Bifurcation dissecting aneurysm: tips and tricks to differentiate from saccular aneurysm.

Bifurcations are a common site for saccular aneurysms, but rarely can be a site for dissecting aneurysms. Identification of these aneurysms is extremely important because the management plan depends on it. We describe a rare case of a ruptured dissecting aneurysm at the right ICA bifurcation in a pre-teen child which posed a diagnostic dilemma but ultimately was successfully managed with flow diversion.

COVID-19 associated multisystemic mucormycosis from India: a multicentric retrospective study on clinical profile, predisposing factors, cumulative mortality and factors affecting outcome.

PURPOSE: The clinical course of COVID-19 has been complicated by secondary infections, including bacterial and fungal infections. The rapid rise in the incidence of invasive mucormycosis in these patients is very much concerning. COVID-19-associated mucormycosis was detected in huge numbers during the second wave of the COVID-19 pandemic in India, with several predisposing factors indicated in its pathogenesis. This study aimed to evaluate the epidemiology, predisposing factor, cumulative mortality and factors affecting outcomes among the coronavirus disease COVID-19-associated mucormycosis (CAM). METHODS: A multicenter retrospective study across three tertiary health care centers in Southern part of India was conducted during April-June 2021. RESULTS: Among the 217 cases of CAM, mucormycosis affecting the nasal sinuses was the commonest, affecting 95 (44%) of the patients, orbital extension seen in 84 (38%), pulmonary (n = 25, 12%), gastrointestinal (n = 6, 3%), isolated cerebral (n = 2) and disseminated mucormycosis (n = 2). Diabetes mellitus, high-dose systemic steroids were the most common underlying disease among CAM patients. The mucormycosis-associated case-fatality at 6 weeks was 14%, cerebral or GI or disseminated mucormycosis had 9 times higher risk of death compared to other locations. Extensive surgical debridement along with sequential antifungal drug treatment improved the survival in mucormycosis patients. CONCLUSION: Judicious and appropriate management of the predisposing factor and factors affecting mortality associated with CAM with multi-disciplinary approach and timely surgical and medical management can be much helpful in achieving a successful outcome.

ISNO consensus guidelines for practical adaptation of the WHO 2016 classification of adult diffuse gliomas.

INTRODUCTION: Recent advances in the molecular biology of adult diffuse gliomas have brought about a paradigm shift in their diagnostic criteria, as witnessed in the World Health Organization (WHO) 2016 guidelines for central nervous system tumors. It is now mandatory to perform several molecular tests to reach a definitive integrated diagnosis in most of the cases. This comes with additional cost and higher turnaround time, which is not always affordable in developing countries like India. In addition, the non-uniform distribution of advanced research and diagnostic testing centers adds to the difficulty. METHODS: The Indian Society of Neuro-oncology (ISNO) multidisciplinary expert panel consisting of neuropathologists, neurosurgeons, and radiation/medical oncologists convened to prepare the national consensus guidelines for approach to diagnosis of adult diffuse gliomas. RESULTS: Algorithms for arriving at an integrated diagnosis of adult diffuse gliomas predominantly using immunohistochemistry and with minimum possible additional molecular testing were agreed upon, thus addressing the problems of cost, accessibility, and turnaround time. Mandatory and optional tests were proposed for each case scenario. CONCLUSION: This document represents the consensus of the various neuro-oncology disciplines involved in diagnosis and management of patients with adult diffuse gliomas. The article reflects a practical adaptation of the WHO recommendations to suit a resource constrained setup.

Necrotizing fasciitis.

Necrotizing fasciitis (NF) is among the most challenging surgical infections faced by a surgeon. The difficulty in managing this entity is due to a combination of difficulty in diagnosis, and also of early as well as late management. For the patient, such a diagnosis means prolonged hospital stay, painful dressings, an extended recovery, and in some unfortunate cases even loss of limb or life. Necrotizing fasciitis is a fairly common condition in surgical practice in the Indian context resulting in a fairly large body of clinical experience. This article reviews literature on MEDLINE with the key words "necrotizing," "fasciitis," and "necrotizing infections" from 1970, as well as from articles cross referenced therein. The authors attempt to draw comparisons to their own experience in managing this condition to give an Indian perspective to the condition.

