RESUMEN
BACKGROUND: Gliosarcoma (GSM) is a highly aggressive variant of brain cancer with an extremely unfavorable prognosis. Prognosis is not feasible by traditional methods because of a lack of staging criteria, and the present study aims to screen more detailed demographic factors to predict the prognostic factors of the tumors. METHODS: For this study, we extracted data of patients diagnosed with GSM from the SEER (Surveillance Epidemiology and End Results) database between 2000 and 2019. To account for the influence of competing risks, we used a Cumulative Incidence Function. Subsequently, univariate analysis was conducted to evaluate the individual variables under investigation. Specifically for patients with GSM, we generated cumulative risk curves for specific mortality outcomes and events related to competing risks. In addition, we used both univariate and multivariate Cox analysis to account for non-GSM-related deaths that may confound our research. RESULTS: The competing risk model showed that age, marital status, tumor size, and adjuvant therapy were prognostic factors in GSM-related death. The analysis results showed that older age (60-70 years, ≥71 years) and larger tumor size (≥5.3 cm) significantly increased the risk of GSM-related death. Conversely, surgical intervention, chemotherapy, and being single were identified as protective factors against GSM-related death. CONCLUSIONS: Our study using a competing risk model provided valuable insights into the prognostic factors associated with GSM-related death. Further research and clinical interventions targeted at minimizing these risk factors and promoting the use of protective measures may contribute to improved outcomes and reduced mortality for patients with GSM.
Asunto(s)
Neoplasias Encefálicas , Gliosarcoma , Humanos , Pronóstico , Gliosarcoma/diagnóstico , Factores de Riesgo , Neoplasias Encefálicas/patología , Incidencia , Programa de VERFRESUMEN
Gliosarcoma is a rare histopathological subtype of glioblastoma. Metastatic spreading is unusual. In this report, we illustrate a case of gliosarcoma with extensive extracranial metastases with confirmation of histological and molecular concordance between the primary tumor and a metastatic lesion of the lung. Only the autopsy revealed the extent of metastatic spread and the hematogenous pattern of metastatic dissemination. Moreover, the case bared a familial coincidence of malignant glial tumors as the patient's son was diagnosed with a high-grade glioma shortly after the patient's death. By molecular analysis (Sanger and next generation panel sequencing), we could confirm that both patient's tumors carried mutations in the TP53 gene. Interestingly, the detected mutations were located in different exons. Altogether, this case draws attention to the fact that sudden clinical aggravation could be caused by the rare phenomenon of metastatic spread and should therefore be always taken into consideration, even at an early disease stage. Furthermore, the presented case highlights the contemporary value of autoptic pathological examination.
Asunto(s)
Neoplasias Encefálicas , Glioblastoma , Gliosarcoma , Neoplasias Pulmonares , Humanos , Gliosarcoma/genética , Gliosarcoma/diagnóstico , Gliosarcoma/patología , Neoplasias Encefálicas/patología , Glioblastoma/patología , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/secundario , Pulmón/patologíaRESUMEN
Purpose: Gliosarcoma is a histopathological variant of glioblastoma, which is characterized by a biphasic growth pattern consisting of glial and sarcoma components. Owing to its scarcity, data regarding the impact of available treatments on the clinical outcomes of gliosarcoma are inadequate. The purpose of this retrospective cohort study was to analyze the prognostic factors of gliosarcoma. Methods: By screening the clinical database of neurosurgical cases at a single center, patients with gliosarcoma diagnosed histologically from 2013 to 2021 were identified. Clinical, pathological, and molecular data were gathered founded on medical records and follow-up interviews. Prognostic factors were derived using the Cox proportional hazards model with backward stepwise regression analysis. Results: Forty-five GSM patients were included. Median overall survival was 25.6 months (95% CI 8.0-43.1), and median relapse-free survival was 15.2 months (95% CI 9.7-20.8). In multivariable analysis, total resection (p = 0.023, HR = 0.192, 95% CI 0.046-0.797) indicated an improved prognosis. And low expression of Ki-67 (p = 0.059, HR = 2.803, 95% CI 0.963-8.162) would be likely to show statistical significance. However, there might be no statistically significant survival benefit from radiotherapy with concurrent temozolomide (n = 33, 73.3%, log-rank p = 0.99) or adjuvant temozolomide (n = 32, 71.1%, log-rank p = 0.74). Conclusion: This single-center retrospective study with a limited cohort size has demonstrated the treatment of gross total resection and low expression of Ki-67 which are beneficial for patients with GSM, while radiotherapy or temozolomide is not.
