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Angiosarcomatous component in gliosarcoma: case report and consideration of diagnostic challenge and hemorrhagic propensity

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The Journal of Biomedical Research 2020年第2期34卷 143-148页

作者： Nancy Jiang Ramiro Larrazabal Waleed Alsunbul Jian-Qiang Lu Department of Radiology McMaster University/Hamilton Health SciencesHamiltonOntario L8L 2X2Canada Department of Surgery/Neurosurgery McMaster University/Hamilton Health SciencesHamiltonOntario L8L 2X2Canada Department of Pathology and Molecular Medicine/Neuropathology McMaster University/Hamilton Health SciencesHamiltonOntario L8L 2X2Canada

An angiosarcomatous component in gliosarcoma may be associated with an increased intraoperative hemorrhagic risk and preoperative diagnostic challenge. We report a unique case of gliosarcoma with an angiosarcomatous component in a 61-year-old man. His brain MRI demonstrated a well-demarcated right occipital tumor with multiple flow voids and rim-like enhancement as well as intratumoral strip and nodular enhancements. He underwent a craniotomy for tumor resection. Intraoperatively, significant tumor hemorrhage required greater efforts to control intraoperative bleeding and to maintain hemostasis. Pathological examination of the tumor revealed alternating gliomatous and sarcomatous/angiosarcomatous components with intratumoral hemorrhage. He was postoperatively treated with chemoradiation. The tumor recurred at 9 months, for which the second resection was performed with similarly greater efforts to achieve hemostasis. The recurrent tumor was pathologically similar despite treatment-associated changes. Awareness of this angiosarcomatous component in gliosarcoma with the hemorrhagic risk is important for both the preoperative diagnosis and surgical management.

关键词： gliosarcoma glioblastoma variant angiosarcomatous component vascularity hemorrhage risk

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gliosarcoma:A rare variant of glioblastoma multiforme in paediatric patient:Case report and review of literature

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World Journal of Clinical Cases 2016年第9期4卷 302-305页

作者： Ugan Singh Meena Sumit Sharma Sanjeev Chopra Shashi Kant Jain Department of Neurosurgery Swai Man Singh Medical College

gliosarcoma is rare central nervous system tumour and a variant of glioblastoma multiforme with bimorphic histological pattern of glial and sarcomatous differentiation. It occurs in elderly between 5^(th) and 6^(th) decades of life and extremely rare in children. It is highly aggressive tumour and managed like glioblastoma multiforme. A 12-year-old female child presented with complaints of headache and vomiting from 15d and blurring of vision from 3d. Magnetic resonance imaging of brain shows heterogeneous mass in right parieto-occipital cortex. A right parieto-occipito-temporal craniotomy with complete excision of mass revealed a primary glioblastoma on histopathological investigation. Treatment consists of maximum surgical excision followed by adjuvant radiotherapy. The etiopathogenesis, treatment modalities and prognosis is discussed. The available literature is also reviewed.

关键词： Paediatric gliosarcoma Surgery Glioblastoma multiforme Radiotherapy

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Clinical outcome of gliosarcoma compared with glioblastoma multiforme: a clinical study in Chinese patients

Clinical outcome of gliosarcoma compared with glioblastoma m...

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中国神经科学学会第十二届全国学术会议

作者： Guobin Zhang Yonggang Wang Department of Neurosurgery China National Clinical Research Center for Neurological Diseases （NCRC-ND） Center of Brain Tumor Beijing Institute for Brain Disorders Beijing Key Laboratory of Brian Tumor Beijing Tiantan Hospital Capital Medical University

