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      Giant parieto-occipital lobe pediatric gliosarcoma: Report of a rare entity and review of literature

      case-report

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          Abstract

          Background:

          Gliosarcoma is a rare high-grade malignant tumor and a variant of glioblastoma characterized by biphasic glial and mesenchymal components. Gliosarcomas occur most commonly in the fifth or sixth decade of life and have a temporal lobe predilection. Occurrence in the pediatric population is extremely rare.

          Case Description:

          Here, we report the case of an 8-year-old child with histologically confirmed gliosarcoma at the parieto-occipital lobe. Only a subtotal resection of the tumor mass could be performed in view of massive bleeding from the tumor bed; and despite postoperative chemotherapy and radiotherapy, the tumor recurred in a short span of time. A repeat surgery was done but the patient could not survive.

          Conclusion:

          To our knowledge, this case constitutes the second youngest case reported in the literature with the lesion in the parieto-occipital region and the third youngest in all pediatric cases of gliosarcoma. This case demonstrates that possibility of gliosarcoma should always be kept in mind in children presenting with features of intracranial high-grade glial tumor. This case also suggests that significant residual after surgery is one variable that may affect the prognosis despite radiotherapy and/or chemotherapy.

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          Most cited references20

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          Genetic profile of gliosarcomas.

          There are distinct genetic pathways leading to the glioblastoma, the most malignant astrocytic brain tumor. Primary (de novo) glioblastomas develop in older patients and are characterized by epidermal growth factor (EGF) receptor amplification/overexpression, p16 deletion, and PTEN mutations, whereas secondary glioblastomas that progressed from low-grade or anaplastic astrocytoma develop in younger patients and frequently contain p53 mutations. In this study, we assessed the genetic profile of gliosarcoma, a rare glioblastoma variant characterized by a biphasic tissue pattern with alternating areas displaying glial and mesenchymal differentiation. Single-strand conformation polymorphism followed by direct DNA sequencing revealed p53 mutations in five of 19 gliosarcomas (26%) and PTEN mutations in seven cases (37%). Homozygous p16 deletion was detected by differential polymerase chain reaction in seven (37%) gliosarcomas. The overall incidence of alterations in the Rb pathway (p16 deletion, CDK4 amplification, or loss of pRb immunoreactivity) was 53%, and these changes were mutually exclusive. Coamplification of CDK4 and MDM2 was detected in one gliosarcoma. None of the gliosarcomas showed amplification or overexpression of the EGF receptor. Thus gliosarcomas exhibit a genetic profile similar to that of primary (de novo) glioblastomas, except for the absence of EGFR amplification/overexpression. Identical PTEN mutations in the gliomatous and sarcomatous tumor components were found in two cases. Other biopsies contained p16 deletions, an identical p53 mutation, or coamplification of MDM2 and CDK4 in both tumor areas. This strongly supports the concept of a monoclonal origin of gliosarcomas and an evolution of the sarcomatous component due to aberrant mesenchymal differentiation in a highly malignant astrocytic neoplasm.
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            Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity

            This report presents the historical experience, clinical presentation, treatment, prognosis, and pathogenesis of gliosarcoma described to date in the English literature. PubMed query of term “gliosarcoma” was performed, followed by a rigorous review of cited literature. Articles selected for analysis included: (1) case reports of gliosarcoma, (2) review articles of gliosarcoma, and (3) studies of the pathogenesis or genetics of gliosarcoma in humans. Our review identified 219 cases of gliosarcoma in 34 reports and eight articles addressing the pathogenesis. Survival in larger series ranged 4–11.5 months. Features unique to gliosarcoma compared to glioblastoma (GBM) include their temporal lobe predilection, potential to appear similar to a meningioma at surgery, repeated reports of extracranial metastases, and infrequency of EGFR mutations. Published experience is limited to small case series, and the pathogenesis remains unclear. Clinical and pathologic characteristics distinct from GBM suggest that they may warrant specific treatment, separate from conventional GBM therapy.
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              Gliosarcoma: a clinical study.

              Gliosarcomas are rare biphasic neoplasms of the central nervous system composed of a glioblastoma multiforme (GBM) admixed with a sarcomatous component. There are conflicting reports regarding their clinical aggressiveness. Four hundred and twenty-two consecutive patients with GBM were treated at our hospital between 1980 and 1999, among them 12 gliosarcomas. The goal of this study was to examine clinical features, treatment, survival and patterns of failure of gliosarcoma patients and to compare them with the entire group of GBM patients. This comparison was refined by a matched pair analysis with a group of 12 GBM patients selected for age, Karnofsky performance status, resection status, fractionation scheme and total dose (control GBM group). Seven gliosarcoma patients were male, five female, with a median age of 56 years (range 37-76 years). The median tumor size was 4.5 cm (range 3-8 cm). The locations, all supratentorial, included temporal in six, parietal in five, frontal in four and occipital in one patient. All patients underwent tumor resection followed by postoperative radiation therapy. Median survival was 11.5 months for the gliosarcoma group, 8.1 months for the entire GBM group (log rank test, P=0.16) and 11.0 months for the control GBM group (log rank test, P=0.36). All gliosarcoma patients had local tumor recurrences and died due to neurologic causes within 19.3 months after radiation therapy. With regard to clinical features, survival and patterns of failure, gliosarcomas and GBM cannot be distinguished clinically. Therefore, the same principles should be applied for the treatment of these tumors.
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                Author and article information

                Contributors
                Journal
                Surg Neurol Int
                Surg Neurol Int
                SNI
                Surgical Neurology International
                Medknow Publications & Media Pvt Ltd (India )
                2229-5097
                2152-7806
                2018
                29 May 2018
                : 9
                : 111
                Affiliations
                [1]Department of Neuro-Surgery, Govind Ballav Pant Institute of Postgraduate Medical Education and Research (GIPMER), New Delhi, India
                Author notes
                [* ]Corresponding author
                Article
                SNI-9-111
                10.4103/sni.sni_31_18
                5991275
                29930877
                2c09ea1f-6714-43ab-8428-9f3ba8c44def
                Copyright: © 2018 Surgical Neurology International

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 27 January 2018
                : 23 April 2018
                Categories
                Pediatric Neurosurgery: Case Report

                Surgery
                brain neoplasm,glioblastoma multiforme,gliosarcoma,pediatric brain tumor
                Surgery
                brain neoplasm, glioblastoma multiforme, gliosarcoma, pediatric brain tumor

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