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ABSTRACT: Muscular dystrophies are progressive, genetic disorders of muscle degeneration. The current gold standard for diagnosis is muscle biopsy or genetic studies. Muscle biopsy is an invasive procedure and genetic testing facilities are available only in a few centers. Thus, a diagnostic test that is easily available, simpler, and less invasive is desirable. Over the past 2 decades, skin biopsy has been evolving as a suitable option. Two cases of sarcoglycanopathy are described here, which have been correctly diagnosed by skin biopsy. Muscle biopsy has been used as the gold-standard diagnostic method. Skin biopsy can substitute for muscle biopsy as the preliminary diagnostic tool directing appropriate molecular testing. However, these results require validation in studies with an adequate sample size. This holds promise for the future when repeated biopsies will be required for evaluating protein rescue in trials of novel treatment options in these disorders.
Journal of child neurology 05/2013; · 1.59 Impact Factor
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ABSTRACT: Dystrophinopathies are diagnosed by genetic studies and muscle biopsy. Most centers have multiplex polymerase chain reaction facilities diagnosing 65% to 70% of dystrophinopathy cases. Muscle biopsy is a time-consuming, invasive procedure whereas skin biopsy is a simple procedure done under local anesthesia. The current study evaluated the diagnostic accuracy of skin biopsy in dystrophinopathy. Overall, 119 confirmed cases of muscular dystrophy (111 males and 8 females) were included in the final analysis, of which 100 (all males) were dystrophinopathy. Skin biopsy diagnosed dystrophinopathy in suspected muscular dystrophy patients with a sensitivity of 98% (92.3%-99.7%), specificity of 99% (93.7%-99.9%), positive predictive value of 94.7% (71.9%-99.7%), and negative predictive value of 90% (66.9%-98.2%). Skin biopsy can be used for screening dystrophinopathy in muscular dystrophy patients (high sensitivity and positive predictive value). It being a simple and minimally invasive procedure, histopathologic and molecular markers of disease progression and response to novel treatment options can be assessed serially.
Journal of child neurology 04/2013; · 1.59 Impact Factor
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ABSTRACT: Uterus-like mass is an extremely rare choristoma of müllerian origin arising in association with neural tube defects. In this article, we describe the case of a 9-year-old girl with spina bifida, a mass lesion within the conus, and a subcutaneous lipoma in the lumbosacral region. Histopathological examination of the conus lesion revealed a uterus-like structure comprising of endometrial glands and stroma surrounded by fascicles of smooth muscle. This case differs from the few previously described cases in absence of neurological symptoms and early age at diagnosis. Thorough histopathological examination of resected tissue is therefore recommended for the diagnosis of this rare entity, as it may not have a typical presentation in all instances.
Pediatric Neurosurgery 04/2013; · 0.70 Impact Factor
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Shipra Agarwal,
Mehar Chand Sharma,
Prerana Jha,
Pankaj Pathak,
Vaishali Suri, Chitra Sarkar,
Kunzang Chosdol,
Ashish Suri,
Shashank Sharad Kale,
Ashok Kumar Mahapatra,
Pankaj Jha
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ABSTRACT: Background
Mutations involving isocitrate dehydrogenase 1 (IDH 1) occur in a high proportion of diffuse gliomas, with implications on diagnosis and prognosis. About 90% involve exon 4 at codon 132, replacing amino acid arginine with histidine (R132H). Rarer ones include R132C, R132S, R132G, R132L, R132V, and R132P. Most authors have used DNA-based methods to assess IDH1 status. Preliminary studies comparing imunohistochemistry (IHC) with IDH1-R132H mutation-specific antibodies have shown concordance with DNA sequencing and no cross-reactivity with wild-type IDH1 or other mutant proteins. The present study compares results of IHC with DNA sequencing in diffuse gliomas.Materials and methodsFifty diffuse gliomas with frozen tissue samples for DNA sequencing and adequate tissue in paraffin blocks for IHC using IDH1-R132H specific antibody were assessed for IDH1 mutations.ResultsConcordance of findings between IHC and DNA sequencing was noted in 88% (44/50) cases. All 6 cases with discrepancy were immunopositive with DIA-H09 antibody. While in 3 of these 6 cases, DNA sequencing failed to reveal any mutations, R132L (arginine replaced by leucine) mutation was found in the rest 3 cases. Interestingly, of the immunopositive cases, 46.6% (14/30) showed immunostaining in only a fraction of tumor cells.ConclusionsIHC is an easy and quick method of detecting IDH1-R132H mutations, but there may be some discrepancies between IHC and DNA sequencing. Although there were no false-negative cases, cross-reactivity with IDH1-R132L was seen in 3, a finding not reported thus far. Because of more universal availability of IHC over genetic testing, cross-reactivity and staining heterogeneity may have bearing over its use in detecting IDH1-R132H mutation in gliomas.
