Gliosarcoma – Clinico-pathology, Genetics and a Review of Rare Congenital Cases

2009 ◽  
Vol 05 (01) ◽  
pp. 20
Author(s):  
Christopher Dunham ◽  
Keyword(s):  
World Health Organization ◽  
Brain Tumours ◽  
Poor Prognosis ◽  
Genetic Data ◽  
World Health ◽  
Recent Analysis ◽  
Molecular Profile ◽  
Low Grade ◽  
Who Grade ◽  
Health Organization

Congenital brain tumours are rare. In general, they are preferentially located in the supratentorial compartment, and despite the occurrence of low-grade entities, these tumours are associated with a very poor prognosis. When strictly defined, the most common forms of congenital neoplasia are teratomas and astrocytomas. Among the astrocytomas, all grades (World Health Organization [WHO] I–IV) and many of the different subtypes are represented. This includes the most prognostically worrisome ‘diffusely infiltrating’ astrocytomas. Gliosarcomas are a variant of glioblastoma (i.e. WHO grade IV astrocytoma) that exhibits both malignant astrocytic (i.e. glioblastoma) and mesenchymal (i.e. sarcoma) components. Despite their bi-phasic histology, genetic analyses of adult gliosarcoma cases suggest not only a molecular profile similar to glioblastoma, but also a monoclonal histogenesis for the glial and sarcomatous elements. Congenital gliosarcomas are extremely rare, with only a handful of cases being described in the literature. Not surprisingly, therefore, detailed clinical, pathological and genetic data are limited. However, based on a recent analysis of congenital glioblastomas, congenital gliosarcomas may constitute an entity that is genetically and prognostically distinct from adult cases.

Association between fluorine-18–labeled fluorodeoxyglucose uptake and 1p and 19q loss of heterozygosity in World Health Organization Grade II gliomas

2007 ◽  
Vol 106 (4) ◽  
pp. 633-637 ◽  
Author(s):  
Florian Stockhammer ◽  
Ulrich-Wilhelm Thomale ◽  
Michail Plotkin ◽  
Christian Hartmann ◽  
Andreas von Deimling
Keyword(s):  
World Health Organization ◽  
Fdg Pet ◽  
Magnetic Resonance Images ◽  
World Health ◽  
Low Grade ◽  
Who Grade ◽  
Fdg Uptake ◽  
Grade Ii ◽  
Health Organization ◽  
Who Grade Ii Gliomas

Object Oligodendroglial tumors harboring combined 1p and 19q loss (1p/19q LOH) are characterized by a favorable prognosis and response to chemotherapy and radiotherapy, but detection of 1p/19q LOH relies on postoperative procedures. The authors investigated the potential of fluorine-18–labeled fluorodeoxyglucose (FDG) uptake in positron emission tomography (PET) to predict 1p/19q LOH preoperatively in tumors whose appearance on initial magnetic resonance images was consistent with that of low-grade glioma. Methods The study population comprised 25 patients who had undergone preoperative FDG-PET followed by tumor resection. Neuronavigation ensured a precise match of FDG uptake wi th the site of biopsy. All tumor specimens were graded according to the World Health Organization (WHO) classification system. Microsatellite analysis was used to identify 1p/19q LOH. In this series, 16 of 25 gliomas corresponded to WHO Grade II. In eight of these 16, 1p/19q LOH was detected. Raised glucose utilization within the tumor was seen in the six of eight WHO Grade II gliomas with 1p/19q LOH and in none of the WHO Grade II gliomas without this genetic alteration (p = 0.003). Conclusions These findings demonstrate the potential of FDG-PET to predict 1p/19q LOH in WHO Grade II gliomas.

scholarly journals Seizure response to temozolomide chemotherapy in patients with WHO grade II oligodendroglioma: a single-institution descriptive study

2018 ◽  
Vol 6 (3) ◽  
pp. 203-208 ◽  
Author(s):  
Aya Haggiagi ◽  
Edward K Avila
Keyword(s):  
Disease Progression ◽  
Seizure Frequency ◽  
World Health ◽  
Low Grade ◽  
Inclusion Criteria ◽  
Who Grade ◽  
Seizure Reduction ◽  
Grade Ii ◽  
Health Organization

Abstract Background Tumor-related epilepsy (TRE) is common in patients with low-grade oligodendrogliomas. TRE is difficult to control despite multiple antiepileptic drugs (AEDs) in up to 30% of patients. Chemotherapy has been used for treatment to avoid potential radiotherapy-related neurotoxicity. This study evaluates the effect of temozolomide on seizure frequency in a homogeneous group with World Health Organization (WHO) grade II oligodendrogliomas. Methods A retrospective analysis was conducted of adult patients with WHO grade II oligodendrogliomas and TRE followed at Memorial Sloan Kettering between 2005 and 2015 who were treated with temozolomide alone either as initial treatment or for disease progression. All had seizures 3 months prior to starting temozolomide. Seizure frequency was reviewed every 2 cycles and at the end of temozolomide treatment. Seizure reduction of ≥50% compared to baseline was defined as improvement. Results Thirty-nine individuals met inclusion criteria. Median follow-up since starting temozolomide was 6 years (0.8-13 years). Reduction in seizure frequency occurred in 35 patients (89.7%). Improvement was independent of AED regimen adjustments or prior antitumor treatment in 16 (41%); of these, AED dosage was successfully reduced or completely eliminated in 10 (25.6%). Twenty-five patients (64.1%) remained on a stable AED regimen. The majority (n = 32, 82%) had radiographically stable disease, 5 (12.8%) had objective radiographic response, and 2 (5.2%) had disease progression. Conclusions Temozolomide may result in reduced seizure frequency, and permit discontinuation of AEDs in patients with WHO II oligodendroglioma. Improvement was observed irrespective of objective tumor response on MRI, emphasizing the importance of incorporating seizure control in assessing response to tumor-directed therapy.