Asunto(s)
Neoplasias Encefálicas , Glioblastoma , Gliosarcoma , Humanos , Temozolomida , Gliosarcoma/diagnóstico , Gliosarcoma/terapia , Gliosarcoma/patología , Estudios Retrospectivos , Pronóstico , Antígeno Ki-67 , Neoplasias Encefálicas/patología , Recurrencia Local de Neoplasia , Glioblastoma/patologíaRESUMEN
Gliosarcoma is a biphasic central nervous system malignancy composed of glial and mesenchymal components. It is recognized as a rare variant of glioblastoma with unique histology, immunostaining properties, and natural history. Although usually described to primarily involve the brain, a search of the published literature revealed four reported cases of gliosarcoma arising in the spinal cord. This report describes the case of a 35-year-old woman with progressive back pain with loss of sensation and power of both lower limbs. Magnetic resonance imaging showed an intramedullary unifocal space-occupying lesion in her thoracolumbar spinal cord. Subtotal resection and subsequent histopathological and immunohistochemical studies confirmed the diagnosis of gliosarcoma of the spinal cord. This report further establishes the clinical entity of primary spinal gliosarcoma and proposes the need to consider this possibility among the differential diagnoses of a space-occupying spinal lesion.
Asunto(s)
Neoplasias Encefálicas , Glioblastoma , Gliosarcoma , Neoplasias de la Médula Espinal , Adulto , Neoplasias Encefálicas/patología , Femenino , Gliosarcoma/diagnóstico , Gliosarcoma/patología , Gliosarcoma/cirugía , Humanos , Imagen por Resonancia Magnética , Neoplasias de la Médula Espinal/diagnóstico por imagen , Neoplasias de la Médula Espinal/cirugíaRESUMEN
Gliosarcoma is an aggressive brain tumor with histologic features of glioblastoma (GBM) and soft tissue sarcoma. Despite its poor prognosis, its rarity has precluded analysis of its underlying biology. We used a multi-center database to characterize the genomic landscape of gliosarcoma. Sequencing data was obtained from 35 gliosarcoma patients from Genomics Evidence Neoplasia Information Exchange (GENIE) 5.0, a database curated by the American Association of Cancer Research (AACR). We analyzed genomic alterations in gliosarcomas and compared them to GBM (n = 1,449) and soft tissue sarcoma (n = 1,042). 30 samples were included (37% female, median age 59 [IQR: 49-64]). Nineteen common genes were identified in gliosarcoma, defined as those altered in > 5% of samples, including TERT Promoter (92%), PTEN (66%), and TP53 (60%). Of the 19 common genes in gliosarcoma, 6 were also common in both GBM and soft tissue sarcoma, 4 in GBM alone, 0 in soft tissue sarcoma alone, and 9 were more distinct to gliosarcoma. Of these, BRAF harbored an OncoKB level 1 designation, indicating its status as a predictive biomarker of response to an FDA-approved drug in certain cancers. EGFR, CDKN2A, NF1, and PTEN harbored level 4 designations in solid tumors, indicating biological evidence of these biomarkers predicting a drug-response. Gliosarcoma contains molecular features that overlap GBM and soft tissue sarcoma, as well as its own distinct genomic signatures. This may play a role in disease classification and inclusion criteria for clinical trials. Gliosarcoma mutations with potential therapeutic indications include BRAF, EGFR, CDKN2A, NF1, and PTEN.