Neuro-Oncology 03/2013; · 5.72 Impact Factor
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ABSTRACT: AIM: To mark out objectively, abnormal areas of MRI, PET and ECoG using neuronavigation so as to [1] enhance the accuracy of margins of the epileptogenic zone [2] understand the relationship of all the 3 modalities to each other. METHODS: Prospective study: 37 patients with intractable epilepsy due to lesional, neocortical pathologies from non-eloquent areas. Prior to surgery, fusion and transfer of MRI and PET images on to a neuronavigation system was performed. At surgery, this was correlated to intra-operative ECoG using the electrode as referential points. An objective score was created for every electrode point which was correlated with MRI, PET abnormality at the point. The extent of surgical resection mapped out using this data. RESULTS: From a total of the data recorded from 1280 electrode points, 23.5% were located over lesion. Over the lesions, 93% of PET and 66% of ECoG points were abnormal. Over the perilesional areas, 43% of PET and 45% of ECoG points were abnormal. Using this data for surgery, both lesional and peri-leisonal areas were resected; 33/37 patients had good outcome (25 Engel I, 8 Engel II) (mean follow up: 23.6 + 3.2 months; range: 18-31 months). CONCLUSION: Multimodal imaging and ECoG using this method seems to provide a better objective localization of the epileptogenic foci.
World Neurosurgery 02/2013; · 0.68 Impact Factor
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Sarat P Chandra,
Ajit Singh,
Nishant Goyal,
Rajender K Laythalling,
Manmohan Singh,
Sharad S Kale,
Manish S Sharma,
Ashish Suri,
Pankaj Singh,
Ajay Garg, Chitra Sarkar,
Bhawani S Sharma,
Ashok K Mahapatra
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ABSTRACT: OBJECTIVE: To describe management and outcome in a large cohort of patients with spinal tuberculosis METHODS: 212 patients of spinal tuberculosis treated between Jan 1999-June 2011 of which, 179 patients were included (> 6 months follow-up, mean age- 34.8 years; age range 10-75 years). The cohort was divided into 2 groups (n = 89 and 90 respectively), Group I :1999-2003 and Group II: 2004-2011. RESULTS: There were 93 males. Mean age was 34.8 + 7.2 years (range: 10-75 years). Mean duration of symptoms was 2.4 months. Sensory-motor deficits were present in 167 (93.5%; 74 patients were paraplegic), 156 (87%) had pain, 127 (71.7%) had bladder involvement and extra spinal tuberculosis was present in 36 patients (22.3%). 92% were on prior chemotherapy, 1/5(th) of the total on second-line chemotherapy. Thoracic spine was commonest (n= 86, 57%), followed by cervical (n=50, 29%), craniovertebral junction (22, 15%) and lumbo-sacral spine (n= 20, 10.5%). 146 patients underwent surgery, 68% instrumented fusions and 16% circumferential fusions. Mean follow-up: 20.2 months (range 6-60 months). 89% improved in motor/sensory deficits, 71% improved in pain, 88% improved in bladder symptoms, paraplegia improved in 77%. Group II had higher incidence of cord compression (p<0.01), severe vertebral body (p<0.001) collapse and paraplegia (p<0.001). Group II underwent more instrumented surgeries (p<0.01), especially circumferential fusions (p<0.001). The improvement of paraplegia was better after 2004 (Group II). Bladder symptoms correlated with the timing of surgery (p<0.1) CONCLUSION: Medical treatment is the main stay, however radical, instrumented surgeries should be offered when indicated. Presence of paraplegia should not preclude surgery. A practical management paradigm is also suggested.
