Pediatric primary intracranial angiosarcoma with epithelioid differentiation: a surgeon's dilemma.

Angiosarcoma is a rare form of soft tissue sarcoma originating from endothelial tissue, accounting for < 1% of all sarcomas. Primary epithelioid angiosarcomas of the central nervous system (CNS) are even more elusive, with only four reports described in the literature. In this article, we describe the first case in pediatric population, with a brief literature review regarding this entity. A 13-year-old girl presented to emergency services with raised intracranial pressure. MRI demonstrated a heterogenous lesion in the temporal lobe. She underwent emergency craniotomy and subtotal excision of the tumor. Eventually the patient developed multiple infarcts and succumbed post operatively. Pre-operative diagnosis on radiology is difficult considering the rarity of this entity and heterogeneity in radiological appearance. One needs to have a high degree of suspicion to consider angiosarcoma as a radiological differential. Overall prognosis remains poor. Early adjuvant treatment may improve overall survival.

Role of Cavernous Sinus Extension and MRI T2 Hypointensity in the Extent of Resection following Trans-Sphenoidal Surgery for Giant Pituitary Adenomas.

Background: Giant pituitary adenomas (GPA) pose a significant neurosurgical challenge. In this study, we present an experience of 60 consecutive patients with GPA operated by trans-sphenoidal surgery (microscopic/endoscopic). Objective: The aim of this study was to elucidate preoperative factors predicting extent of resection (EOR) following transsphenoidal surgery for GPA. Materials and Methods: The study was a retrospective evaluation of 60 adult patients (>18 years) with GPA operated over a decade. The patients were analyzed for their clinical presentation, EOR, radiology, visual outcome, endocrinological outcome, and complications. Results: The mean age of the cohort was 41.5 ± 13.3 years, mean tumor volume was 38.2 ± 19.1 cm3, and mean follow-up was 30.6 ± 21.7 months. Improvement in vision was seen in forty patients (66.7%), while another 16 (26.7%) had stable vision as prior to surgery. Duration of visual symptoms was found to be statistically significantly associated with postoperative improvement of vision (P = 0.001). Twenty-four patients (40.0%) underwent subtotal resection (STR), while the remaining 36 patients (60.0%) underwent either gross total or near-total resection. Factors associated with STR were retrosellar (P = 0.04), subfrontal (P = 0.02), Knosp 3,4 cavernous sinus extension (P = 0.03), and MRI T2 hypointensity (P = 0.02). During follow-up, eight patients (13.3%) had radiological evidence of growth of residual tumor. Conclusions: Most cases of GPA can be adequately handled by trans-sphenoidal surgery. The presence of retrosellar, subfrontal, cavernous sinus extensions, and T2 hypointensity should alert the surgeon for likelihood of STR, postoperative residual tumor hemorrhage and need for second-stage endonasal or transcranial surgery. However, utilization of extended endoscopic route has allowed good debulking of tumors having subfrontal and parasellar extensions, which was not possible previously using traditional microscopic transsphenoidal surgery.

Primary Dural Acute Lymphoblastic Leukaemia/Lymphoma Masquerading Meningioma: Report of a Case and Review of Literature.

Acute lymphoblastic leukaemia/lymphoma (ALL) is a systemic disease which primarily involves bone marrow or lymphoid organs. Extranodal presentation of ALL is uncommon, and ALL presenting as a dural mass is exceedingly rare. Here we present a case of primary dural B-cell ALL which was preoperatively diagnosed as meningioma on clinico-radiological grounds. A 27-year-old female patient presented with left hemicranial headache for one month's duration along with progressive vision loss of in both eye and altered behaviour. Contrast enhanced magnetic resonance imaging (CE-MRI) suggest dural based mass with dural tail sign. Histopathological examination of the resected specimen revealed B-cell ALL. Further systemic investigations didn't suggest any peripheral blood, bone marrow or lymph node involvement. To the best of our knowledge, only two cases of primary dural ALL have been reported in the literature so far. This report highlights the diagnostic difficulty in extramedullary precursor lymphoid neoplasm.

Factors determining the requirement of surgical intervention and prognosis in cases of traumatic bifrontal contusions: A prospective observational study.

