Multiple primary diffuse large B-cell lymphoma masquerading as meningioma.

Background: Primary non-Hodgkin's lymphoma with multiple extra- and intra-calvarial extensions without systemic spread in an immunocompetent patient is extremely rare. They masquerade commonly as meningioma and can present as mass lesions with raised intracranial pressure. Case Description: We report one such case of primary diffuse large B-cell lymphoma (DLBCL) in a young female involving the scalp, dural involvement in the right frontal region, left parietal, and posterior fossa and mimicking both clinically and radiologically as meningioma. She was managed surgically. Histological examination showed features suggestive of DLBCL (germinal center type). She was planned for adjuvant therapy. However, at 2 months following surgery, she succumbed due to systemic involvement of the disease. Conclusion: DLBCL is seen rarely in neurosurgical practice. They can present as tumors with adjacent extra- and intra-cranial masses. They pose a diagnostic challenge as it can be easily confused with meningioma. Tumor resection is performed to confirm diagnosis and in patients who present with raised intracranial pressure. Chemotherapy is the preferred treatment, and adjuvant therapy should be started early.

Coexistence of neural tube defects and spinal arteriovenous shunts: a case series and review of literature.

BACKGROUND: Spinal arteriovenous shunts and spinal dysraphism both have a different underlying cause, disease spectrum and developmental process; hence, these entities rarely coexist in a patient. Here, we reported four cases of coexistence of adult-onset spinal arteriovenous shunt and spinal dysraphism in the same patient along with their therapeutic embolisation. Additionally, we conducted an extensive literature review to explore the potential theories and explanations for this coexistence. METHODS: We retrospectively searched our imaging database from January 2015 to December 2023 to identify instances of spinal arteriovenous shunts occurring in patients with spinal dysraphism or neural tube defect disorders. MRI and angiographic imaging, clinical presentation, treatment and follow-up were analysed. RESULTS: Four patients with arteriovenous fistula/shunt and spinal dysraphism were included in the study. The mean age of presentation was 35.5 years. The most common symptoms were sensory disturbance and motor weakness. Arteriovenous fistula or shunt was located at the lumber region in one patient and at the sacral region in three cases. Two patients have a prior history of surgery in first decade. Two patients were treated with glue embolisation. The internal iliac artery was a common feeder in all cases. CONCLUSIONS: The rare coexistence of neural tube defects with spinal vascular abnormalities should be considered when assessing a middle-aged patient with neural tube defect and myelopathy. Correct diagnosis can help in treatment planning and thereby improve prognosis.

An Institutional Perspective on the Number of Stent Retriever Pass and Rate of Recanalization in Mechanical Thrombectomy for Acute Ischemic Stroke: When to Stop?

Background and purpose: Mechanical thrombectomy is the standard treatment modality for flow restoration in acute ischemic stroke. In cases of persistent occlusion, the optimal number of retrieval attempts before considering procedure termination is currently undetermined and is a topic for research. Therefore in this study, we studied the impact of the number of stent retrieval maneuvers on the recanalization of vessels. Methods: In this retrospective single-center observational study we included 52 patients with large vessel occlusion who underwent stent retriever mechanical thrombectomy. Successful recanalization rate was defined as modified TICI (Thrombolysis in Cerebral Infarction) 2b or 3. Result: The overall successful recanalization rate was 44.24%. The recanalization rate per stent retrieval attempt was the highest in 1st attempt (28.84%) and no recanalization was observed with the 3rd, 5th, and 6th attempts (p<0.001). At most 6 retrieval attempts were used. Conclusions: After two retrieval attempts, 91% of the patients were successfully recanalized and other after the 5th attempt could not result in recanalization.

Mechanical Thrombectomy in Medium Vessels Occlusion (MeVOs): An Institutional Experience with M2 Divisions of Middle Cerebral Artery.