scholarly journals Can Proliferation Biomarkers Reliably Predict Recurrence in World Health Organization 2003 Defined Endometrial Stromal Sarcoma, Low Grade?

PLoS ONE ◽  
2013 ◽  
Vol 8 (10) ◽  
pp. e75899 ◽  
Author(s):  
Weiwei Feng ◽  
Anais Malpica ◽  
Ivar Skaland ◽  
Einar Gudlaugsson ◽  
Stanley J. Robboy ◽  
...  
Keyword(s):  
World Health Organization ◽  
Endometrial Stromal Sarcoma ◽  
World Health ◽  
Low Grade ◽  
Stromal Sarcoma ◽  
Health Organization

scholarly journals Recent Changes of Classification for Squamous Intraepithelial Lesions of the Head and Neck

2018 ◽  
Vol 142 (7) ◽  
pp. 829-832 ◽  
Author(s):  
Kyung-Ja Cho ◽  
Joon Seon Song
Keyword(s):  
World Health Organization ◽  
Head And Neck ◽  
World Health ◽  
Low Grade ◽  
Squamous Intraepithelial Lesions ◽  
Cell Hyperplasia ◽  
Oral Epithelial Dysplasia ◽  
Grading Systems ◽  
Health Organization ◽  
Intraepithelial Lesions

Context.— Interpretation of atypical squamous lesions of the head and neck has always been a nettlesome task for pathologists. Moreover, many different grading systems for squamous intraepithelial lesions have been proposed in past decades. The recent World Health Organization 2017 classification presents 2 types of 2-tier systems for laryngeal and oral precursor lesions. Objective.— To review the recent changes in classification and the clinical significance for squamous intraepithelial lesions of the head and neck. Data Sources.— Personal experience and data from the literature. Conclusions.— The 2-tier grading system for laryngeal dysplasia, presented by World Health Organization in 2017, is expected to improve diagnostic reproducibility and clinical implication. However, the diagnostic criteria for low-grade dysplasia do not distinguish it clearly from basal cell hyperplasia. The World Health Organization 2017 classification of oral epithelial dysplasia remains unclear, and complicated and variable grading systems still make head and neck intraepithelial lesions difficult to interpret.

scholarly journals Behavior-Oriented Nomogram for the Stratification of Lower-Grade Gliomas to Improve Individualized Treatment

2020 ◽  
Vol 10 ◽  
Author(s):  
Ruo-Lun Wei ◽  
Li-Wei Zhang ◽  
Jian-Guo Li ◽  
Feng-Dong Yang ◽  
Ya-Ke Xue ◽  
...  
Keyword(s):  
Dna Methyltransferase ◽  
Predictive Performance ◽  
Individualized Treatment ◽  
World Health ◽  
Low Grade ◽  
Lower Grade ◽  
Validation Group ◽  
Who Grade ◽  
Health Organization ◽  
Cortical Involvement

Secondary glioblastomas (sGBM) are derived from previously lower-grade [World Health Organization (WHO) grades II or III] gliomas. Lower-grade benign-behaving gliomas may retain their former grade following recurrence, or may become malignant higher-grade glioblastomas. Prediction of tumor behavior in lower-grade gliomas is critical for individualized glioma therapy. A total of 89 patients were included between January 2000 and January 2019 in the present study to establish a nomogram via univariate and multivariate logistic regression analyses. Nomogram predictive performance was tested in the validation group. We then analyzed 36 O-6-methylguanine-DNA methyltransferase (MGMT) unmethylated lower-grade gliomas from patients seen at West China Hospital of Sichuan University. Survival statistics were calculated with the Kaplan-Meier method. Two clinical factors (molecular diagnosis and WHO grade), five radiological factors (location, cortical involvement, multicentricity, uniformity, and margin enhancement), one biomarker (1p19q codeletion), and a combination of three biomarkers (IDH+/ATRX-/TP53-) were associated with glioma upgrading. Nomograms positive for these prognostic factors had an AUC of 0.880 in the derivation group and 0.857 in the validation group. The calibration and score-stratified survival curves for the derivation group and validation group were good. An operational nomogram was published at https://warrenwrl.shinyapps.io/DynNomapp/. The overall survival of secondary gliomas in the MGMT-unmethylated cohort were influenced independently by the use of temozolomide during the treatment of formerly low-grade gliomas (p=0.00096). Clinical and radiological factors and biomarker-based behavior-oriented nomograms may offer a feasible identification tool for the detection of sGBM precursors. This method may further assist neurosurgeons with the stratification of lower-grade glioma cases and thus the development of better, more individualized treatment plans.