Reporting a Case of Cervical Vertebral Body Primary Malignant Melanoma-A Rare Entity.

Melanomas of vertebral body are usually metastatic lesions. Isolated vertebral body melanomas are rare may be due to unknown primary. Only threesuch cases havebeen reported in literature. We are reporting a 30-year-old female presented with progressive quadriparesis and bladder involvement. On evaluation, an extradural lesion at the C4-5 level with the destruction of C4 vertebral body and anterior in the prevertebral space seen with areas of blooming. The patient underwent surgery and biopsy was suggestive of melanoma. On further evaluation, we could find any other lesion in the body. The lesion can be either metastatic with unknown primary or primarily arising from vertebrae. Primary vertebral body melanomas are rare, surgical decompressions followed by immunotherapy may prolong the survival in this patients.

Recurrence in medulloblastoma - influence of clinical, histological and immunohistochemical factors.

INTRODUCTION: The therapy for medulloblastomas has gone through several changes over the years. Research has shown that patients with medulloblastoma cannot be satisfactorily stratified into risk groups based on clinical and therapeutic factors alone. AIM: To study the clinical, histological features and expression of immunohistochemical markers in medulloblastoma, and to correlate these features with recurrence rates. MATERIALS AND METHODS: Sixty-three cases of medulloblastomas operated at our institute from 1996-2003 were selected and retrospectively analyzed for therapy received, histological features and immunohistochemical expression of GFAP, synaptophysin, erb B2, p53, Bcl-2 and Trk-C along with assessment of MIB-1 labeling index(LI). RESULTS: The mean age of the 63 cases at presentation was 11.6 yrs. Near/Gross- total resection was achieved in 74.6% (47/63) of the cases. CONCLUSION: We propose a clinical-histological-immunohistochemical' model for medulloblastoma where extent of resection, administration of chemotherapy, presence of gross anaplasia and c-erb B2 overexpression status are the most important predictors of recurrence rates.

Intraoperative Transcranial Embolization following Decompressive Craniectomy for a Dural Arteriovenous Fistula Presenting with Acutely Raised Intracranial Pressure.

We report the clinical details, imaging findings, and management of a 58-year-old female who presented with rapidly deteriorating neurological function and acute raise in intracranial pressure secondary to posterior fossa dural arteriovenous fistula (DAVF). In this report, we present the emergency management of the DAVF with single-stage combined surgical decompressive craniotomy and direct transcranial embolization.

Utility and pitfalls of high field 3 tesla intraoperative MRI in neurosurgery: A single centre experience of 100 cases.

OBJECTIVE: In India, few centers are using 1.5 Tesla intraoperative MRI systems. We are using a 3 Tesla iMRI system. We share our initial experience of 3T iMRI in neurosurgical procedures with evaluation of its utility and pitfalls. METHODS: A prospective observational study conducted between August 2017 to July 2018 at Yashoda Hospital, Secunderabad. All patients undergoing iMRI guided resection of intracranial SOL were included. RESULTS: First 100 patients with various intracranial SOLs were included. The mean time required in shifting and image acquisition was 85.6 minutes in first 20 cases which was reduced to 37.4 minutes in next the next cases. Primary GTR was achieved in 44% cases, and residues were detected in 56%, secondary GTR was achieved in 37% cases, and surgery was discontinued in 19%. Maximum residues were detected in intraaxial sols and pituitary macroadenomas. No major iMRI associated complications were seen, minor issues involving transportation and minor contact burns were seen in 4 cases, insignificant anesthetic procedure related complications in 19 cases. CONCLUSION: As per our experience iMRI is an excellent tool to guide and improve the extent of safe resection by 37% in brain tumor surgeries. Good image quality, less time for image acquisition was observed advantages of 3T system. iMRI success depends on multidepartment coordinated teamwork and multiple iterations of the process to smoothen the workflow.

Novel glioblastoma markers with diagnostic and prognostic value identified through transcriptome analysis.