Asunto(s)
Antineoplásicos/uso terapéutico , Biomarcadores de Tumor/genética , Neoplasias Encefálicas/genética , Regulación Neoplásica de la Expresión Génica , Glioblastoma/genética , Gliosarcoma/genética , Protocolos de Quimioterapia Combinada Antineoplásica , Biomarcadores de Tumor/metabolismo , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/patología , Inhibidor p16 de la Quinasa Dependiente de Ciclina/genética , Inhibidor p16 de la Quinasa Dependiente de Ciclina/metabolismo , Bases de Datos Factuales , Receptores ErbB/genética , Receptores ErbB/metabolismo , Femenino , Perfilación de la Expresión Génica , Glioblastoma/diagnóstico , Glioblastoma/tratamiento farmacológico , Glioblastoma/patología , Gliosarcoma/diagnóstico , Gliosarcoma/tratamiento farmacológico , Gliosarcoma/patología , Humanos , Masculino , Persona de Mediana Edad , Mutación , Neurofibromina 1/genética , Neurofibromina 1/metabolismo , Fosfohidrolasa PTEN/genética , Fosfohidrolasa PTEN/metabolismo , Pronóstico , Proteínas Proto-Oncogénicas B-raf/genética , Proteínas Proto-Oncogénicas B-raf/metabolismo , Telomerasa/genética , Telomerasa/metabolismo , Proteína p53 Supresora de Tumor/genética , Proteína p53 Supresora de Tumor/metabolismoRESUMEN
OBJECTIVE: Gliosarcomas (GS) comprise ~ 2 - 8% of glioblastomas and are associated with a similar poor prognosis. GS have rarely been found with a primitive neuroectodermal component (PNET). We present a case of gliosarcoma with PNET features (GS-PNET) that mimicked a neuroendocrine carcinoma on initial biopsy. MATERIALS AND METHODS: A 68-year-old male presented with 2 weeks of increasing headaches and difficulties with reading, writing, and word-finding. He was found to have a left-sided parieto-occipital heterogeneously enhancing mass. RESULTS: Pathologic analysis after surgical resection initially diagnosed a poorly differentiated carcinoma with neuroendocrine features, and adjuvant therapy was guided by this diagnosis as well as systemic imaging, which was suggestive of gastrointestinal primary malignancy with central nervous system (CNS) metastasis. Subsequent progression and re-resection established a diagnosis of GS with PNET component. Genomic profiling showed shared PTEN, TERT promotor, and TP53 mutations in the original and recurrent tumors. CONCLUSION: There have only been 5 previously reported cases of GS-PNET, to our knowledge, with this case representing the first with comprehensive molecular profiling. The case also highlights the importance of further work-up of presumed metastatic carcinoma with indeterminate immunostaining and/or suspected non-epithelioid component.
Asunto(s)
Neoplasias Encefálicas/patología , Gliosarcoma/patología , Anciano , Biomarcadores/análisis , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Carcinoma Neuroendocrino/diagnóstico , Diagnóstico Diferencial , Gliosarcoma/diagnóstico , Gliosarcoma/genética , Humanos , Masculino , Mutación , Fosfohidrolasa PTEN/genética , Telomerasa/genética , Proteína p53 Supresora de Tumor/genéticaRESUMEN
BACKGROUND: Gliosarcoma (GSM) is a distinct and aggressive variant of glioblastoma multiforme (GBM) with worse prognosis and few treatment options. It is often managed with the same treatment modalities with temozolomide (TMZ) as in GBM. However, the therapeutic benefits on GSM from such treatment regimen is largely unknown. Patient-derived xenograft (PDX) models have been used widely to model tumor progression, and subsequently to validate biomarkers and inform potential therapeutic regimens. Here, we report for the first time the successful development of a PDX model of secondary GSM. METHODS: Tissue obtained from a tumor resection revealed a secondary GSM arising from GBM. The clinical, radiological, and histopathological records of the patient were retrospectively reviewed. Samples obtained from surgery were cultured ex vivo and/or implanted subcutaneously in immunocompromised mice. Histopathological features between the primary GBM, secondary GSM, and GSM PDX are compared. RESULTS: In explant culture, the cells displayed a spindle-shaped morphology under phase contrast microscopy, consistent with the sarcomatous component. GSM samples were subcutaneously engrafted into immunocompromised mice after single-cell suspension. Xenografts of serial passages showed enhanced growth rate with increased in vivo passage. We did not observe any histopathological differences between the secondary GSM and its serial in vivo passages of PDX tumors. CONCLUSIONS: Our PDX model for GSM retained the histopathological characteristics of the engrafted tumor from the patient. It may provide valuable information to facilitate molecular and histopathological modelling of GSM and be of significant implication in future research to establish precise cancer medicine for this highly malignant tumor.