World Neurosurgery 01/2013; · 0.68 Impact Factor
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ABSTRACT: Cell cycle regulator genes are major target of mutation in many human malignancies including glioblastomas (GBMs). CDKN2A is one such tumor suppressor gene which encodes p16INK4a protein and serves as an inhibitor of cell cycle progression. Very few studies are available regarding the association of CDKN2A deletion with p16 protein expression in GBMs. There is limited data on the frequency of CDKN2A deletion in different age groups. The aim of the present study was to analyze the frequency of CDKN2A gene deletions in GBM and correlate CDKN2A deletional status with (i) age of the patient (ii) p16 protein expression and (iii) other genetic alterations, namely EGFR amplification and TP53 mutation. A combined retrospective and prospective study was conducted. Sixty seven cases were included. The patients were grouped into pediatric (≤18 years), young adults (19-40 years) and older adults (>40 years). CDKN2A and EGFR status were assessed by Fluorescence in situ Hybridization.TP53 mutation was analyzed by PCR based method. p16 expression was assessed using immunohistochemistry. CDKN2A deletion was noted in 40.3% cases of GBM with majority being homozygous deletion (74%). It was commoner in primary GBMs (65.8%) and cases with EGFR amplification (50%). A variable frequency of CDKN2A was observed in older adults (42.3%), young adults (44%), and pediatric patients (31.25%). The difference however was not statistically significant. There was statistically significant association between CDKN2A deletion and p16 immunonegativity with a high negative predictive value of immunohistochemistry.
Neuropathology 01/2013; · 2.02 Impact Factor
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Sumit Bansal,
Pankaj Ailawadhi,
Ashish Suri,
Shashank Sharad Kale,
P Sarat Chandra,
Manmohan Singh,
Rajender Kumar,
Bhawani S Sharma,
Ashok Kumar Mahapatra,
Mehar C Sharma, Chitra Sarkar,
Pramod Bithal,
Hari Har Dash,
Sailesh Gaikwad,
Nalin Kumar Mishra
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ABSTRACT: We retrospectively reviewed the outcomes of 195 patients with intramedullary tumors who underwent surgery between January 2001 and December 2010 at a single institution. The symptomatology, neurological and neuroradiological findings, operative details, perioperative and postoperative complications, histopathological data and follow-up examinations of the 137 (70.2%) males and 58 (29.7%) females were studied and analyzed. Epidermoid was the most common intramedullary tumour in children (23%), whereas in adults, ependymomas were more common (46%). Ependymomas were more amenable to resection (total excision in 57.7% and near-total excision in 39.4%) as compared to astrocytomas (total excision in 29%; near total excision in 60.5%). At the final clinical follow-up, 24 patients (16.4%) had improved in McCormick grade, 112 patients (76.7%) remained unchanged and 11 patients (7.5%) had worsened. Complete removal of the lesion is the primary goal of surgery. We conclude that the strongest predictor of functional outcome was the preoperative neurological condition, beyond the histological differentiation of the intramedullary tumor.
Journal of Clinical Neuroscience 12/2012; · 1.25 Impact Factor
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Amol Raheja,
Vaishali Suri,
Ashish Suri, Chitra Sarkar,
Arti Srivastava,
Sujata Mohanty,
Krishan G Jain,
Meher C Sharma,
Hruda N Mallick,
Pradeep K Yadav,
Mani Kalaivani,
Ravindra M Pandey
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ABSTRACT: Object Bone marrow-derived stem cells enhance the rate of regeneration of neuronal cells leading to clinical improvement in nerve injury, spinal cord injury, and brain infarction. Recent experiments in the local application of bone marrow-derived mononuclear cells (BM-MNCs) in models of sciatic nerve transection in rats have suggested their beneficial role in nerve regeneration, although the effects of variable doses of stem cells on peripheral nerve regeneration have never been specifically evaluated in the literature. In this paper, the authors evaluated the dose-dependent role of BM-MNCs in peripheral nerve regeneration in a model of sciatic nerve transection in rats. Methods The right sciatic nerve of 60 adult female Wistar rats (randomized into 2 test groups and 1 control group, 20 rats in each group) underwent transection under an operating microscope. The cut ends of the nerve were approximated using 2 epineural microsutures. The gap was filled with low-dose (5 million BM-MNCs/100 μl phosphate-buffered saline [PBS]) rat BM-MNCs in one group, high-dose (10 million BM-MNCs/100 μl PBS) rat BM-MNCs in another group, and only PBS in the control group, and the approximated nerve ends were sealed using fibrin glue. Histological assessment was performed after 30 days by using semiquantitative and morphometric analyses and was done to assess axonal regeneration, percentage of myelinated fibers, axonal diameter, fiber diameter, and myelin thickness at distal-most sites (10 mm from site of repair), intermediate distal sites (5 mm distal to the repair site), and site of repair. Results The recovery of nerve cell architecture after nerve anastomosis was far better in the high-dose BM-MNC group than in the low-dose BM-MNC and control groups, and it was most evident (p < 0.02 in the majority of the parameters [3 of 4]) at the distal-most site. Overall, the improvement in myelin thickness was most significant with incremental dosage of BM-MNCs, and was evident at the repair, intermediate distal, and distal-most sites (p = 0.001). Conclusions This study emphasizes the role of BM-MNCs, which can be isolated easily from bone marrow aspirates, in peripheral nerve injury and highlights their dose-dependent facilitation of nerve regeneration.