Background: Traumatic brain injury, being a notorious cause of mortality and morbidity across the globe, presents with a variety of lesions. One of the distinct patterns of injury is characterized by contusions of both frontal lobes, labeled "traumatic bifrontal contusions" (TBCs). TBC is often associated with the presence of significant edema and mass effect leading to rapid clinical deterioration after a usually benign presentation at the time of first evaluation. Formulating a management plan in a patient with TBC is often more difficult than in a patient with a major intracranial hematoma. Methods: A prospective observational study with aims and objectives to identify predictors of an unfavorable outcome, analysis of the evolution of TBC, evaluation of the specific indications for surgery, and determination of the prognosis. All head trauma patients harboring bifrontal contusions were included in the study. Patients with other associated operable injuries involving blunt trauma abdomen and orthopedic injuries, counter-coupe injuries, and obvious open fractures noted over calvaria were excluded from the study. Glasgow coma scale (GCS) was recorded during the first assessment, followed by non-contrast computerized tomography (NCCT) Head. Results: A total of 53 patients satisfying inclusion and exclusion criteria were included in the study. The average GCS score recorded before surgical intervention was 9. The mean and median best motor response noted was M5. The interval from the time of injury to the first NCCT of the brain at the study hospital ranged from 3 h to 163 h, averaging 17.66 h. The median category w.r.t Marshall's CT classification observed was "Diffuse Injury IV." The volume of the contusions in each scan was estimated, and the average anterior cranial fossa volume observed was 125 mL. "Upfront surgery" ("Bifrontal decompressive craniectomy" or "unilateral Fronto-Temporo-Parietal [FTP] decompressive hemicraniectomy") was carried out on the day of admission based on the findings on the first NCCT brain. About 49% of patients at presentation needed surgical intervention as per existing protocols. The duration of observation for patients who were initially observed but eventually had to undergo surgery ranged from 1 to 5 days, with an average observation period of 2 days. The duration of observation in those who did not subsequently need surgery ranged from 7 to 10 days, with an average duration of 9 days. Conclusion: What leads to the poorly predictable, delayed, and rapid deterioration that sets TBCs apart from other traumatic brain injuries is still unclear. Our study finds that having a low threshold for repeat CT imaging of the patient led to earlier identification of progression, and a low threshold for surgical intervention led to favorable outcomes.

Primary Hypofractionated Gamma Knife Radiosurgery for Giant Cavernous Sinus Hemangiomas.

Cavernous sinus hemangioma (CSH) are notoriously difficult to excise because of their location, propensity for profuse bleeding during surgery, and relationship to complex neurovascular structures. Radiosurgery offers an alternative treatment modality in cases of small and medium-sized CSH. However, no reports are available in the world literature detailing gamma knife radiosurgery (GKRS) in large (3-4 cm) and giant (>4 cm) CSH. Two patients with giant CSH (Tumor volume was 72.2 and 99.8 cm3, respectively) were treated with frame-based fractionated GKRS (5 Gy × 5 #). The treatment was done with Leksell Perfexion with frame in situ and interfraction interval of 24 h. The tumor was engulfing the optic apparatus, and chiasma could not be separated delineated. The patients were followed at 3 months interval with clinic-radiologic evaluation. Following GKRS, both patients showed remarkable clinical improvement in presenting complaints of headache and visual deterioration. Sixth nerve paresis recovered completely in case no. 1. Significant reduction in tumor volumes (85.1 and 75.6% respectively) was noticed in both the patients at 6 months follow-up radiology. Transient alopecia was noted in case 1 at 3 months follow-up that completely resolved by the 6 months. There was no complication till the last follow-up of 9 months. We report the first account of five fraction frame-based hypo fractionated GKRS for giant CSH. At an interval as short as 3 months, giant CSH shows remarkable clinical improvement. Primary hypofractionated GKRS may be considered an alternative effective modality in these difficult lesions with a favorable safety profile.

Vim stereotactic radiosurgical thalamotomy for drug-resistant idiopathic Holmes tremor: a case report.

We share our experience with stereotactic gamma knife thalamotomy (GKT) for medically refractory Holmes tremor (HT). A 22-year-old patient underwent gamma knife thalamotomy at ventrointermediate nucleus for disabling HT of the right upper limb. A single 4-mm isocenter was used to target the ventral intermediate nucleus with 130 Gy radiation. At 4 months follow up, we observed 84% improvement in his Fahn-Tolosa-Marin (FTM) rating scale with significant improvement in the right upper limb dystonic tremor. There was only subtle improvement in the ataxic component of the right lower limb. At 1 year after stereotactic GKT, there was sustained neurological improvement with no side effect, We present the stereotactic GKT as a treatment modality for drug-resistant HT. Moreover, it may be considered an alternate treatment modality especially in patients reluctant or contraindicated for any invasive surgical technique. CLINICAL TRIAL REGISTRATION NUMBER: Not required.

Lateral Ventricle Atypical Teratoid/Rhabdoid Tumor (AT/RT): Case Report and Review of Literature.

