Clinical and histopathological spectrum of cranial small round cell tumors: An experience from a tertiary care center.

BACKGROUND: Small round cell tumors (SRCTs) are a group of malignant neoplasms with minimal or no differentiation, characterized by the presence of round cells with high nuclear-cytoplasmic ratio. Although SRCTs can occur in any part of the body, involvement of central nervous system (CNS) is uncommon. AIM: We aimed to study the clinicopathological spectrum of cranial SRCT diagnosed in our institute over a period of four years (2016-2019). MATERIAL AND METHODS: A retrospective review of medical records (2016-2019) with a morphological diagnosis of cranial SRCT was made. Both intra-axial and extra-axial tumors were included. A total of 60 cases were retrieved, and the clinical and histopathological features were studied. Special cytochemical staining and immunohistochemistry were performed, where needed. RESULTS: The mean age at presentation was 18.4 years (range, 1-60 years), with a male-to-female ratio of 2.5:1. The most common site was posterior fossa of brain (n = 28, 47%), followed by dorso-lumbar spine (n = 9, 15%). The most common type of tumor was medulloblastoma (n = 29, 48.3%), followed by Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) (n = 11, 18.3%), non-Hodgkin lymphoma (NHL) (n = 9, 15%), neuroblastoma (n = 3, 5%), and CNS embryonal tumor, NOS (n = 2, 3.3%). One case each of atypical teratoid rhabdoid tumor (ATRT), rhabdomyosarcoma, pineoblastoma, melanoma, rhabdomyosarcoma, and undifferentiated pleomorphic sarcoma was also documented. CONCLUSIONS: SRCTs have a variable age of presentation. Their incidence in CNS is low as compared to other organ systems. On light microscopy, the histopathology of these lesions is overlapping, posing a great diagnostic dilemma for the pathologist. The use of ancillary techniques like immunohistochemistry helps in arriving at the correct diagnosis. Treatment strategy and tumor prognosis also vary along the entire spectrum of SRCT, thus making exact characterization essential for proper management.

Epilepsy surgery in a pediatric population: a retrospective study of 129 children from a tertiary care hospital in a developing country along with assessment of quality of life.

PURPOSE: To assess the outcome of a pediatric population operated for drug-resistant epilepsy from a large tertiary care center in India. METHODS: Retrospectively: quality of life (QOL); prospectively: preoperative assessment included interictal EEG, MRI (as per epilepsy protocol), video-EEG. Ictal SPECT (with subtraction) and PET were performed when required. QOL scores were assessed using the HASS or SSQ for seizure severity, Quality of Life in Childhood Epilepsy (QOLCE) for QOL, and Child Behavior Check List (CBCL) for behavior. RESULTS: 142 were operated from January 2000 to June 2011 by the senior author. 118 patients with at least 1 year of follow-up were included in the study. Mean age at surgery was 9.8 ± 4.3 years. In addition, 40 patients underwent QOL assessment prospectively both before and after surgery. Mean duration of epilepsy was 5.3 ± 3.3 years. A class I outcome (Engel's) was seen in 79.5% patients, class II in 8.6%, class III in 10.7%, and class IV in 1 patient. As per surgical procedures, class I outcome in patients who underwent temporal resection, hemispherotomy and extratemporal resection was 76, 87 and 72%, respectively. QOL scores correlated with duration of seizures, epileptic encephalopathy and outcome of surgery, but not with side of surgery, age and sex. CONCLUSIONS: This study, the largest reported from India, has demonstrated satisfactory results for epilepsy surgery in children.

P53 and Ki-67 Expression in Primary Pediatric Brain Tumors: Does it Correlate with Presentation, Histological Grade, and Outcome?

CONTEXT: Pediatric brain tumors are a vexing problem for the neurosurgeon due to the fragile patient cohort. We attempt to find parameters which can help us to treat and prognosticate these patients in a better way. AIMS: This study aims to correlate clinical presentation, outcome, and histological grade with P53 and Ki-67 expression in primary pediatric brain tumors. SETTING DESIGN: This was a prospective, observational study. PATIENTS AND METHODS: Forty-seven patients with primary brain tumors in the age group 0-18 years were included in this study. Clinical presentation was noted. Patients were operated, and specimen was sent for histopathological and immunohistochemistry examination for p53 and Ki-67. The WHO classification of 2007 was used to grade the tumors. Follow-up was done at 3 and 6 months with Glasgow outcome score. Expression of p53 and Ki-67 in different tumors was correlated with clinical presentation, tumor grade and outcome. ANALYSIS METHOD: Statistical Package for Social Science version 17. P < 0.05 was considered statistically significant. RESULTS: There was statistically significant correlation between high tumor grade and high Ki-67 levels (P = 0.000). On post hoc analysis, there was a significant difference between p53 levels in Grade 1 and Grade 4 tumors. There was statistically significant correlation between neurological deficit and higher p53 levels (P = 0.040). There was statistically significant correlation between poor outcome and higher p53 (P = 0.034) and Ki-67 (P = 0.000) levels at 3 months follow-up which continued at 6 months. CONCLUSIONS: From this study, we conclude that p53 and Ki-67 expression in pediatric brain tumors is associated with poor outcome and correlates with tumor grade. Moreover, p53 expression correlates with neurological deficit.