Intraorbital Meningiomas: A Pathologic Review Using Current World Health Organization Criteria

2010 ◽  
Vol 134 (5) ◽  
pp. 766-770 ◽  
Author(s):  
Deepali Jain ◽  
Katayoon B. Ebrahimi ◽  
Neil R. Miller ◽  
Charles G. Eberhart
Keyword(s):  
Optic Nerve ◽  
World Health Organization ◽  
Neurofibromatosis Type ◽  
Clinical Findings ◽  
World Health ◽  
Who Grade ◽  
Brain Invasion ◽  
Adjacent Structures ◽  
Grade Ii ◽  
Health Organization

Abstract Context.—Meningiomas represent approximately 4% of all intraorbital tumors and can arise from the optic nerve or extend into the orbit from adjacent structures. Objective.—To examine a cohort of intraorbital meningiomas and use the current World Health Organization (WHO) scheme to assess the effect of changes to the classification of tumors at this site. Design.—The histopathology and clinical findings of intraorbital meningiomas resected between 1968 and 2008 at our institution were reviewed according to the WHO 2007 classification scheme. Results.—A total of 51 intraorbital meningiomas were reviewed. The mean age at presentation was 45 years, but 5 tumors arose in children. Two patients were known to have neurofibromatosis type 2, and 1 had inherited retinoblastoma. Orbital meningiomas were more frequently encountered in women (30 cases) than in men (21 cases). In 21 patients, the tumor was associated with the optic nerve. The most common (25 of 51 tumors; 49%) histopathologic subtype was meningothelial. Most (47 of 51; 92%) of the tumors were WHO grade I. Four tumors (8%) were WHO grade II, with 4 or more mitotic figures per 10 high-power fields, brain invasion, chordoid histology, or a combination of these features. Conclusions.—Intraorbital meningiomas were most frequently of the meningothelial or transitional subtypes and were WHO grade I. One relatively common intracranial subtype, fibrous meningioma, was not encountered. The percentage of WHO grade II tumors in the orbit (8%) is similar to that reported for intracranial tumors using the current grading scheme.

Low-Grade Papillary Urothelial Carcinoma of the Urinary Bladder: A Clinicopathologic Analysis of a Post–World Health Organization/International Society of Urological Pathology Classification Cohort From a Single Academic Center

2010 ◽  
Vol 134 (8) ◽  
pp. 1160-1163
Author(s):  
Hiroshi Miyamoto ◽  
Fadi Brimo ◽  
Luciana Schultz ◽  
Huihui Ye ◽  
Jeremy S. Miller ◽  
...  
Keyword(s):  
Urothelial Carcinoma ◽  
World Health Organization ◽  
International Society ◽  
World Health ◽  
Low Grade ◽  
High Grade ◽  
The Mean ◽  
Papillary Urothelial Carcinoma ◽  
Health Organization ◽  
Grade Progression

Abstract Context.—Few large cohort studies have addressed outcome in patients with noninvasive low-grade papillary urothelial carcinoma (LG-UrCa) following implementation of the 2004 World Health Organization/International Society of Urological Pathology (WHO/ISUP) consensus classification. Objective.—To evaluate our cohort of LG-UrCa cases classified according to 2004 WHO/ISUP to reassess outcome and interobserver agreement. Design.—Files were searched for all patients diagnosed with LG-UrCa between 1998 and 2008. All sections were reevaluated for accuracy of classification. Results.—A total of 112 cases initially diagnosed as LG-UrCa were identified. Of those, 8 of 55 cases (15%) initially diagnosed by nonurologic pathologists were reclassified as high-grade papillary urothelial carcinoma and were excluded. The mean length of follow-up was 40.1 months (range, 2–113 months). Tumor recurrence was encountered in 56 of 104 patients (53.8%), including 37 (35.6%) with LG-UrCa or lower-grade tumors and 19 (18.3%) with high-grade papillary urothelial carcinoma. Of the 19 patients demonstrating grade progression, 7 (37%) also developed stage progression (invasive carcinoma, n  =  5; metastatic carcinoma, n  =  2). Seven patients eventually underwent radical cystectomy. None of the 104 patients died of bladder cancer. The mean number of recurrence episodes was 3.11. The mean durations of time to first recurrence and time to grade progression were 13.9 months and 25.1 months, respectively. The mean size of initial tumors was 1.73 cm. There was no significant correlation between tumor size, patient age, sex, or smoking history and the likelihood for recurrence or grade progression. A significantly higher rate of recurrence was seen in patients with multiple tumors at initial diagnosis (P  =  .04). Conclusions.—A tendency to underdiagnose high-grade papillary urothelial carcinoma continues to exist. More than half (53.8%) of patients with LG-UrCa developed recurrence, with an 18.3% incidence of grade progression and a 6.7% incidence of stage progression. Patients with multiple initial tumors had significantly higher risk of developing recurrence.

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