A 4-year-old male child presented with features of raised intracranial pressure due to tumor in the left lateral ventricle with shunt blockage. Ventriculoperitoneal shunt was done earlier (one month ago). Craniotomy and gross total excision of the tumor was achieved. Histopathological examination was suggestive of Atypical Teratoid/Rhabdoid tumor. Patient relatives were not compliant with the advice for adjuvant therapy and patient expired after three months of definitive surgery due to aggressive course of the disease. To the best of our knowledge only six cases of AT/RT of the lateral ventricle in pediatric population has been described in literature. The tumor may mimic radiologically with benign pathology and can have an aggressive course with poor outcome. Differential diagnosis of AT/RT must be kept in cases of lateral ventricle tumor in pediatric population.

COVID-19: changing patterns among neurosurgical patients from North India, efficacy of repeat testing, and inpatient prevalence.

OBJECTIVE: COVID-19 has affected surgical practice globally. Treating neurosurgical patients with the restrictions imposed by the pandemic is challenging in institutions with shared patient areas. The present study was performed to assess the changing patterns of neurosurgical cases, the efficacy of repeated testing before surgery, and the prevalence of COVID-19 in asymptomatic neurosurgical inpatients. METHODS: Cases of non-trauma-related neurosurgical patients treated at the Postgraduate Institute of Medical Education and Research (PGIMER) before and during the COVID-19 pandemic were reviewed. During the pandemic, all patients underwent a nasopharyngeal swab reverse transcription-polymerase chain reaction test to detect COVID-19 at admission. Patients who needed immediate intervention were surgically treated following a single COVID-19 test, while stable patients who initially tested negative for COVID-19 were subjected to repeated testing at least 5 days after the first test and within 48 hours prior to the planned surgery. The COVID-19 positivity rate was compared with the local period prevalence. The number of patients who tested positive at the second test, following a negative first test, was used to determine the probable number of people who could have become infected during the surgical procedure without second testing. RESULTS: Of the total 1769 non-trauma-related neurosurgical patients included in this study, a mean of 337.2 patients underwent surgery per month before COVID-19, while a mean of 184.2 patients (54.6% of pre-COVID-19 capacity) underwent surgery per month during the pandemic period, when COVID-19 cases were on the rise in India. There was a significant increase in the proportion of patients undergoing surgery for a ruptured aneurysm, stroke, hydrocephalus, and cerebellar tumors, while the number of patients seeking surgery for chronic benign diseases declined. At the first COVID-19 test, 4 patients (0.48%) tested were found to have the disease, a proportion 3.7 times greater than that found in the local community. An additional 5 patients tested positive at the time of the second COVID-19 test, resulting in an overall inpatient period prevalence of 1%, in contrast to a 0.2% national cumulative caseload. It is possible that COVID-19 was prevented in approximately 67.4 people every month by using double testing. CONCLUSIONS: COVID-19 has changed the pattern of neurosurgical procedures, with acute cases dominating the practice. Despite the fact that the pandemic has not yet reached its peak in India, COVID-19 has been detected 3.7 times more often in asymptomatic neurosurgical inpatients than in the local community, even with single testing. Double testing displays an incremental value by disclosing COVID-19 overall in 1 in 100 inpatients and thus averting its spread through neurosurgical services.

Impact of the COVID-19 Pandemic on Neurosurgical Practice in India: Results of an Anonymized National Survey.

BACKGROUND: The COVID-19 pandemic has created an unprecedented challenge for medical professionals throughout the world to tackle the rapidly changing scenario. The objective of this survey was to analyze the change in neurosurgical practice in India following the COVID-19 outbreak and assess its impact on practising neurosurgeons. MATERIALS AND METHODS: Between May 7th and 23rd, 2020, a validated questionnaire was circulated amongst practising neurosurgeons across the country by social media and e-mails, regarding changes in the patterns of patients seen, adaptations made in their practice, effect on surgeries performed, financial burden, and impact on their personal lives. The responses were kept anonymous and were analyzed for correlations between the changes observed and independent factors such as hospital affiliations, teaching professions, and neurosurgical experience. RESULTS: Our survey showed a drastic fall in the number of neurosurgical patients seen in the outpatient department (OPD) as well as the number of surgeries performed. A drop of 76.25% was seen in OPD patients (P = 0.000) and that of 70.59% in surgeries performed (P = 0.000). There was no uniformity among the neurosurgeons in the number of COVID-19 tests being done before elective/emergency surgery and in the use of protective gear while examining patients. Private practitioners were more affected financially as compared to those in the government sector. The pandemic has affected the research work of 53.23% of all respondents, with those in the teaching profession (70.96%) more affected than those in the non-teaching profession (24.67%). CONCLUSIONS: Evidence-based policies, screening COVID-19 tests with better sensitivity, and better-quality personal protective equipment kits in adequate numbers are required to protect our medical professionals from COVID-19. Mental health issues among neurosurgeons may also be an issue, this being a high risk speciality and should be closely watched for.

