Safety and Efficacy of Biopsy in Patients with Diffuse Intrinsic Pontine Gliomas.

BACKGROUND: Diffuse intrinsic pontine gliomas are aggressive tumors that carry a poor prognosis with a 2-year survival rate of <10%. The imaging appearance is often pathognomonic, and surgical biopsy is not mandatory to initiate treatment in children. Studies of biopsy samples provide insight into the disease's molecular pathobiology and open prospects for targeted therapy. This study was conducted to determine the diagnostic yield and safety of stereotactic biopsies. METHODS: This is a prospective observational study from a single tertiary health care center. All patients with clinical and radiological features diagnostic of diffuse intrinsic pontine gliomas (DIPGs) who underwent biopsy from July 2018 to June 2023 were included. Biopsies were performed using either stereotactic frame-based, frameless, or endoscopic techniques. RESULTS: A total of 165 patients with DIPGs were evaluated in the study period. The option of biopsy with its associated risks and benefits was offered to all patients. A total of 76 biopsies were performed in 74 patients (40 children and 34 adults, including 2 repeat biopsies). The median age was 15 years. Diffuse midline gliomas, H3K27M altered, was the most common histopathological diagnosis (85% pediatric and 55.9% adults). The diagnostic efficacy of the procedure was 94.7%. The complication rate was 10.8%, with no permanent neurological deficits due to surgery. There was no procedure-related mortality. CONCLUSIONS: Establishing the safety of the procedure could be an important step toward popularizing the concept, which might offer a better understanding of the disease. Brainstem eloquence and a lack of direct benefit to patients are the primary obstacles to brainstem biopsy.

Factors predicting recurrence in benign spinal nerve sheath tumors: A retrospective study of 457 patients from a single institution.

BACKGROUND: Benign Nerve sheath tumors (NST) comprise almost one-third of primary spinal tumours. The majority are sporadic. They have low rates of recurrence but an occasional recurrence may need re-surgery. The present study was designed to identify the variables that can predict the risk of their recurrence. METHODS: A retrospective chart review was done including all the histologically proven benign spinal NSTs operated between 2001 and 2019 in our institute. Demographic, operative and postoperative follow-up data were recorded. Recurrence was defined as local reappearance after definite surgical excision or symptomatic increase in size of a residual tumour on follow-up imaging studies. Statistical analysis was done to determine the significant variables associated with local recurrence. RESULTS: 457 patients with a median age of 38 years operated for 459 NSTs qualified for the study. The most frequent location of occurrence of tumours was found to be Low Cervical level (C3-C7 levels). Majority of Schwannoma were located intradurally while Neurofibroma were dumb-bell shaped and extradural. Most of the tumours had solid consistency. Post operatively, 7.7% patients developed complications. 7.8% tumours developed local recurrence after median period of 12 months. The patients developing recurrence were younger compared to nonrecurring tumors. On univariate analysis, male gender, Low cervical and Cervicothoracic junction location were associated with higher recurrence. On multivariate analysis, location at Cervicothoracic junction reached significance. CONCLUSION: Overall recurrence risk among all NST was 7.8% with a median progression free survival of 36 months. The location of tumour at cervicothoracic location was the significant risk factors for recurrence of tumour in our study.

Symptomatic Post-Radiosurgery Intratumoral Hemorrhage in a Case of Vestibular Schwannoma: A Case Report and Review of the Literature.

Gamma knife radiosurgery (GKRS) is considered an established treatment for vestibular schwannoma (VS) in selected patients. Spontaneous intratumoral hemorrhage in VS after GKRS is very rare. In this report, we present a 63-year-old gentleman who had right-side severe sensorineural hearing loss on MRI showing a right cerebellopontine angle tumor (volume 4.96 cm3) with an internal acoustic meatus extension. He underwent GKRS with the prescription dose of 12 Gy to the 50% isodose line, covering 4.66 cm3 (i.e., 94%) of the tumor. Ten days later, he experienced a symptomatic intra-lesional hemorrhage with a mass effect over the brainstem. When symptoms did not resolve after an initial conservative approach, he underwent surgical decompression of the lesion. Postoperatively, the patient had facial palsy but was free of disabling vertigo and ataxia. At the 6-month follow-up, he was doing well without any other complaints. Although rare, an intralesional bleed can occur after GKRS in VS and should be suspected when new severe symptoms develop immediately after therapy.

Improvement in Obsessive-Compulsive Disorder Following Right Anterior Temporal Lobectomy and Amygdalohippocampectomy in a Patient with Refractory Temporal Lobe Epilepsy with Right Mesial Temporal Sclerosis.

There are reports of co-occurrence of obsessive-compulsive disorder (OCD) in patients with temporal lobe epilepsy (TLE). We present a report of a patient with refractory TLE due to hippocampal sclerosis with concomitant OCD on pharmacotherapy for both. She underwent surgery for standard anterior temporal lobectomy with amygdalohippocampectomy and reported improvement in obsessive-compulsive symptoms subsequently. We seek to further evidence of interaction between the two conditions and argue to undertake future research exploration on the same.

Moyamoya disease: Experience with direct and indirect revascularization in 70 patients from a nonendemic region.

INTRODUCTION: Moyamoya disease (MMD) is a chronic cerebrovascular disease characterized by stenosis/occlusion of bilateral terminal internal carotid arteries with the development of collaterals at base of the brain. We describe our experience of treating 70 patients (133 involved hemispheres, 108 hemispheres operated) surgically. PATIENTS AND METHODS: Surgically treated patients with MMD from 2006 to 2014 were reviewed retrospectively. There were 70 patients (54 pediatric, 36 females, range 2-46 years). Seven had a unilateral disease. One hundred and eight of 133 hemispheres underwent surgery; 58 underwent combined revascularization (superficial temporal artery - middle cerebral artery bypass and encephalo-duro-arterio-myo-synangiosis [EDAMS]); and, 50 underwent an indirect revascularization (EDAMS). 17/23 adult brain hemispheres and 41/85 pediatric hemispheres underwent combined revascularization. RESULTS: The median follow-up was 15.9 months (range 3-62 months). The mean modified Rankin scale score at presentation and follow-up were 2.27 ± 1.034 and 1.80 ± 1.269, respectively. There was an overall significant improvement in the clinical status in the operated patients (P < 0.001) at follow-up. The clinical improvement (admission to follow-up) was better in pediatric patients compared to the adults (P < 0.001 vs. 0.769). The combined revascularization gave better clinical improvement than the indirect one (P = 0.024 vs. 0.0312). There were three postoperative strokes and one death. The morbidity rate was 2.8%, and the mortality rate, 1.4%. Other patients were symptom-free at the latest follow-up. Angiographic outcome did not statistically correlate with age, anastomosis type, or Suzuki grade, though there was trend toward better angiographic outcomes in patients who underwent a combined revascularization. CONCLUSION: Both the combined and indirect revascularization procedures are effective in treating MMD. Pediatric patients had a better clinical improvement after surgery than the adult patients . Patients undergoing combined revascularization had a better clinical status compared to those who only underwent indirect revascularization. Combined revascularization surgery should be the surgical strategy in all age groups as it is feasible in a significant proportion of pediatric patients too.