PURPOSE: Current methods of classification of astrocytoma based on histopathologic methods are often subjective and less accurate. Although patients with glioblastoma have grave prognosis, significant variability in patient outcome is observed. Therefore, the aim of this study was to identify glioblastoma diagnostic and prognostic markers through microarray analysis. EXPERIMENTAL DESIGN: We carried out transcriptome analysis of 25 diffusely infiltrating astrocytoma samples [WHO grade II--diffuse astrocytoma, grade III--anaplastic astrocytoma, and grade IV--glioblastoma (GBM)] using cDNA microarrays containing 18,981 genes. Several of the markers identified were also validated by real-time reverse transcription quantitative PCR and immunohistochemical analysis on an independent set of tumor samples (n = 100). Survival analysis was carried out for two markers on another independent set of retrospective cases (n = 51). RESULTS: We identified several differentially regulated grade-specific genes. Independent validation by real-time reverse transcription quantitative PCR analysis found growth arrest and DNA-damage-inducible alpha (GADD45alpha) and follistatin-like 1 (FSTL1) to be up-regulated in most GBMs (both primary and secondary), whereas superoxide dismutase 2 and adipocyte enhancer binding protein 1 were up-regulated in the majority of primary GBM. Further, identification of the grade-specific expression of GADD45alpha and FSTL1 by immunohistochemical staining reinforced our findings. Analysis of retrospective GBM cases with known survival data revealed that cytoplasmic overexpression of GADD45alpha conferred better survival while the coexpression of FSTL1 with p53 was associated with poor survival. CONCLUSIONS: Our study reveals that GADD45alpha and FSTLI are GBM-specific whereas superoxide dismutase 2 and adipocyte enhancer binding protein 1 are primary GBM-specific diagnostic markers. Whereas GADD45alpha overexpression confers a favorable prognosis, FSTL1 overexpression is a hallmark of poor prognosis in GBM patients.

Mixed-density extradural hematomas on computed tomography-prognostic significance.

BACKGROUND: It has been variably reported that patients who acutely present with low- or mixed-density blood on CT scan are associated with poor clinical outcome. The aim of the study was to correlate the presence or absence of mixed density within EDHs on CT scanning with the clinical outcome. METHODS: This is a retrospective study of a total of 109 patients with EDHs who were operated on from August 2001 to August 2002. The CT scans were reviewed and classified into 2 categories-predominantly hyperdense and mixed density. This was correlated with clinical details and outcome. RESULTS: In all, 43.2% (16/37) of patients in the mixed-density category presented with GCS of no more than 8 as compared with 23.6% (17/72) of patients in the hyperdense group (P < .05). Mean hematoma volume in the mixed-density group was 72 cm(3) as compared with 42 cm(3) in the hyperdense group (P < .05). Mortality rate was significantly higher in the mixed-density category (21.6% vs 4.2%, P < .05). CONCLUSION: The study portends mixed density in EDH as a potent poor prognostic indicator. The mixed density of the clot probably indicates that the clot is rapidly increasing in size and requires even earlier and more aggressive treatment.

Fatal intratumoral hemorrhage in posterior fossa tumors following ventriculoperitoneal shunt.

We report on two patients with rare major intratumoral hemorrhage following ventriculoperitoneal shunt in posterior fossa tumors. A 28-year-old woman with a midline posterior fossa lesion, whose imaging features suggested a fourth ventricular ependymoma with obstructive hydrocephalus, was subjected to a right ventriculoperitoneal shunt. Her consciousness deteriorated, and she experienced massive intratumoral hemorrhage and later died. An eight-year-old girl presented with raised intracranial pressure and ataxia caused by vermian astrocytoma with obstructive hydrocephalus. She also developed a massive tumor bleed following a ventriculoperitoneal shunt and was subjected to emergency decompression of the tumor with the bleeding. She remained vegetative at discharge and died 18 months later. Intratumoral hemorrhage is a rare but important cause of morbidity and mortality in patients with posterior fossa tumors who undergo ventriculoperitoneal shunt.

Targeted endovascular treatment of haemorrhagic posterior fossa proliferative angiopathy.