Background: Mechanical thrombectomy has been established as a safe, standard and effective treatment option for occlusions of the proximal segment of the middle cerebral artery (MCA), as demonstrated in numerous studies. However, performing thrombectomy in the M2 divisions of MCA presents inherent challenges. In this institutional experience, we aim to delineate the recanalisation rates achieved through mechanical thrombectomy in cases involving the M2 segment of the MCA. Methods: We conducted a retrospective analysis of patients who underwent thrombectomy due to M2 MCA occlusions in the period from January 2018 to December 2021. Various factors affecting recanalisation rates were assessed. Results: A total of 15 patients with M2 segment occlusions of the middle cerebral artery were included in the study, comprising 11 in the superior division and 4 in the inferior division. The successful recanalisation rate was 72.33%, with notably higher success observed in cases of inferior division occlusion. The primary outcome of our study was the mTICI recanalisation status, categorised as successful recanalisation (mTICI = 2b or mTICI = 3) and unsuccessful recanalisation (mTICI = 1 or mTICI = 2a) and mRS at 6 months. None of the predictors assessed reached statistical significance. Conclusions: Mechanical thrombectomy demonstrates favourable efficacy and recanalisation rates in cases of M2 MCA division occlusion. Notably, inferior division occlusions exhibit a higher likelihood of successful recanalisation.

Imaging and MR Spectroscopy in Papillary Craniopharyngioma.

Sellar-suprasellar masses exhibit a diverse range of differential diagnoses and it is feasible to establish a preliminary diagnosis before surgery by evaluating conventional CT scans and contrast-enhanced MRI results. Nevertheless, certain cases may present with inconclusive findings, making it challenging to anticipate the underlying tissue composition accurately through imaging alone. Researchers have explored the application of Proton MR spectroscopy in analyzing suprasellar tumors, and their investigations have revealed that it can complement traditional MRI by enhancing the accuracy of preoperative diagnoses. In this context, we report three biopsy-proven cases of suprasellar papillary craniopharyngioma where the MRS spectra derived from the solid component exhibited noticeable lipid peaks alongside reduced levels of choline and NAA. These findings played a pivotal role in facilitating the correct preoperative identification of papillary craniopharyngioma.

A Rare Case of Brucellosis with Multivalvular Endocarditis and Complete Heart Block.

BACKGROUND: Brucellosis is a public health concern that affects multiple organs. However, cardiovascular problems arise infrequently, affecting fewer than 2% of cases, typically presenting as endocarditis. CASE PRESENTATION: A 50-year male was admitted with low-grade fever, night sweats, weight loss (13 kg), malaise, and generalized weakness for the past 6 months. On clinical examination, he was febrile with 39.0°C, an average heart rate of 54 bpm, and 100/40 mmHg blood pressure. On cardiovascular examination, S1 and S2 were soft with pan systolic murmur present in the mitral area, and the early diastolic murmur was present in the left third intercostal space. Electrocardiography was suggestive of third-degree heart block with AV dissociation. Transthoracic echocardiography showed mobile vegetations attached to multiple valves- an aortic valve (18.2x11.9mm) and a mitral valve (2.9x7.5mm) with perivalvular abscess. He was given oral doxycycline (100mg B.D.) and rifampicin (600mg/day); the patient responded, but the AV block did not resolve. CONCLUSION: This report has drawn attention to multivalvular involvement and cardiac rhythm abnormalities in Brucellosis (in this case, A.V. dissociation was present) because early diagnosis and treatment can cause a significant decrease in morbidity as well as mortality by appropriate treatment.

Unveiling Distinctive MRI Characteristics in the Diagnosis of GFAP Astrocytopathy: A Rare Autoimmune Neuroinflammatory Disorder.

Glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune inflammatory disorder affecting the central nervous system, involving the meninges, brain parenchyma, and spinal cord. The distinctive radiologic feature observed on magnetic resonance imaging (MRI) is characterized by periventricular radial and linear contrast enhancement. This case report details a 45-year-old male who initially exhibited constitutional symptoms, followed by encephalitis, lower limb weakness, and urinary retention. The MRI findings revealed meningoencephalitis with longitudinal extensive myelitis. Notably, the cerebrospinal fluid analysis confirmed the presence of anti-GFAP antibodies.