An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

BACKGROUND: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. MATERIALS AND METHODS: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. RESULTS: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. CONCLUSION: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

Neglected Traumatic Atlantoaxial Rotatory Dislocation in Adult: A Case Report.

Atlantoaxial rotatory dislocations (AARDs) are common in the pediatric population and rarely seen in adults. We describe a case of neglected AARD and subsequent management. A 25-year-old man developed a Fielding type 1 AARD following a road traffic accident. He was managed conservatively for 1.5 months before being referred to us. The patient underwent surgery 1.5 months after the accident. Closed reduction failed and C1-2 fixation with the Harms technique was performed after intraoperative reduction resulting in correction of deformity. Delay of treatment makes intraoperative reduction more difficult and increases the possibility of the chronic permanent change of neck muscles and ligaments. Hence, a high index of suspicion with a thorough clinical examination and judicious use of radiological investigations is paramount to the appropriate management of such cases.

Co-Occurrence of Craniopharyngioma and IgG4-Related Hypophysitis: An Epiphenomenon or a Mere Coincidence?

BACKGROUND: Craniopharyngiomas are sellar-suprasellar tumors that commonly present in children, with nonspecific symptoms of increased intracranial pressure, visual disturbances, and pituitary insufficiencies. Rarely has secondary hypophysitis (lymphocytic and xanthogranulomatous) been reported in association with craniopharyngioma. CASE DESCRIPTION: We have reported the case of a 16-year-old boy who had presented with gradually progressive diminution of vision in the right eye, intermittent headache, deceleration in growth velocity, and the lack of development of secondary sexual characteristics. Imaging revealed a sellar-suprasellar cystic lesion (3.8 × 3.1 × 3.5 cm) with calcifications. Laboratory tests revealed hypothyroidism, hypocortisolism, hypogonadism, and growth hormone deficiency. Craniopharyngioma was provisionally diagnosed. He underwent pterional craniotomy and gross total excision of the lesion. The excised tissue showed features of adamantinomatous craniopharyngioma, with a dense lymphoplasmacytic infiltrate and fibrosis involving the pituitary and dura mater. The lymphocytes were CD20-positive, and the plasma cells stained positive for IgG4 (50-60 IgG4-positive plasma cells/high power field). Consequently, the possibility of IgG4-related hypophysitis was considered in our patient. His serum IgG4 level was not elevated. Systemic involvement by IgG4-related disease was thoroughly ruled out. Fluorodeoxyglucose positron emission tomography/computed tomography did not show any clinically significant hypermetabolism anywhere in the body. At the 3-month follow-up examination, his headache had resolved. However, he had not regained vision in his right eye. Repeat imaging studies showed no residual tumor tissue. CONCLUSIONS: The present case might represent the first ever report, to the best of our knowledge, of secondary IgG4-related hypophysitis due to craniopharyngioma, or it might, perhaps, be a chance association of these 2 entirely different disease entities.

Drug Refractory Epilepsy - A Series of Lesions with Triple Pathology.

The associations between gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), and cortical dysplasias remain debatable. We report five cases of drug refractory epilepsy with temporal lobe lesions. On resection, histopathological examination showed distinctive areas of gangliogliomas and DNETs with cortical dysplasia. The coexistence of the above three lesions as distinct entities in a single lesion is virtually unknown. This points to the presence of a possible etiological relationship among them. Finally, we also delve into a plausible hypothesis for such a pathogenesis.

Stereotactic radiosurgery in hemangioblastoma: Experience over 14 years.

BACKGROUND: Although gamma knife has been advocated for hemangioblastomas, it is not used widely by neurosurgeons. OBJECTIVE: We review our experience over 14 years in an attempt to define the role of stereotactic radiosurgery (SRS) in the management of hemangioblastomas. PATIENTS AND METHODS: A retrospective study was conducted on all patients of hemangioblastoma who underwent SRS at our institute over a period of 14 years (1998-2011). Gamma knife plans, clinical history, and radiology were reviewed for all patients. RESULTS: A total of 2767 patients underwent gamma knife during the study period. Of these, 10 (0.36%) patients were treated for 24 hemangioblastomas. Eight patients (80%) had von Hippel-Lindau disease while two had sporadic hemangioblastomas. The median peripheral dose (50% isodose) delivered to the tumors was 29.9 Gy. Clinical and radiological follow-up data were available for eight patients. Of these, two were re-operated for persisting cerebellar symptoms. The remaining six patients were recurrence-free at a mean follow-up of 48 months (range 19-108 months). One patient had an increase in cyst volume along with a decrease in the size of the mural nodule. CONCLUSIONS: SRS should be the first option for asymptomatic hemangioblastomas. Despite the obvious advantages, gamma knife is not widely used as an option for hemangioblastomas.