Cerebral proliferative angiopathy (CPA) is a rare vascular abnormality and separate from "classical" brain arteriovenous malformations. Haemorrhage due to proliferative angiopathy is rarely reported. We describe the clinical features, imaging findings and targeted endovascular management for a 12-year-old boy having proliferative angiopathy of the posterior fossa presenting with haemorrhage. Targeted endovascular embolisation in CPA is not previously described in the literature. The optimal treatment options for haemorrhagic CPA are debatable, and we wish to highlight the role of targeted treatment for culprit focal lesion demonstrable on imaging.

Rete mirabile of posterior inferior cerebellar artery: A rare cause of subarachnoid hemorrhage.

Rete mirabile is a congenital aberrant arterial collateral network resulting from abnormal embryonic activity during native vessel formation. Even though frequently observed in mammals, this entity is rare in humans except when there is hypoplasia, aplasia or narrowing of native vessels. Rete mirabile is reported involving internal carotid and vertebral arteries. Rete mirabile of the posterior inferior cerebellar artery (PICA) is not described in the literature. We report the clinical presentation, imaging findings and management of PICA rete mirabile formation with subarachnoid hemorrhage.

Glioblastoma multiforme after stereotactic radiotherapy for acoustic neuroma: case report and review of the literature.

Indications for the use of radiotherapy in the management of a variety of benign intracranial neoplastic and nonneoplastic pathologies are increasing. Although the short-term risks are minimal, the long-term risks of radiation-induced de novo secondary neoplasms or malignant progression of the primary benign tumor need to be considered. There are currently 19 reported cases of tumors linked with stereotactic radiotherapy/radiosurgery, to which we add our second institutional experience of a patient who succumbed to a glioblastoma multiforme (GBM) after stereotactic radiotherapy for an acoustic neuroma (AN). Review of these 20 cases revealed 10 de novo secondary tumors, of which eight were malignant, with six being malignant gliomas. The majority of the cases (14 of 20) involved AN, with most being in patients with neurofibromatosis-2 (NF2; 8 of 14), reflecting the large numbers and long-term use of radiotherapy for AN. Accelerated growth of the primary benign AN, some 2 to 6 years after focused radiotherapy, was found in six of eight NF2 patients, with pathological verification of a malignant nerve sheath tumor documented in most. The exact carcinogenic risk after radiotherapy is unknown but likely extremely low. However, the risk is not zero and requires discussion with the patient, with specific consideration in young patients and those with a cancer predisposition.

Malignant and benign ganglioglioma: a pathological and molecular study.

Gangliogliomas are generally benign tumors, composed of transformed neuronal and glial elements, with rare malignant progression of the glial component. The current study of a rare case of a woman harboring a ganglioglioma with areas of malignant transformation addresses two fundamental questions: (1) Are the ganglioglioma and its malignant component clonal in origin? (2) What are the genetic alterations associated with the initiation and subsequent malignant progression of ganglioglioma? By using the human androgen receptor gene (HUMARA) assay, we found the ganglioglioma and the malignant component to be clonal in origin, suggestive of initial transformation of a single neuroglial precursor cell with subsequent malignant progression. Conventional and array comparative genomic hybridization (approximately 2.5-Mb resolution) analyses found chromosomal losses to be predominant in the benign areas of the ganglioglioma, with gains more prevalent in the malignant component. Regions of chromosomal loss, postulated to harbor genes involved in the initiation of ganglioglioma, included 1p35-36, 2p16-15, 3q13.1-13.3, 3q24-25.3, 6p21.3-21.2, 6q24-25.2, 9p12, Xp11.3-11.22, and Xq22.1-22.3. Direct analysis demonstrated loss of p19 expression and p53 mutation in the malignant areas, highly suggestive of these alterations being involved in the malignant progression of the ganglioglioma. Additional chromosomal alterations specific to the malignancy involved gains on 1p35-34.2, 2q24.1-32.3, 3q13.1-13.3, 6q13-16.2, 7q11.2-31.3, 8q21.1-23, 11q12-31, and 12q13.2-21.3. This molecular-pathological study has provided insight into the pathogenesis of gangliogliomas and associated rare malignant progression. Deciphering the specific genes residing in these chromosomal regions may further our understanding of not only these rare tumors but also the more common gliomas.