Asunto(s)
Neoplasias Encefálicas/terapia , Quimioradioterapia/efectos adversos , Glioblastoma/terapia , Gliosarcoma/diagnóstico , Neoplasias Primarias Secundarias/diagnóstico , Animales , Biomarcadores de Tumor/análisis , Biomarcadores de Tumor/genética , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Encéfalo/cirugía , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patología , Proteínas de Ciclo Celular/análisis , Proteínas de Ciclo Celular/genética , Quimioradioterapia/métodos , Craneotomía , Femenino , Glioblastoma/diagnóstico , Glioblastoma/genética , Glioblastoma/patología , Gliosarcoma/etiología , Gliosarcoma/genética , Gliosarcoma/patología , Humanos , Imagen por Resonancia Magnética , Ratones , Persona de Mediana Edad , Mutación , Neoplasias Primarias Secundarias/etiología , Neoplasias Primarias Secundarias/genética , Neoplasias Primarias Secundarias/patología , Cultivo Primario de Células , Temozolomida/uso terapéutico , Células Tumorales Cultivadas , Ensayos Antitumor por Modelo de XenoinjertoRESUMEN
Gliosarcoma (GSM) is a rare central nervous system tumor. Clinical management of it is similar to glioblastoma (GBM). However, due to a few comparative studies exist, uncertainty and disagreements remain in the literatures. To assess the available evidence on the value of different treatments and to carry out an up-to-date evaluation to summarize the evidence for the optimal treatment in GSM patients. Free words were used to search for the relevant studies without language limitations in electronic databases including PubMed, Ovid EMBASE, Cochrane Central Register of Controlled Trials from inception to September 15, 2019. Pooled hazard ratio (HR) with 95% confidence interval (CI) were calculated using a random-effects model. The main endpoint was all-cause mortality. Overall, 10 studies published between 2008 and 2018 including 803 patients were selected for the meta-analysis. Temozolomide (TMZ)-dominated chemotherapy was associated with a reduced risk of overall survival (OS), with HR 0.49 (95% CI 0.37-0.66). The pooled HR of OS was 0.40 (95% CI 0.29-0.56) between radiotherapy and without radiotherapy. The pooled HR (0.52, 95% CI 0.32-0.85) indicated gross total resection (GTR) had a positive impact on OS in GSM. In patients with GSM, survival benefits as currently performed are associated with TMZ-dominated chemotherapy and high-dose radiotherapy. Our systematic review and meta-analysis also demonstrate GTR is associated with a reduction in all-cause mortality in patients with primary GSM.
Asunto(s)
Antineoplásicos Alquilantes/administración & dosificación , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Gliosarcoma/diagnóstico , Gliosarcoma/terapia , Temozolomida/administración & dosificación , Ensayos Clínicos como Asunto/métodos , Terapia Combinada/métodos , Humanos , Resultado del TratamientoRESUMEN
OBJECTIVE: Gliosarcoma (GSC) is a rare histological variant of glioblastoma (GBM). Due to limited evidence regarding clinical, genetic and radiographic characteristics of GSC, this study aimed to analyze independent outcome predictors of GSC, and to address the differences between GSC and GBM concerning the baseline characteristics and patients' survival. METHODS: Patients treated between 2001 and 2018 for the diagnosis of GBM and GSC were included in this study. Patients' records were reviewed for demographic, clinical, genetic and radiographic characteristics. Univariate, multivariate and propensity score matched analyses were performed. RESULTS: In the GSC sub-cohort (N = 56), patients' age, preoperative clinical status, midline tumor location and tumor size were found to be independently associated with overall survival. As compared to GBM individuals (N = 1249), a temporal location (p = 0.002), presence of eccentric tumor cysts (p < 0.001), a higher ratio of TP53 staining (p = 0.002) and a lower ratio of GFAP staining (p = 0.005) were characteristic for GSC. The diagnosis of GSC was associated with a poorer survival (p = 0.002) independently of the patients' age, sex, clinical status and extent of resection, However, this association was no more significant, when enhancing the multivariate analysis with molecular-genetic characteristics (IDH1 mutation and MGMT promotor methylation status). DISCUSSION: Certain radiographic and molecular-genetic patterns present the distinct characteristics of GSC. There is an association between the diagnosis of GSC and a poorer outcome. This difference might be linked to different genetic alterations in GBM and GSC. Prospective studies are needed to further elucidate the characteristics of GSC and develop targeted treatment approaches for this rare variant.
