Spinal Dermoid and Epidermoid Cyst: An Institutional Experience and Clinical Insight into the Neural Tube Closure Models.

Objectives The spinal dermoid and epidermoid cysts (SDECs) are rare entities comprising less than 1% of pediatric intraspinal tumors. The present study aims to extrapolate the clinicoradiological data, in order to identify the most plausible neural tube closure model in human and provide a retrospective representation from our clinical experience. Materials and Methods We collected the details of all histologically proven, newly diagnosed primary SDECs who underwent excision over the past 20 years. Secondary or recurrent lesions and other spinal cord tumors were excluded. Surgical and follow-up details of these patients as well as those with associated spinal dysraphism were reviewed. Clinical and radiological follow-up revealed the recurrence in these inborn spinal cord disorders. Results A total of 73 patients were included retrospectively, having a mean age of 22.4 ± 13.3 years, and 41 (56.2%) cases fell in the first two decades of life. Twenty-four (32.9%) dermoid and 49 (67.1%) epidermoid cysts comprised the study population and 20 of them had associated spinal dysraphism. The distribution of SDECs was the most common in lumbosacral region ( n = 30) which was 10 times more common than in the sacral region ( n = 3). Bladder dysfunction 50 (68.5%) and pain 48 (65.7%) were the most common presenting complaints. During follow-up visits, 40/48 (83.3%) cases showed sensory improvement while 11/16 (68.7%) regained normal bowel function. There was no surgical mortality with recurrence seen in eight till the last follow-up. Conclusions The protracted clinical course of the spinal inclusion cysts mandates a long-term follow-up. The results of our study support the multisite closure model and attempt to provide a retrospective reflection of neural tube closure model in humans by using SDECs as the surrogate marker of neural tube closure defect.

A clinico-pathological and neuro-radiological study of angiomatous meningioma: Aggressive look with benign behaviour.

Angiomatous meningioma is a rare variant tumor classified as WHO grade 1 meningioma and accounts for about 2.1% of all meningioma. Their clinical presentation, surgical management, and prognosis are almost similar to the classical meningioma. Despite of benign nature and being comparatively small in size, they look aggressive on radiology images like massive peritumoral edema and intense contrast enhancement. Being a unique subtype of meningioma, the studies on angiomatous meningioma are very limited. In this cross sectional retrospective study, we described the clinical presentation, radiology, histopathological features and differential diagnosis of 30 cases of angiomatous meningioma from a single centre. The clinical parameters include demographic profile, symptoms and radiological findings including location, extent, pattern, histopathology with World Health Organization (WHO) grade-2016, extent of tumour excision, recurrence and surgical outcome. Incidence of angiomatous meningioma in our study was 2.46% with male predominance. The most common location in our study was convexity. 27 out of 30 had histopathology reports of angiomatous meningioma and 3 had lipoangiomatous meningioma. The high vascularity and disproportionate peri-tumoral edema makes it a surgical challenge for excision. The complications and surgical outcome were analysed. The radiological anticipation of tumour subtype, meticulous pre-operative planning and intraoperative precautions remains a key for success.

Malignant Meningitis Associated with Hydrocephalus.

Malignant meningitis (MM) is the diffuse involvement of the leptomeninges by infiltrating cancer cells, most frequently from lung and breast cancers. This review is aimed to discuss the current advances in the diagnosis and management of MM, along with management of MM-associated hydrocephalus. We reviewed the literature using PubMed and Google Scholar search engines, focusing on various recent randomized controlled trials and clinical trials on MM. Given the hallmark multifocal involvement, the clinical symptoms and signs are also random and asymmetric. There are three important pillars for establishing a diagnosis of MM: clinical examination, neuroimaging, and CSF cytological findings. Several factors should be considered in decision-making, including performance status, neurological findings (clinical, MRI, and CSF flow dynamic), and evaluation of the primary tumor (nature and systemic dissemination). Response Assessment in Neuro-Oncology (RANO) working group recommended the objective assessment of disease for evaluating the progression and response to therapy. Pillars of current management are mainly focal irradiation and intrathecal or systemic chemotherapy. Symptomatic hydrocephalus is managed with a ventriculoperitoneal shunt, lumboperitoneal shunt, or endoscopic third ventriculostomy as palliative procedures, providing significant improvement in performance scores in the limited survival time of patients with MM. Studies using novel therapeutic approaches, such as new biological or cytotoxic compounds, are ongoing. Despite the use of all the combinations, the overall prognosis remains grim; therefore, decision-making for treatment should predominantly be based on attaining an optimal quality of life.

Factors affecting seizure outcome in Long-term epilepsy associated tumors (LEATs) in children and young adolescents.

OBJECTIVE: Tumors with seizures as primary mode of presentation are collectively called Long-term epilepsy associated tumors (LEATs or Epileptomas). The overall survival is good so 'seizure outcome' becomes the primary goal rather than neuro-oncological outcome. METHODS: A retrospective analysis of our surgical database (2015-19) was done to find operated patients of intra-axial brain tumors with age less than 25-years and who had presented with seizures. RESULTS: The mean age at presentation was 16.44 years (SD + 6.82 years). Complex partial seizures/focal unaware seizures were the most common type of seizures encountered (n = 22) with mean duration of seizures was 49.50 months (SD + 31.04 months). The most common pathology was glioneuronal tumors (GNTs) (n = 17). Gross total resection (GTR) group had a significantly better seizure outcome as compared with the Subtotal resection (STR) group (p = 0.006). Presence of focal or partial seizure was a significant factor pointing towards a better seizure control (p = 0.005). CONCLUSION: The shorter duration of symptoms, partial/focal seizures and gross total excision were predictors of a good seizure-outcome. Age of the patient and the histopathology of the tumor does not affect seizure-outcome on comparing GNTs with non GNTs.