Journal of Neurosurgery 10/2012; · 2.96 Impact Factor
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ABSTRACT: Alveolar soft part sarcoma (ASPS) is a relatively rare tumor that mostly presents as a slow growing mass in the deep soft tissue of the extremities. A substantial number of cases in children occur in the head and neck region; however, in any age group, it is very rarely reported in the sinonasal region. We report a case of ASPS of the paranasal sinuses with sellar extension in a 25-year-old man that masqueraded as a giant invasive pituitary adenoma. This is only the fifth case of sinonasal ASPS in literature. The clinical and radiological diagnoses were misleading, but an extensive pathology workup including electron microscopy helped reach an accurate diagnosis in this unusual case.
Annals of diagnostic pathology 08/2012;
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ABSTRACT: Papillary glioneuronal tumors (PGNT) have been recently included as a distinct entity in the WHO classification of tumors of the central nervous system. Their molecular pathogenesis is not clear. In the current study, we present the morphological, immunohistochemical, and molecular features of four cases of PGNT reported over the past 11 years.
Over a period of 11 years (January 2000-February 2010), there were four cases of PGNT, which were reviewed for histomorphological features. TP53 and IDH1 mutations were assessed using antibodies against p53 protein and for mutant IDH1(R132H) protein, respectively. Immunohistochemistry was also performed for epidermal growth factor receptor (EGFR) protein. Fluorescence in situ hybridization assay was used for analyzing 1p/19q deletion status.
All the tumors showed the characteristic biphasic morphology. Rare findings included minigemistocyte-like cells in one, angiomatous areas in three, focal necrosis in one, and a high MIB-1 labeling index of 12 and 13 %, respectively, in two of the cases. All lacked EGFR, IDH1 expression, and 1p/19q deletions. Interestingly, antibody for p53 labeled the tumor cells, mainly those showing glial differentiation, in two cases. At a mean follow-up of 30 months, there was no evidence of disease progression except in one case which recurred after 24 months.
PGNT are rare CNS neoplasms. Despite showing focal morphological features reminiscent of oligodendroglial tumors and presence of astrocytic component, they usually lack the common genetic alterations involved in the pathogenesis of gliomas. Multi-institutional pooling of cases may aid in elucidating their oncogenetic pathway.
Child s Nervous System 08/2012; 28(11):1897-904. · 1.54 Impact Factor
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ABSTRACT: Paediatric glioblastomas (GBM) are rare and currently there is insufficient information regarding their pathogenesis unlike their adult counterparts. Hence, this study was undertaken to gain insight into the genetic and epigenetic alterations in paediatric GBMs. The following mutations were studied by sequencing viz. TP53, IDH1 and H3F3A. Fluorescence in situ hybridization was done to assess EGFR amplification, PTEN deletion, and CDKN2A deletion. Also, MGMT methylation status was studied using methylation specific PCR. Further, 21 GBM cases along with 3 control normal brains from paediatric epilepsy surgery cases were studied using genome wide methylation profiling by Illumina Infinium HumanMethylation27 assay. Beta values were used to carry out Hierarchical Clustering Analysis (HCA) for all the genes and all samples. Differences between average beta values of GBM and controls were calculated. For validation of epigenetic alterations, mRNA expression analysis was performed using quantitative real time PCR. Comparison with methylation data from adult GBM cases from the same institute was also done. Paediatric GBMs were characterized by TP53 mutation in 47% of cases, CDKN2A deletion in 31%, PTEN deletion in 40% and MGMT methylation in 50%. No case showed IDH1 mutation or EGFR amplification. HCA showed at least two clusters of paediatric GBM. In total, there were 162 hypermethylated and 1318 hypomethylated genes. There were 9 genes hypermethylated and 11 hypomethylated in both paediatric and adult GBMs. Interestingly, two genes were hypomethylated in pediatric while hypermethylated in adult GBM viz. FSD1 and GPR62. However, there was no gene which was hypermethylated in pediatric and hypomethylated in adult. Expression analysis of these genes was done using real time PCR. This study highlights differences in the genetic and epigenetic alterations between pediatric and adult GBMs. Such a detailed understanding of the molecular pathogenesis is crucial for identification of relevant targets for designing of new therapeutic agents.