Critical neurological structure sparing radiosurgery of vestibular schwannoma: dosimetric comparison of different techniques and dose prescription methods.

AIM: To investigate potential sparing of critical neurological structures (CNSs) during radiosurgery of vestibular schwannoma (VS) employing different techniques and dose prescription methods. MATERIALS AND METHODS: Fused CT and MRI datasets of eight patients with unilateral VS representing a wide range of target volume (0.48 to 12.08 cc; mean = 3.56 cc), shape and proximity to CNSs such as cochlea, trigeminal nerve and brainstem were re-planned employing static conformal field (SCF), dynamic conformal arc (DCA) and intensity modulated radiosurgery (IMRS) techniques. For every patient, five plans were created for a fixed margin dose of 12 Gy prescribed at 80% in three plans (SCF_80%, DCA_80%, and IMRS_80%) and 50% in another two plans (SCF_50% and DCA_50%). All plans were compared using standard dosimetric indices. RESULTS: Primary goal of every plan to cover ≥99% of target volume with 12 Gy was fulfilled for all patients with minimum significant dose to target (D99) ≥11.99 Gy. Best conformity index (CI Paddick = 0.62 ± 0.12) was observed in SCF_80% and DCA_80% plans whereas; sharpest dose gradient index of 3.40 ± 0.40 was resulted from DCA_50%. All five plans resulted similar maximum dose to brainstem (11.04 ± 2.23 to 11.53 ± 1.10 Gy), cochlea (9.02 ± 1.79 to 10.15 ± 1.26 Gy) and trigeminal nerve (11.55 ± 1.38 to 12.19 ± 2.12 Gy). Among 80% prescription plans, IMRS_80% reduces mean and D5 (P < 0.05) to all CNSs. Prescription of dose at 50% isodose sharpened the dose gradient and significantly (P < 0.05) reduced mean dose and D5 to all CNSs at the cost of target conformity (P = 0.01). Mean dose to cochlea and trigeminal nerve were least at 4.53 ± 0.86 and 6.95 ± 2.02 Gy from SCF_50% and highest at 6.65 ± 0.70 and 8.40 ± 2.11 Gy from DCA_80% plans respectively. CONCLUSION: This dosimetric data provides a guideline for choosing optimum treatment option and scope of inter institutional dosimetric comparison for further improvement in radiosurgery of Vestibular Schwannoma (VS).

A fourteen gene GBM prognostic signature identifies association of immune response pathway and mesenchymal subtype with high risk group.

BACKGROUND: Recent research on glioblastoma (GBM) has focused on deducing gene signatures predicting prognosis. The present study evaluated the mRNA expression of selected genes and correlated with outcome to arrive at a prognostic gene signature. METHODS: Patients with GBM (n = 123) were prospectively recruited, treated with a uniform protocol and followed up. Expression of 175 genes in GBM tissue was determined using qRT-PCR. A supervised principal component analysis followed by derivation of gene signature was performed. Independent validation of the signature was done using TCGA data. Gene Ontology and KEGG pathway analysis was carried out among patients from TCGA cohort. RESULTS: A 14 gene signature was identified that predicted outcome in GBM. A weighted gene (WG) score was found to be an independent predictor of survival in multivariate analysis in the present cohort (HR = 2.507; B = 0.919; p<0.001) and in TCGA cohort. Risk stratification by standardized WG score classified patients into low and high risk predicting survival both in our cohort (p = <0.001) and TCGA cohort (p = 0.001). Pathway analysis using the most differentially regulated genes (n = 76) between the low and high risk groups revealed association of activated inflammatory/immune response pathways and mesenchymal subtype in the high risk group. CONCLUSION: We have identified a 14 gene expression signature that can predict survival in GBM patients. A network analysis revealed activation of inflammatory response pathway specifically in high risk group. These findings may have implications in understanding of gliomagenesis, development of targeted therapies and selection of high risk cancer patients for alternate adjuvant therapies.