Asunto(s)
Astrocitoma/patología , Neoplasias Encefálicas/patología , Glioblastoma/patología , Gliosarcoma/patología , Adulto , Anciano , Anciano de 80 o más Años , Astrocitoma/diagnóstico , Astrocitoma/cirugía , Neoplasias Encefálicas/cirugía , Metilasas de Modificación del ADN/genética , Enzimas Reparadoras del ADN/genética , Femenino , Glioblastoma/diagnóstico , Glioblastoma/cirugía , Gliosarcoma/diagnóstico , Gliosarcoma/cirugía , Humanos , Masculino , Persona de Mediana Edad , Mutación/genética , Procedimientos Neuroquirúrgicos/métodos , PronósticoRESUMEN
Gliosarcoma is an unusual subtype of glioblastoma multiforme. Its characteristic features are biphasic configuration, constituting a definite, separate glial and sarcomatous differentiation, on histological evaluation. Herein, we present a rare case of Gliosarcoma that had presented only once in our center in last 13 years. A 60 years old, diabetic, hypertensive male patient came to e emergency department with disturbed level of consciousness and right sided hemiplegia which was progressive over four days. On examination he was, conscious, unoriented in time, person or place, his mouth deviated to left and vitally stable. After initial evaluation, CT scan and MRI were advised. These showed a complex left parieto-occipital heterogeneous mass lesion with cystic and solid components, measuring approximately 5.2x4cm. The mass lesion was seen displacing the occipital horn anteriorly and inferiorly with probable extension into the lateral ventricular cavity. There was no associated midline shift or definite herniation. The lesion was diagnosed as highly suggestive of brain tumor with a differential diagnosis of glioblastoma multiforme or ependymoma. Blood picture revealed a rapidly increasing level of anemia. Surgical intervention comprising left parieto-occipital craniotomy and near total resection of the tumor was carried out. On histopathological and immunohistochemical evaluation the diagnosis of GS was established. A plan of a combination of adjuvant chemotherapy and radiation was formulated that was however, declined by the family. On regular follow up, the patients clinical state rapidly deteriorated with persistence of seizures and requirement of repeated blood transfusions. The patient finally passed away after eighth months.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Gliosarcoma/diagnóstico , Adulto , Antineoplásicos/uso terapéutico , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/terapia , Craneotomía , Diagnóstico Diferencial , Resultado Fatal , Gliosarcoma/patología , Gliosarcoma/terapia , Humanos , Imagen por Resonancia Magnética , Masculino , Tomografía Computarizada por Tomografía de Emisión de Positrones , Radioterapia Adyuvante , Insuficiencia del TratamientoRESUMEN
Objective: Gliosarcoma (GSC), a rare malignant brain tumor, is considered as a variant of isocitrate dehydrogenase 1 wild type (IDH1-WT) glioblastoma (GBM). This study aimed to retrospectively analyze the clinical characteristics of GSC and compare whether there are some differences of treatment strategies and outcomes between GSC and GBM patients through Surveillance, Epidemiology, and End Results (SEER) database.Patients and methods: The clinical data of adults diagnosed with primary GSC between 2004 and 2015 were queried from SEER database. The Kaplan-Meier curve and the Cox model were performed to analyze the relationships between clinical parameters and patients' prognosis. Similar analyses were conducted for all primary GBM patients of SEER.Results: In total, 527 GSC and 20,541 GBM patients with complete and valid clinical information were finally enrolled for further analysis. Compared with GBM, GSC owned a proclivity to temporal lobe rather than frontal lobe (p < 0.001), a less conservative extension of resection (EOR) (p < 0.001), and a higher sensitivity to radiotherapy (p < 0.001). As shown by univariate analysis, surgery, radiotherapy and chemotherapy could prolong the overall survival (OS) time of GSC, but EOR did not confer an advantage to the outcomes of patients, no matter whether combined radio/chemotherapy was given. In multivariate analysis, age more than 60 and lack of radio/chemotherapy were identified as independent risk factors for OS of GSC patients.Conclusions: Our study found that although EOR seemed to be important to GBM, the extent of surgery did not show a clear relationship with the improved prognosis of GSC. Additionally, radiotherapy and chemotherapy could prolong patients' survival time significantly, which suggests a more positive role of them in treating GSC and needs further investigations.