Neuro-Oncology 07/2012; Neuro Oncol (2012) 14 (suppl 1):i56-i68.doi: 10.1093/neuonc/nos102(14 (suppl 1)-doi: 10.1093/neuonc/nos102):i56-i68.. · 5.72 Impact Factor
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Clinical neuropathology 07/2012; 31(4/2012-Clinical neuropathology, vol. 31-no. 4/2012 (232-290)):232-290. · 1.04 Impact Factor
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Clinical neuropathology 07/2012; vol. 31(4.2012-vol. 31-No. 4/2012):232-290. · 1.04 Impact Factor
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Journal of Pediatric Hematology/Oncology 05/2012; · 1.16 Impact Factor
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ABSTRACT: Dysembryoplastic neuroepithelial tumor (DNT) is a relatively newly described entity and is an important cause of intractable
epilepsy. We report 32 cases of DNT who were operated and treated in our hospital over a period of 12years. Immunostaining
for various proliferative markers and tumor suppressor gene proteins was done to assess the proliferative potential of these
tumors. The most common presentation was partial complex seizures followed by generalized tonic–clonic seizures, focal motor
seizures, and myoclonus. The most common location was temporal lobe followed by frontal and in one patient lesion was multifocal.
All patients were seizure free at the last postoperative follow-up which varies from 12 to 96months with mean of 33.7months.
Microscopic examination showed classical histology comprising of intracortical multinodular microcystic lesions with floating
neurons. Proliferative indices were very low (<1%) and tumor suppressor gene protein expression was not seen in the present
study. Cortical dysplasia of the surrounding brain was observed in 37.3% of cases.
Neurosurgical Review 04/2012; 32(2):161-170. · 2.04 Impact Factor
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ABSTRACT: Our objective was to examine the reliability of histological diagnosis achieved vis a vis the number of biopsy bits obtained
along a single trajectory of the stereotactic needle. A retrospective analysis of stereotactic biopsies performed in a single
tertiary care neurosciences center, during a period of 11 years, between 1995 to 2005 was done. The overall diagnostic accuracy
achieved on histopathology was correlated with the number of bits obtained by stereotactic biopsy. A total of 86 cases were
analyzed, which consisted of 58 males and 28 females. Age ranged from 6 to 75 years, with a mean age of 36.1 years. Twenty
percent of the patients were in the pediatric age group and 15% were ≥60 years of age. Most common sites biopsied were thalamus/basal
ganglia (55.8%), followed by eloquent areas and other sites. A definitive histological diagnosis was established in 70 cases
(diagnostic yield, 81.3%), which encompassed 65 neoplastic and 5 nonneoplastic lesions. Astrocytic lesions, the most common,
include 10 pilocytic astrocytomas (PA), 29 diffuse astrocytomas (DA), 11 anaplastic astrocytomas (AA), and 7 glioblastoma
multiforme (GBM). In 16 cases no definite histological diagnosis could be offered. The number of biopsies ranged between 1
and 6 bits (mean, 2; median, 1). The majority (68.7%) of the biopsies were 1 or 2-bits. The diagnostic accuracy increased
from 76.5% for single biopsies to 84% and 88.2% for 2 and 3 bits, respectively, and 100% for biopsies with 5 to 6 bits. Overall,
a trend of higher diagnostic yield was seen in cases with more biopsies when compared with single bit biopsies. Thus, this
small series confirms that stereotactic procedures involving multiple bits are associated with a high diagnostic yield.
Brain Tumor Pathology 04/2012; 23(2):71-75. · 1.19 Impact Factor
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ABSTRACT: Classification and grading of astrocytic tumors has been the subject of several controversies and no universally accepted
classification system is yet available. Nevertheless, acceptance of a common system is important for assessing prognosis as
well as easy comparative evaluation and interpretation of the results of multi-center therapeutic trials. We report the results
of a single center study on comparative survival evaluation along with assessment of interclassification concordance in 102
cases of supratentorial astrocytic tumors in adults (≥ 16 years of age). Hematoxylin and eosin (H&E) stained slides of these
102 cases were reviewed independently by two pathologists and each case classified or graded according to four different classification
systems viz. Kernohan, Daumas-Duport (SAM-A), TESTAST-268 and WHO. The histological grading was then correlated with the survival
curves as estimated by the Kaplan-Meier method. The most important observation was that similar survival curves were obtained
for any one grade of tumor by all the four classification systems. Fifty three of the 102 cases (51.9%) showed absolute grading
concordance using all 4 classifications with maximum concordant cases belonging to grades 2 and 4. Intra-classification grade-wise
survival analysis revealed a statistically significant difference between grade 2 and grades 3 or 4, but no difference between
grades 3 and 4 in any of the classification systems. It is apparent from the results of this study that if specified criteria
related to any of the classification systems is rigorously adhered to, it will produce comparable results. Hence, preferential
adoption of any one classification system in practice will be guided by the relative ease of histologic feature value evaluation
with maximum possible objectivity and reproducibility. We recommend the Daumas-Duport (SAM-A) system since it appears to be
the simplest, most objectivized for practical application and highly reproducible with relative ease.