Correlation Between Optical Coherence Tomography Angiography Findings at 3 to 6 Weeks and Functional Outcome at 3 Months Following Acute Ischaemic Stroke Due to Extracranial Carotid Artery Atherosclerotic Disease.

We wanted to evaluate if optical coherence tomography angiography OCTA findings could predict the functional outcome in extracranial carotid artery atherosclerotic disease (ECAD) associated stroke. This exploratory study was performed on adults with acute ischaemic stroke due to ECAD at 3-6 weeks following stroke onset with risk factor matched controls without carotid artery stenosis. Twenty-three stroke patients (cases) and 23 controls were enrolled. There was significant difference between cases and controls in deep vessel density at the macula (p = .0007) and in radial peripapillary capillary perfusion density (RPCPD) at the optic nerve head (ONH) (p = .0007). Statistically significant difference was noted in the total superficial vessel density (SVD) at the macula (SVD within 1 standard deviation [SD] versus SVD beyond 1 SD of control data) in the ipsilateral eye and functional outcome at 3 months (poor versus very good outcome, modified Rankin scale [mRS] 0-1 versus mRS 2-6, respectively; p = .0361). There was statistically insignificant correlation between the RPCPD at the ONH and the National Institutes of Health Stroke Scale score at admission, mRS at discharge, and mRS at 3 months following stroke onset (r = .33, r = .35, r = .39; p = .11, p = .09, p = .06, respectively). The findings of this exploratory study suggested that OCTA findings may predict 3 month outcomes in cases of ECAD-related stroke and could be useful in decision making in future intervention studies as to whether intervene or not in patients having critical or non-critical ECAD for preventing stroke.

Clinicoradiological Parameters and Biochemical and Molecular Alterations Predicting Remission and Recurrence After Surgical Treatment of Corticotroph Adenomas-Cushing Disease.

OBJECTIVE: Endonasal endoscopic transsphenoidal surgery (TSS) and resection of pituitary adenomas are considered the gold standard treatment for Cushing disease (CD). Even with various recent advances in management, disease persistence and recurrence are common in these patients. The remission rate in the global population after surgery has been reported to vary widely from 64% to 93%. This study aims to determine the various clinical, biochemical, radiological, and histological factors that correlate with persistence and recurrence in patients with CD. This study also aims to understand the clinicopathological significance of EGFR-MAPK, NF-κB, and SHH pathway activation and to study the protein expression of activation markers of these pathways (i.e., c-Fos, c-Jun, GLI-1, pMEK, NR4A1, and p44) in functioning corticotroph pituitary adenomas. METHODS: From January 2009 to September 2022, the clinical data of 167 patients who underwent surgical treatment (n = 174 surgeries) for CD with a median follow-up of 8.1 years (range, 1-13.29 years) were ambispectively analyzed. The preoperative clinical, biochemical, and radiological features, operative findings, postoperative clinical and biochemical data, and histopathological and molecular profiles were retrieved from the electronic medical records. The patients were followed up to assess their remission status. RESULTS: Among the 174 surgeries performed, 140 were primary surgeries, 22 were revision surgeries, 24 surgeries were for pediatric patients, and 12 surgeries were for patients with Nelson syndrome. In the primary surgery cohort, 74.3% were female, and the average age was 28.73 ± 10.15 years. Of the primary surgery cohort, 75% of the patients experienced remission compared with 47.4% after revision surgery. The remission rate for the pediatric patients was 55.5%. The postoperative day 1 plasma cortisol (P < 0.001; area under the curve, 0.8894; range, 0.8087-0.9701) and adrenocorticotropic hormone (P < 0.001; area under the curve, 0.9; range, 0.7386-1) levels were seen to be strong independent predictors of remission in the primary surgery cohort. The remission rate after endoscopic TSS was greater than that after microscopic TSS in patients undergoing primary surgery (81.08% vs. 57.14%; P = 0.008). The presence of adenoma on histopathological examination (HPE) was also a strong predictor of disease remission (P = 0.020). On stratifying by surgical approach and HPE, microscopically operated patients without histopathological evidence of adenoma had significantly higher odds of nonremission (odds ratio, 38.1; 95% confidence interval, 4.2-348.3) compared with endoscopically operated patients with adenoma found on HPE. A lower immunoreactivity score for NR4A1 was found to correlate with higher remission rates (P = 0.074). However, none of the molecular markers studied (i.e., c-Fos, c-Jun, GLI-1, pMEK, and p44) showed a significant correlation with the preoperative cortisol values. CONCLUSIONS: The remission rate after primary surgery is higher than that after revision surgery and is lower for pediatric patients than for adults. The postoperative day 1 plasma cortisol and adrenocorticotropic hormone levels are strong independent predictors of remission in the primary surgery cohort. An endoscopic approach with histopathological evidence of adenoma is associated with a higher remission rate; thus, endoscopy should be the approach of choice for these patients with the goal of identification of an adenoma on HPE.