gliosarcoma（GSM） is a rare biphasic neo-plasms of the central nervous system composed of a glioblastoma multiforme（GBM） admixed with a sarco-matous component. In clinical practice GSM is generally managed similarly to GBM. However, there are conflicting reports regarding their clinical aggressiveness, cell line of origin and possible prognosis compared with those of GBM. The objective of this study was to compare clinic-pathological features in GSM patients with the GBM patients during the same study period. 518 patients with GBM were treated at our hospital between 2008 and 2013, among them 51 were GSM. In this series the GSMs rep-resented 9.8 % of all GBMs and included 58.8 % male with a median age of 44.7 years. The locations, all supra-tentorial, included temporal in 41.2 %, frontal in 25.5 %, parietal in 19.6 %, and occipital in 13.7 %. All patients underwent tumor resection followed by post-operative radiation and adjuvant chemotherapy. The O6-methylgua-nine-DNA methyltransferase promoter methylation studies were significantly more frequent in the GBMs than GSMs（80.1 % vs. 44.7 %, P \ 0.001）. The median progression free survival and overall survival for the patients with GSM were 8.0 and 13.0 months, respectively, as compared with 9.0 and 14.0 months in the GBM group（log rank test P = 0.001 and 0.004, respectively）. The Cox proportional hazards regression model indicated that the extent of tumor resection（HR = 1.518, P = 0.009） and pathological types（HR = 0.608, P = 0.002） were the significant prognostic factors in our own series. With regard to clinical features and outcomes, GSM and GBM cannot be distinguished clinically. GSM in China may be managed similarly to GBM, with maximal safe surgical resection followed by chemoradiotherapy. Our study adds further evidence to support GSM as a unique clinical entity with a likely worse prognosis than GBM.

关键词： gliosarcoma Glioblastoma multiforme Prognosis

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A Phase II Study of Antineoplastons A10 and AS2-1 in Children with Recurrent, Refractory or Progressive Primary Brain Tumors—Final Report (Protocol BT-22)

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Journal of Cancer Therapy 2014年第10期5卷 977-988页

作者： Stanislaw R. Burzynski Tomasz J. Janicki Gregory S. Burzynski Ania Marszalek Sheldon Brookman Burzynski Clinic Houston Texas USA

Primary malignant brain tumors are a leading cause of cancer-related death in children. This Phase II study evaluated the efficacy and safety of Antineoplastons A10 and AS2-1 (ANP) in children who developed progression during standard treatment. A total of 43 children were recruited to the study, but only 41 met eligibility criteria. There were twelve cases of glioblastoma multiforme (GBM), eight anaplastic astrocytomas (AA), twelve diffuse intrinsic pontine gliomas (DIPG), three supertentorial primitive neuroectodermal tumors (sPNET), three cases of medulloblastoma and one case each of anaplastic ependymoma (AE), atypical teratoid rhabdoid tumor (AT/RT), and disseminated pilocytic astrocytoma (PAD). ANP was administered intravenously daily every four hours (median dose of A10 8.74 g/kg/d and AS2-1 0.35 g/kg/d), until objective response (OR) was documented, and then a further eight months. All enrolled patients were included in safety, but only eligible patients in the efficacy evaluation. A total of 12.2% of patients obtained OR;2.4% complete response (CR) and 9.8% partial response (PR). Stable disease (SD) was determined in 17.1% and progressive disease (PD) in 43.9% of cases. There were 26.8% of nonevaluable (NE) cases due to premature discontinuation. Out of five OR cases, four patients were diagnosed with recurrent DIPG and one with recurrent AA. Median progression-free survival (PFS) was 2.5 months. Median overall survival was 4.8 months. OS at 6 months was 46.3%, one year was 12.2%, and 4.8% at two, five, and ten years. The longest survivor is a patient diagnosed with DIPG and gliosarcoma who remains alive more than 15 years. A group of eleven patients reported grade 3 and 4 toxicity including hypernatremia in eight cases, somnolence in two cases, and hypokalemia in one case. There were no chronic toxicities, and the quality of life was very good. The largest group of patients were represented by DIPG, GBM, and AA. The best results were obtained in the DIPG a

关键词： Anaplastic Astrocytoma Antineoplastons A10 and AS2-1 Brainstem Glioma Diffuse Intrinsic Pontine Glioma (DIPG) gliosarcoma Phase II Clinical Trial Recurrent Glioma

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