Pathology & Oncology Research 04/2012; 6(1):46-52. · 1.37 Impact Factor
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ABSTRACT: This is a study of 64 cases of recurrent astrocytic tumors of all four WHO grades wherein a comparative evaluation of initial
vs. recurrent tumor was done with respect to histological grading, MIB-1 labeling index (LI) and apoptotic index (AI). The
aim was to identify factor/s that could influence interval to recurrence and/or malignant progression. Recurrence was noted
in all grades and upon recurrence, 93.3% of grade II (low grade diffuse) astrocytomas and 63.6% of grade III anaplastic astrocytomas
underwent malignant progression. However, none of the Grade I tumors showed evidence of malignant progression. Though interval
to recurrence varied considerably, there was a correlation with histological grade of the initial tumor in that grade I and
II tumors had a significantly longer mean interval to recurrence (43 months and 54.8 months respectively) as compared to grade
III and IV (glioblastoma multiforme) tumors (17.6 and 12.8 months respectively). The interval to recurrence was also longer
for grade II and III tumors which showed progression on recurrence (55.3 months for Grade II → Grade III; 54 months for Grade
II → Grade IV and 20.6 months for Grade III → IV) as compared to tumors which recurred to the same grade (12.5 months for
Grade III → Grade III and 12.8 months for Grade IV→ Grade IV). A statistically significant inverse correlation of MIB-1 LI
with interval to recurrence was noted. Higher the MIB-1 LI, shorter was the interval to recurrence. Further a cut off MIB-1
LI value of 2.8% could be proposed in predicting recurrence free survival. Interestingly, MIB-1 LI of grade II tumors, which
had progressed to grade IV was significantly higher than MIB-1 LI of grade II tumors which had progressed to grade III. Thus,
this study establishes the potential role of MIB-1 LI of the initial tumor in determining interval to recurrence. However,
apoptotic index has no role in predicting either interval to recurrence or malignant progression.
Pathology & Oncology Research 04/2012; 7(4):267-278. · 1.37 Impact Factor
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Geetika Singh,
Supriyo Mallick,
Vikas Sharma,
Nikhil Joshi,
Suvendu Purkait,
Prerana Jha,
Mehar Chand Sharma,
Vaishali Suri,
Pramod Kumar Julka,
Ashok Kumar Mahapatra,
Manmohan Singh,
Shashank Sharad Kale, Chitra Sarkar
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ABSTRACT: Gliosarcoma is a rare variant of glioblastoma multiforme (GBM) with similar clinical presentation and prognosis but a distinct genetic profile. The clinicopathological features of 22 cases of gliosarcoma were analyzed with respect to age, sex, KPS score, operative diagnosis, extent of resection and histopathological subtype (predominantly sarcomatous [PS], predominantly gliomatous [PG] or mixed). Twelve cases were PS, six were PG and four were mixed. The histological subtype did not correlate with the operative diagnosis; however, it did significantly correlate with the extent of resection (P = 0.014). In 14 cases with available survival data it was found that none of the clinicopathological parameters significantly correlated with survival (P > 0.05). Methyl guanine DNA methyl transferase promoter methylation studies were performed using methylation-specific PCR in 16 cases which showed a methylation rate of 31.25% (5/16). The promoter methylation status did not correlate with the histological subtype and did not significantly affect survival (P > 0.05). Although gliosarcomas continue to be treated in the same way as GBM, the role of chemotherapy with temozolomide is not clear. This cohort is the largest to date to uniformly receive the Stupp's protocol which is currently "standard of care" for GBM. A median overall survival of 18.5 months is substantially higher than previous studies, suggesting that temozolomide should be included in gliosarcoma therapy.
Neuropathology 03/2012; 32(5):534-42. · 2.02 Impact Factor