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review.

BACKGROUND: Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection. METHODS: A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described. RESULTS: The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2 years (range 2 months to 18 years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired. CONCLUSION: IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis.  Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.

Novel EGFR mutations in diffuse midline gliomas using cost-effective strategies: A report of 2 cases.

Background: Diffuse midline gliomas (DMGs) are malignant tumors predominantly affecting children, often leading to poor outcomes. The 2021 World Health Organization classification identifies 3 subtypes of DMGs, all characterized by the loss of H3K27 trimethylation. Here, we report 2 cases of DMG with Epidermal Growth Factor Receptor (EGFR) mutations within exon 20, contributing to the understanding of the molecular complexity of these pediatric brain tumors. Methods: An economical immunohistochemical panel was designed to aid in the diagnosis of most DMGs in resource-constrained regions. Sanger sequencing was employed to identify rare EGFR mutations in exon 20 of 2 cases. Results: Molecular analyses of 2 cases of DMG revealed novel EGFR mutations within exon 20. These mutations were identified using cost-effective diagnostic approaches. The presence of EGFR mutations expands the molecular landscape of DMGs and highlights the genetic heterogeneity within this tumor entity. Conclusions: These findings underscore the molecular heterogeneity of DMGs and the significance of identifying novel mutations, such as EGFR mutations in exon 20. Further research into the molecular mechanisms underlying DMGs is warranted to advance therapeutic strategies and improve outcomes for pediatric patients.

Acute bilateral sensorineural hearing loss as presentation of leptomeningeal metastases.

We describe a rare occurrence of bilateral acute severe sensorineural hearing loss in a middle-aged man that heralded the diagnosis of metastatic gastric cancer.

Evaluation of KIAA1549::BRAF fusions and clinicopathological insights of pilocytic astrocytomas.

BACKGROUND: Pilocytic astrocytoma (PAs) represents a significant portion of childhood primary brain tumors, with distinct histological and radiological features. The prevalence of KIAA1549::BRAF fusion in PAs has been well-established, this study aims to assess the prevalence of KIAA1549::BRAF fusions and explore their associations with tumor characteristics, radiological findings, and patient outcomes in PAs. METHODS: Histologically confirmed cases of PAs from a 5-year period were included in the study. Demographic, histopathological, and radiological data were collected, and immunohistochemistry was performed to characterize tumor markers. FISH and qRT-PCR assays were employed to detect KIAA1549::BRAF fusions. Statistical analyses were conducted to examine associations between fusion status and various other parameters. RESULTS: Histological analysis revealed no significant differences in tumor features based on fusion status. However, younger age groups showed higher fusion prevalence. Radiologically, fusion-positive cases were distributed across different tumor subtypes SE, CWE and NCWE. Survival analysis did not demonstrate a significant impact of fusion status on overall survival, however most cases with recurrence and death harboured KIAA1549::BRAF fusion. Of 200 PAs, KIAA1549::BRAF fusions were detected in 64 % and 74 % of cases via qRT-PCR and FISH, respectively. Concordance between the two platforms was substantial (86 %). CONCLUSION: KIAA1549::BRAF fusions are prevalent in PAs and can be reliably detected using both FISH and qRT-PCR assays. Cost considerations suggest qRT-PCR as a more economical option for fusion detection in routine clinical practice.