Asunto(s)
Neoplasias Encefálicas , Gliosarcoma , Adulto , Neoplasias Encefálicas/epidemiología , Neoplasias Encefálicas/terapia , Glioblastoma/diagnóstico , Glioblastoma/epidemiología , Glioblastoma/terapia , Gliosarcoma/diagnóstico , Gliosarcoma/epidemiología , Gliosarcoma/terapia , Humanos , Pronóstico , Estudios RetrospectivosRESUMEN
BACKGROUND: The authors report a case of a woman aged 33 years who suffered from the combination of primary gliosarcoma and arteriovenous malformation as the first clinical presentation of intracranial hemorrhage. CASE DESCRIPTION: Subsequently, gene examination rarely finds BRAF V600E mutation in the surgical specimen. The lesion was not completely identified with magnetic resonance imaging and digital subtraction angiography. CONCLUSIONS: This case illustrates the occult and unusual feature of gliosarcoma. The pathogenesis of such coexistence might be related to underlying genetic alterations.
Asunto(s)
Malformaciones Arteriovenosas/genética , Astrocitoma/genética , Gliosarcoma/genética , Proteínas Proto-Oncogénicas B-raf/genética , Adulto , Angiografía de Substracción Digital/métodos , Malformaciones Arteriovenosas/patología , Astrocitoma/diagnóstico , Astrocitoma/patología , Femenino , Gliosarcoma/diagnóstico , Gliosarcoma/patología , Humanos , Hemorragias Intracraneales/diagnóstico , Hemorragias Intracraneales/patologíaRESUMEN
BACKGROUND: Gliosarcomas are malignant tumors of the central nervous system. As a variant of glioblastomas (GBM), they are treated in a similar fashion. However, there is growing evidence to suggest that they may be a separate entity. METHODS: Due to the rarity of primary gliosarcomas (PGS), here we publish data from a single center spanning over 14 years, comprising possibly one of the biggest case series in the literature to our knowledge. RESULTS: The mean age at presentation was 59 years with male preponderance (1.75:1). The most common presenting symptoms were balance and mobility issues (61%), followed by headaches (50%) and visual problems (39%). Tumours were most likely to involve the frontal and parietal lobes (27% and 21% respectively). Patients under 50 had a significant survival advantage (50% versus 32%). All patients had surgery, 79% had adjuvant radiotherapy, with a further 21% also receiving chemotherapy. Median survival from surgery of patients diagnosed with PGS was 6.6 months. Median and one-year survival were significantly better for patients who received radiotherapy (14 months; 46% one year survival) and improved further with combined radio- and chemotherapy (30 months; 77%, one year survival). CONCLUSIONS: For patients of good functional status, adjuvant chemo-radiotherapy is warranted and should be offered as it confers a much-improved overall survival.
Asunto(s)
Neoplasias Encefálicas , Gliosarcoma , Adulto , Anciano , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/epidemiología , Neoplasias Encefálicas/terapia , Femenino , Gliosarcoma/diagnóstico , Gliosarcoma/epidemiología , Gliosarcoma/terapia , Humanos , Masculino , Persona de Mediana Edad , Reino Unido/epidemiologíaRESUMEN
Because the study population with gliosarcoma (GSM) is limited, the understanding of this disease is insufficient. In this study, the authors aimed to determine the clinical characteristics and independent prognostic factors influencing the prognosis of GSM patients and to develop a nomogram to predict the prognosis of GSM patients after craniotomy. A total of 498 patients diagnosed with primary GSM between 2004 and 2015 were extracted from the 18 Registries Research Data of the Surveillance, Epidemiology, and End Results (SEER) database. The median disease-specific survival (DSS) was 12.0 months, and the postoperative 0.5-, 1-, and 3-year DSS rates were 71.4%, 46.4% and 9.8%, respectively. We applied both the Cox proportional hazards model and the decision tree model to determine the prognostic factors of primary GSM. The Cox proportional hazards model demonstrated that age at presentation, tumour size, metastasis state and adjuvant chemotherapy (CT) were independent prognostic factors for DSS. The decision tree model suggested that age <71 years and adjuvant CT were associated with a better prognosis for GSM patients. The nomogram generated via the Cox proportional hazards model was developed by applying the rms package in R version 3.5.0. The C-index of internal validation for DSS prediction was 0.67 (95% confidence interval (CI), 0.63 to 0.70). The calibration curve at one year suggested that there was good consistency between the predicted DSS and the actual DSS probability. This study was the first to develop a disease-specific nomogram for predicting the prognosis of primary GSM patients after craniotomy, which can help clinicians immediately and accurately predict patient prognosis and conduct further treatment.