Background Brain and other central nervous systems (CNS) tumors are a heterogeneous group of neoplasms that are prevalent in all age groups and gender. The study aimed to investigate the pattern of distribution of World Health Organization (WHO) classified primary brain and other CNS tumors among different age groups and gender at a tertiary care center in Northeast India. This is the first study of CNS tumors from this region of the country. The data obtained can be useful for correct diagnosis, timely treatment, and management of CNS tumors in this area. It can also provide relevant information concerning research and funding for this disease. Data collection was done retrospectively from January 1996 to March 2022. Distribution frequency as per age, gender, histopathology type, and tumor location was estimated. Data analysis was performed using the SPSS software. Results A total of 1441 primary brain and other CNS tumors were examined in the study. There were 232 pediatric cases (16.1%) and 1209 adult cases (83.9%). The ratio of males to females was 1.4. The majority of the cases in the overall cohort were meningiomas (n = 346; 24%). Among the pediatric cohort, the maximum occurrence of gliomas, glioneuronal, and neuronal tumors (n = 68; 29.3%) was seen while meningiomas (n = 336; 27.8%) showed the highest prevalence in the adult cohort. There were 248 other distinct cases of CNS (17.2%) of which 62 (26.7%) were identified within the pediatric cohort and the remaining 186 (15.4%) were detected among the adult population. Most of the tumor sites was supratentorial areas (n = 759; 52.7%) with the next being spinal cord (n = 258; 18%). Conclusion This is the first study from Northeast India that highlights the prevalence of WHO-classified CNS tumors. With newer and advanced health care and diagnostic facilities, there is an increased incidence of CNS tumors in developing countries. Our study may help in understanding the epidemiological aspects and highlight the need for research, funding, and maintenance of a hospital-based tumor registry for this particular ailment.
Background: Hypoglossal nerve schwannomas are uncommon, benign, and slow growing tumors arising from Schwann cells of the peripheral nerve sheath. They are well-documented in adult population. To the best of our knowledge, dumbbell-shaped hypoglossal schwannomas are rare and only 40 cases have been described in literature till date. They are managed surgically which poses signicant challenges including preservation of facial nerve. A 57 year old female presented with periodic spells of headache Case presentation: , imbalance while walking, trouble swallowing, right-sided tongue atrophy, and chronic chest infection. She was subsequently diagnosed with right cerebello-pontine (CP) angle hypoglossal schwannoma with extracranial extension. The tumor was managed by surgery and was performed in two stages: Stage one involved right retromastoid-sub occipital (RMSO) craniectomy with gross total excision of the lesion in the right CP angle followed by endoscopic assisted transoral-transpharyngeal excision of the tumor in the neck. Post-surgery, the patient showed gradual improvement. She could walk independently, her headache sub-sided and she showed slow improvement of lower cranial nerve function. However, during last follow up, it was observed that atrophy on the right side of the tongue still persisted. This case highlights t Conclusion: he importance of accurate diagnosis and treatment of hypoglossal schwannoma. Complete surgical excision is the recommended treatment for hypoglossal schwannoma which subsequently lowers the risk of recurrence and gives a good prognosis
Purpose: Pilocytic astrocytoma (PA) is a central nervous system (CNS) World Health Organization (WHO) grade 1 glial tumor that is highly prevalent in children and young adults. The main aim of the study was to assess the frequency, clinicopathological features and treatment of PAs along with their immunohistochemical and molecular analyses. Methods: Approximately, 144 glial tumors were diagnosed in patients from 3 to 75 yrs of age from January 2015 to March 2022. The tumors were classified according to 2021 CNS WHO classification. Nine pediatric PA patients were identified and their clinical data analyzed. Immunohistochemistry (IHC), fluorescence in-situ hybridization (FISH) and molecular analysis using the real time polymerase chain reaction (RT-PCR) were performed for the PAs. Standard deviations were calculated using the Microsoft excel for statistical validations. Results: The mean age of the glioma patients was 41.7 yrs ± 18.2 with a male/female ratio of 1.3:1. The most common form of glial tumor was found to be astrocytoma CNS WHO grade 2 (31.9 %). Other frequently occurring tumors were astrocytoma CNS WHO grade 3 (29.2 %), astrocytoma CNS WHO grade 4 (13.9 %), diffuse astrocytoma CNS WHO grade 2 (7.4 %), PA CNS WHO grade 1 (6.9 %), and oligodendroglioma CNS WHO grade 3 (4.2 %). The pediatric PA cohort had a mean age of 9.2 yrs ± 4.9 with a male/female ratio of 2:1. Glial fibrillary acidic protein (GFAP) positive immunostaining and retention of transcriptional regulator ATRX expression was seen in all the tested PAs. None of the PAs showed isocitrate dehydrogenase (IDH1), MIB1, and tumor protein p53 expression. The KIAA1459-BRAF fusion was detected in four PAs. Surgical intervention with total or radical tumor excision was performed for the PA patients. Most PA patients exhibited improved condition post-surgery. Conclusion: With the advent of healthcare and newer diagnostic facilities there is increased incidence of glial tumors in developing countries. Combination of histological, immunohistochemical, and molecular analysis is very important for the diagnosis, accurate treatment, and prognosis of PA patients.
Spinal nerve root tumors can arise throughout the spine and at multiple levels, likely representing plexiform neurofibromas that grow from the nerve root into the intraspinal space either intradurally or epidurally and exit through the neural foramen, producing a dumbbell-shaped appearance. Although many cases of dumbbell-shaped extramedullary neurofibromas in the cervical spine have been reported, to the best of our knowledge, there are no reports of trident-shaped extramedullary neurofibromas. A 26-year-old woman presented with swelling over the right side of her neck. Diagnostic workup included magnetic resonance imaging (MRI) and contrast-enhanced computed tomography (CECT) of the neck, which revealed an intradural, extramedullary tumor mass at the right C2–C6 level with an extraspinal extension. Spinal cord compression or canal compromise is the most reliable indication for surgery. The solitary cervical neurofibroma was treated surgically in a single stage through laminoplasty and excision of the intradural tumor along with that of the neck component. This was performed without any complications. A single-stage double approach was adopted in this case. After total excision, the shape of the tumor was found to be more like a trident than a dumbbell. Hence, here we would like to suggest a new nomenclature for this neurofibroma, the trident neurofibroma.
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