamethine cyanine dyes (HMCDs) are a class of near-infrared fluorescence (NIRF) compounds that have recently emerged as promising agents for drug delivery. Initially explored for their use in imaging and monitoring neoplasms, their tumor-targeting properties have recently been investigated for their use as drug carrier systems. This review will explore the recent developments in the tumour-targeting properties of a specific group of NIRF cyanine dyes and the preclinical evidence for their potential as drug-delivery systems in the treatment of primary and metastatic brain tumors. Highlighting the challenges in neurooncology, this review puts forward a unique drug-delivery system to re-invigorate the possible use of existing anti-cancer agents, previously neglected for their lack of permeability and specificity, for the treatment of brain tumors and metastases.

TSPO-PET tracers are sensitive to a single-nucleotide polymorphism (rs6971-SNP), resulting in low-, medium- and high-affinity binders (LABs, MABs and HABS), but the clinical relevance of [18F]GE-180 is still unclear. We evaluated the impact of rs6971-SNP on in vivo [18F]GE-180 binding in a healthy brain and in pseudo-reference tissue in neuro-oncological and neurodegenerative diseases. Standardized uptake values (SUVs) of [18F]GE-180-PET were assessed using a manually drawn region of interest in the frontoparietal and cerebellar hemispheres. The SUVs were compared between the LABs, MABs and HABs in control, glioma, four-repeat tauopathy (4RT) and Alzheimer’s disease (AD) subjects. Second, the SUVs were compared between the patients and controls within their rs6971-subgroups. After excluding patients with prior therapy, 24 LABs (7 control, 5 glioma, 6 4RT and 6 AD) were analyzed. Age- and sex-matched MABs (n = 38) and HABs (n = 50) were selected. The LABs had lower frontoparietal and cerebellar SUVs when compared with the MABs and HABs, but no significant difference was observed between the MABs and HABs. Within each rs6971 group, no SUV difference between the patients and controls was detected in the pseudo-reference tissues. The rs6971-SNP affects [18F]GE-180 quantification, revealing lower binding in the LABs when compared to the MABs and HABs. The frontoparietal and cerebellar ROIs were successfully validated as pseudo-reference regions.

A 39-year-old female Chinese non-smoker was diagnosed with epidermal growth factor receptor mutation-positive lung adenocarcinoma with cerebral metastases and commenced erlotinib. After 5 weeks, she presented with a 3-day history of severe bilateral facial weakness (House-Brackmann grade V/VI) and hypogeusia consistent with bilateral facial nerve palsies. MRI demonstrated new, symmetrical contrast-enhancing foci at the expected location of the facial nerves, consistent with leptomeningeal progression. Erlotinib was ceased and osimertinib was commenced. Facial nerve motor and sensory function began to improve within 1 week and by 2 weeks had returned to near normal. Review at 2 and 6 months demonstrated normal facial nerve function and progressive resolution of the facial nerve lesions on MRI. While rare, leptomeningeal malignancy may present as simultaneous bilateral facial nerve palsies. Osimertinib has superior central nervous system penetration and in this case was associated with rapid and sustained clinical and radiographical resolution of the facial nerve lesions.

We present the case of an adolescent with an intramuscular ancient schwannoma of the axillary nerve which, to the best of our knowledge, has not been reported before. Due to its deep location, intramuscular schwannoma has less clinical signs and hence, is difficult to diagnose. Ancient schwannoma, characterised by degeneration due to long course, is rare and can be mistaken for malignancy due to heterogeneous intensity and degeneration evident on MRI and nuclear atypia on histopathology. It is important to differentiate it from malignancy based on a clinically benign swelling with a long history, well-encapsulated mass on MRI with the split fat sign and absence of significant mitotic activity despite nuclear atypia. The aim of surgery should be enucleation of the tumour while preserving the function of the parent nerve.

Objective To review the rare entity of pediatric intracranial hemangiomas and discuss surgical pitfalls and pathophysiology in regard to cerebral edema formation. Materials and methods We describe an extremely rare case of intracranial infantile hemangioma in a neonate with massive cerebral edema, surgically resected urgently because of acute herniation. We review the literature of 46 other pediatric cases of intracranial hemangioma, including congenital capillary hemangiomas. We analyze the data on age, sex distribution, tumor location, growth pattern, edema formation, histopathology, treatment, and outcome. Results Isolated intracranial infantile hemangiomas in the neonatal period are extremely rare. Some but not all hemangiomas can be treated pharmacologically. Extensive cerebral edema is a frequent finding and can become an enormous problem at surgical resection. The pathogenesis of the edema formation in hemangiomas is complex and is yet not well understood. Conclusion Surgical resection of intracranial hemangiomas is associated with a high morbidity, and pharmacological treatment should, if possible, always be considered first, at least for preoperative optimization. The severity of cerebral edema varies among intracranial hemangiomas, which may be an indicator of different molecular properties of the individual lesions. This implies that further sub-classification of intracranial hemangiomas may be necessary.

Melanocytic schwannoma (MS) is a rare nerve sheath tumour characterised by melanin-producing neoplastic schwann cells that typically affects the posterior spinal nerve roots. We report an ultrarare case of recurrent/metastatic MS associated with Carney complex in a young woman with family history of breast cancer. This highlights the novel approach of combined checkpoint inhibitors (CPI) and radiotherapy. The patient was initially treated with Nivolumab along with concurrent external beam radiotherapy. There was sustained clinical benefit achieved for over 15 months with preserved quality of life. Addition of Ipilimumab, which she tolerated reasonably well, helped to control the progressive disease again for another 12 months. She harboured a rare PRKAR1AR228mutation (Carney complex) and received appropriate targeted therapy. She survived for 51 and 35 months from her initial diagnosis and start of CPI, respectively, which to the best of our knowledge is the longest documented survival in this rare entity.