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Gliosarcoma/diagnóstico , Nomogramas , Factores de Edad , Anciano , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/cirugía , Craneotomía , Femenino , Gliosarcoma/mortalidad , Gliosarcoma/patología , Gliosarcoma/cirugía , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Modelos de Riesgos Proporcionales , Sistema de Registros , Programa de VERF , Análisis de SupervivenciaRESUMEN
OBJECTIVES: To retrospectively review the radiological and clinicopathological features of gliosarcoma (GSM) and differentiate it from glioblastoma multiforme (GBM). METHODS: The clinicopathological data and imaging findings (including VASARI analysis) of 48 surgically and pathologically confirmed GSM patients (group 1) were reviewed in detail, and were compared with that of other glioblastoma (GBM) cases in our hospital (group 2). RESULTS: There were 28 men and 20 women GSM patients with a median age of 52.5 years (range, 24-80 years) in this study. Haemorrhage (n = 21), a salt-and-pepper sign on T2-weighted images (n = 36), unevenly thickened wall (n = 36) even appearing as a paliform pattern (n = 32), an intra-tumoural large feeding artery (n = 32) and an eccentric cystic portion (ECP) (n = 19) were more commonly observed in the GSM group than in GBM patients. Based on our experience, GSM can be divided into four subtypes according to magnetic resonance imaging (MRI) features. When compared to GBM (group 2), there were more patients designated with type III lesions (having very unevenly thickened walls) and IV (solid) lesions among the GSM cases (group 1). On univariate prognostic analysis, adjuvant therapy (radiotherapy, chemotherapy, and radiochemotherapy) and existence of an eccentric cyst region were prognostic factors. However, Cox's regression model showed only adjuvant therapy as a prognostic factor for GSM. CONCLUSIONS: When compared to GBM, certain imaging features are more likely to occur in GSM, which may help raise the possibility of this disease. All GSM patients are recommended to receive adjuvant therapy to achieve a better prognosis with radiotherapy, chemotherapy or radiochemotherapy all as options. KEY POINTS: ⢠Diagnosis of gliosarcoma can be suggested preoperatively by imaging. ⢠Gliosarcoma can be divided into four subtypes based on MRI. ⢠Paliform pattern and ECP tend to present in gliosarcoma more than GBM. ⢠The cystic subtype of gliosarcoma may predict a more dismal prognosis. ⢠All gliosarcoma patients should receive adjuvant therapy to achieve better prognosis.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Encéfalo/diagnóstico por imagen , Gliosarcoma/diagnóstico , Imagen por Resonancia Magnética/métodos , Tomografía Computarizada por Rayos X/métodos , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
Determination of the isocitrate dehydrogenase (IDH) mutation status, presence or absence of mutation in IDH genes (IDH1 or IDH2), has become one of the most important molecular features taken into account in the management of patients with diffuse gliomas. Tumors that are IDH-mutant have a better prognosis than their counterparts with similar histologic grade and IDH-wildtype phenotype. IDH1-R132H is the most common IDH mutation, present in ~90% of IDH-mutant cases. This mutation yields an altered protein that can be detected by immunohistochemistry. We evaluated the IDH1-R132H antibody (clone H09) to determine IDH mutation status as the first line test and compared with the results of polymerase chain reaction (PCR) testing that can detect more types of mutations in IDH1 or IDH2. A total of 62 gliomas were evaluated: 30 glioblastomas (including 3 gliosarcomas), 11 grade III diffuse gliomas, 17 grade II diffuse gliomas, and 4 circumscribed gliomas. Twelve of 62 cases were IDH-mutant by immunohistochemistry and 15 of 62 by PCR. PCR detected the following mutations: IDH1-R132H (11 cases), IDH1-R132C (1 case), IDH2 R172, NOS (1 case), IDH1 R132, NOS (1 case), and IDH2-R172K (1 case). The R132H antibody had high specificity (100%) and sensitivity (80%) to detect IDH mutation status; the discordant results were 3 false-negatives. IDH-R132H immunostain is suitable as a first line test. Nonimmunoreactive cases could be studied by PCR following recommendations of the 2016 World Health Organization guidelines.
