IDH1, ATRX, p53, and Ki67 Expression in Glioblastoma patients: Their Clinical and Prognostic Significance-A Prospective Study.

Context Glioblastoma multiforme (GBM) is a malignant and aggressive primary brain tumor with a poor prognosis. This adverse prognosis is due to the tumor's tendency for advancement and recurrence caused by highly intrusive nature of the persisting GBM cells that actively escape from the main tumor mass into the surrounding normal brain tissue. On the basis of biomarker illustration, it can be classified into molecular subgroups. Aims (1) To determine the expression of IDH1, ATRX, p53, and Ki67 by immunohistochemistry, in a cohort of GBMs. (2) To determine whether altered protein expression of any of these growth-control genes in GBM will show association with patient survival. (3) To establish prognostically distinct molecular subgroups of GBM, irrespective of histopathological diagnosis. Results In this prospective observational study, 35 histologically diagnosed cases of glioblastoma were enrolled. The mean age at the time of presentation was 43.46 ± 17.25 years with a male:female ratio of 1.3:1. Of the 35 cases, microvascular proliferation was seen in 23 cases. Large foci of necrosis (>50%) were seen in 10 cases and 27 cases had mitotic count ≥ 5/high power field (HPF). Of 35 cases, 5 (14.3%) cases showed IDH1 immunopositivity and 30 (85.7%) cases were negative for IDH1. ATRX was retained in 24 (68.6%) cases, while it was lost in 11 (31.4%) cases. The p53 immunoexpression was seen in 31 (88.6%) cases, whereas p53 was negative in 4 (11.4%) cases. The overall median survival (OS) was 6 months. In two protein pairs, the three compositions were IDH1-/p53+ (74.3%), ATRX +/IDH1- (62.9%), and ATRX +/p53+ (57.1%). Combined three-protein immunohistochemical analysis revealed five different molecular variants. Also, 8.6% (3/35) of the samples had aberrant protein expression of all three proteins, i.e., ATRX-/p53 +/IDH1 + , while 11.4% (4/35) were wild-type protein expression group, i.e., ATRX +/p53-/IDH1-. Conclusion In patients with single protein expression, Kaplan-Meier survival analysis showed statistically better OS in IDH1 mutant glioblastomas. In cases with double protein pairs, IDH1/p53 revealed statistically significant association with better median OS. The survival analysis of patients with IDH1/ATRX/p53 protein combinations also denoted a better OS. Hence, GBM can be grouped into prognostically relevant subgroups using these protein expression signatures individually, as well as the combined protein expression signatures.

Epidemiological Profiling and Trends of Primary Intracranial Tumors: A Hospital-Based Brain Tumor Registry from a Tertiary Care Center.

Background and Objectives Hospital-based cancer registry is an essential tool for augmentation of the standard of care, administration motive, and resource for population-based cancer registries. Here, we presented hospital-based brain tumor registry (HBBTR) to outline a comprehensive epidemiological data, both clinical and histopathological, as well as trends of central nervous system tumors. In addition, we compare this data with national brain tumor data as well as an international brain tumor registry. Materials and Methods For the generation of this 7-year HBBTR data of all primary intracranial tumors operated, diagnosed, and registered at the Department of Pathology, Sawai ManSingh, between January 1, 2013 and December 31, 2019, was collected, analyzed, and compared with Tata Memorial Hospital, National Institute of Mental Health and Neurosciences, and Central Brain Tumor Registry of the United States. Results A total of 3,526 patients were of primary intracranial tumors. Out of which, male patients were 1,982 (56.2%), while 1,544 (43.8%) were female patients. Maximum proportion of tumors was in fifth decade. Overall, pediatric and adult patients constituted of 15.5 and 84.5% of the cases, respectively. Among all primary intracranial tumors, meningiomas (20%) were most common followed by glioblastoma multiformat (18%) and least common were germ cell tumors (0.1%) followed by pineal tumors (0.3%). In pediatric cohort astrocytic tumors (30.1%) are most common followed by embryonal tumors (20.8%), while in adults meningiomas (23.1%) were most common followed by glioblastomas (20.3%). Our registry showed similar trends of tumors with national data as compared with international data in median age of presentation. Conclusion This HBBTRs provide prevalence of primary intracranial tumors at a tertiary care center and could be a part of population-based registry.

Symptomatic Primary Ovarian Leiomyoma in a Postmenopausal Woman: A Rare Entity.

Leiomyomas are benign mesenchymal neoplasms mostly seen in the uterus and are one of the most common pelvic masses seen in women, but primary ovarian leiomyomas are rare among all the benign ovarian tumors, which account only for 0.5%-1%. The definitive diagnosis of such lesions is difficult prior to surgical excision, as there are no pathognomonic symptoms or characteristic imaging findings. Here, we report a case of primary ovarian leiomyoma with brief review of literature, highlighting the differential diagnosis of ovarian spindle cell lesions. The correct diagnosis of an ovarian leiomyoma requires identification of the nature of tumor as smooth muscle. An immunohistochemistry marker analysis is recommended for definitive diagnosis.

Extra-Axial Cerebellopontine Angle Medulloblastoma in an Infant.

Medulloblastoma is a common tumor of the posterior fossa, representing 20-25% of all pediatric neoplasms. It commonly occurs in the midline (cerebellar vermis) and rarely at the cerebellopontine angle. Most of them are intra-axial, and an extra-axial location of this tumor is very rare. Extra-axial cerebellopontine angle medulloblastoma is extremely uncommon and has never been reported in an infant. We report an extra-axial cerebellopontine angle medulloblastoma in a 1-year-old child.