Asunto(s)
Neoplasias Encefálicas/diagnóstico , Glioma/diagnóstico , Gliosarcoma/diagnóstico , Inmunohistoquímica/métodos , Isocitrato Deshidrogenasa/metabolismo , Reacción en Cadena de la Polimerasa/métodos , Anticuerpos/metabolismo , Reacciones Falso Negativas , Humanos , Isocitrato Deshidrogenasa/genética , Isocitrato Deshidrogenasa/inmunología , Mutación/genética , Pronóstico , Reproducibilidad de los Resultados , Sensibilidad y EspecificidadRESUMEN
Gliosarcoma is a highly aggressive primary brain tumour. It is a relatively rare tumour and comprises of two histological components, glial and sarcomatous. Gliosarcomas carry a poorer prognosis than that of Glioblastoma Multiforme (GBM). The current review highlights important histological and radiological features of gliosarcoma in the light of recent literature, and also touches upon the treatment options and outcomes of various types of gliosarcoma.
Asunto(s)
Neoplasias Encefálicas/patología , Gliosarcoma/patología , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/terapia , Gliosarcoma/diagnóstico , Gliosarcoma/diagnóstico por imagen , Gliosarcoma/terapia , Humanos , NeuroimagenRESUMEN
Gliosarcoma is a very rare brain tumor accounting for 1.8 -8% of all glial tumors. It has been classified by the World Health Organization as a variant of glioblastoma. It is a tumor with double glial and sarcomatous component. Patient's clinical picture is polymorphic, imaging data are evocative, diagnosis is based on histology. Treatment is always surgical. Prognosis is closely linked to the quality of resection. We here report two clinical cases with the aim of assessing the diagnostic, therapeutic and prognostic features of this rare entity.
Asunto(s)
Neoplasias Encefálicas/patología , Gliosarcoma/patología , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Gliosarcoma/diagnóstico , Gliosarcoma/terapia , Humanos , Masculino , Persona de Mediana Edad , PronósticoRESUMEN
A 90-year-old woman presented with 1-year history of right-sided progressive proptosis, neovascular glaucoma, blindness, and worsening ocular pain. No funduscopic examination was possible because of a corneal opacity. Head CT scan without contrast demonstrated a heterogeneous 4.1 cm (anterior-posterior) by 1.7 cm (transverse) cylindrical mass arising in the right optic nerve and extending from the retrobulbar globe to the optic canal. She underwent palliative enucleation with subtotal resection of the orbital optic nerve and tumor. Pathological examination showed effacement of the optic nerve by an infiltrative high-grade glial neoplasm with biphasic sarcomeric differentiation. Invasion into the uvea and retina was present. The neoplasm was negative for melan-A, HMB45, tyrosinase, synaptophysin, smooth muscle actin, and epithelial membrane antigen. The glioma had strongly intense, but patchy immunopositivity for glial fibrillary acidic protein. Multiple foci of neoplastic cells had pericellular reticulin staining. The overall features were diagnostic of a gliosarcoma (World Health Organization grade IV) of the optic nerve. Postoperative MRI demonstrated postsurgical changes and residual gliosarcoma with extension into the optic chiasm. The patient died 2 and a half months after her enucleation surgery at her nursing home. Autopsy was unavailable due to the caregiver wishes, making a definitive cause of death unknown. Gliosarcoma is a rare variant of glioblastoma, and this is the first documented case presenting as a primary neoplasm of the optic nerve.
Asunto(s)
Gliosarcoma/diagnóstico , Neoplasias del Nervio Óptico/diagnóstico , Nervio Óptico/diagnóstico por imagen , Anciano de 80 o más Años , Resultado Fatal , Femenino , Humanos , Imagen por Resonancia Magnética , Glioma del Nervio Óptico/diagnóstico , Tomografía Computarizada por Rayos XRESUMEN
Gliosarcoma is a rare variant of glioblastoma multiforme characterized by a biphasic pattern of glial and mesenchymal differentiation. Fine-needle aspiration (FNA) is routinely employed to diagnose a number of primary and secondary malignancies in a variety of clinical settings. Herein, we describe the cytomorphologic features of a gliosarcoma metastatic to an extracranial location accompanied by corresponding clinico-radiologic and histopathologic findings in a 51-year-old man with a posterior mediastinal mass. The cytologic smears displayed a pleomorphic tumor comprised of spindled and epithelioid malignant cells. This case illustrates the ability of FNA to adequately diagnose a rare malignancy in the appropriate clinico-radiologic setting.