scholarly journals Gliosarcoma with Osteosarcomatous Component: A Case Report and Short Review Illustration

Author(s):  
Yanming Chen ◽  
Sujuan Zhou ◽  
Xuelan Zhou ◽  
Xiaoxiao Dai ◽  
Liping Wang ◽  
...  

Abstract Background: Gliosarcoma (GS) represents a rare variant of glioblastoma in the central nervous system, characterized by biphasic histopathological features of gliomatous and sarcomatous components. Here, we present an unusual case of GS, which also demonstrated osteosarcomatous component. Case presentations: The patient underwent gross total resection (GTR) of right temporal lobe lesion. Subsequently received external beam radiation therapy with 60 Gy and chemotherapy, postoperatively. The sarcomatous portion of the typical fibrosarcoma pattern mingled with areas of osteoid structure in this 65-year-old feminine case. The molecular pathological analysis demonstrated IDH1/2 wild-type and MGMT promoter island methylated phenotype. Target Enrichment Sequencing (TES) was performed on the gliomatous and sarcomatous components of the tumor tissues. TERT promoter, RB1, NF1, TP53 mutations and copy number variations (CNVs) on chromosome 7, 10q, 11q, 12, 13, 17 and 22 were observed in gliomatous and fibro-sarcomatous mixed tumor tissue; While we found TERT promoter, RB1, TP53 mutations and CNVs on chromosome 2q, 3q, 7, 8, 9, 10, 11, 12, 13, 15, 16, 17, 18, 19 and 22 in osteosarcomatous tumor tissue. Noteworthy, EGFR amplification was not observed in both gliomatous and sarcomatous tumor tissues. Conclusions: In conclusion, integrated with histopathology, molecular pathology, and genomic alteration analysis, we report a case of GS with an extremely rare histopathologic phenotype of osteosarcomatous differentiation, also suffered lung multi-metastases. Additionally, by reviewing the literature, our study of this unusual differentiation of GS into osteosarcoma provides novel insight into the natural history of GS.

2010 ◽  
Vol 113 (2) ◽  
pp. 310-318 ◽  
Author(s):  
Kaoru Tamura ◽  
Masaru Aoyagi ◽  
Hiroaki Wakimoto ◽  
Noboru Ando ◽  
Tadashi Nariai ◽  
...  

Object Recent evidence suggests that a glioma stem cell subpopulation might contribute to radioresistance in malignant gliomas. To investigate this hypothesis, the authors examined recurrent malignant gliomas for histopathological changes after high-dose irradiation with Gamma Knife surgery (GKS) and external beam radiation therapy (EBRT). Methods Thirty-two patients with malignant gliomas (Grade 3 in 8 patients, Grade 4 in 24) underwent GKS in combination with EBRT. Serial MR and L-[methyl-11C] methionine PET images were employed to assess remnant or recurrent tumors after GKS. Twelve patients underwent surgical removal after GKS and EBRT. Histological sections were subjected to immunohistochemistry for MIB-1, factor VIII, and stem cell markers, nestin and CD133. Results The site of GKS treatment failure was local in 16 (76.2%) of 21 patients with glioblastomas showing progression; in 9 of these 16 patients, the recurrence clearly arose within the target lesion of GKS. Histopathological examination after GKS and EBRT showed variable mixtures of viable tumor tissues and necrosis. Viable tumor tissues exhibited high MIB-1 indices but reduced numbers of tumor blood vessels. There was marked accumulation of CD133-positive glioma cells, particularly in remnant tumors within the necrotic areas, in sections obtained after GKS plus EBRT, whereas CD133-positive cells appeared very infrequently in primary sections prior to adjuvant treatment. Conclusions The results indicate that CD133-positive glioma stemlike cells can survive high-dose irradiation, leading to recurrence, despite prolonged damage to tumor blood vessels. This could be an essential factor limiting the effectiveness of GKS plus EBRT for malignant gliomas.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii173-ii173
Author(s):  
Tareq Juratli ◽  
Naema Nayyar ◽  
Michael Young ◽  
Megha Subramanian ◽  
Priscilla Brastianos ◽  
...  

Abstract INTRODUCTION Esthesioneuroblastoma (ENB) is a rare, malignant neuroectodermal tumor of the olfactory epithelium. To date, a few recurrent genetic alterations have been identified in ENB. Here, we sought to examine the genomic signature on a series of clinically well-characterized aggressive ENB samples. METHODS We performed whole-exome sequencing in a cohort of 26 ENB samples from 12 patients, containing 11 matched primary-metastatic samples. Additionally, targeted sequencing was carried out in all samples to determine TERT promoter hotspot mutations. Furthermore, we performed immunohistochemistry (IHC) using an antibody that recognizes the dystrophin central rod domain in all available specimens. RESULTS Our cohort consisted of 9 male and 3 female patients with a median age of 66 years at first diagnosis (4- 77 years). One patient was staged Kadish B at the time of diagnosis and eleven were staged Kadish C. The median overall survival was 3.85 years (0.3 – 16 years). Consistent with previous findings, each tumor exhibited a different mutational signature and the mutational landscape appears to be predominantly driven by copy number variations. Interestingly, we detected intragenic deletions in dystrophin (DMD) as the most common and consistent alteration in ENB patients (in 11 of 12 patients, 91.6%). DMD deletions, when identified within the primary ENB, were preserved in all subsequent metastatic lesions. Moreover, DMD deletions where concurrently identified in three cases with multiple metastases. IHC revealed the concurrent loss of dystrophin expression, the protein encoded by DMD, in all cases with DMD deletions. Otherwise, no other recurrent genomic findings were detected, including TERT promoter mutations. CONCLUSIONS Our findings validate previously described DMD deletions as the most common recurrent genomic alteration in primary ENB. Furthermore, our data demonstrate that DMD deletions were perpetuated in subsequent metastatic lesions, and when identified in any metastasis, were present in other metastases from the same patient.


2007 ◽  
Vol 177 (4S) ◽  
pp. 376-377 ◽  
Author(s):  
Bryan J. Donnelly ◽  
John C. Saliken ◽  
Penny Brasher ◽  
Scott Ernst ◽  
Harold Lau ◽  
...  

2020 ◽  
pp. 1-10
Author(s):  
Dhiego C. A. Bastos ◽  
Rafael A. Vega ◽  
Jeffrey I. Traylor ◽  
Amol J. Ghia ◽  
Jing Li ◽  
...  

OBJECTIVEThe objective of this study was to present the results of a consecutive series of 120 cases treated with spinal laser interstitial thermal therapy (sLITT) to manage epidural spinal cord compression (ESCC) from metastatic tumors.METHODSThe electronic records of patients treated from 2013 to 2019 were analyzed retrospectively. Data collected included demographic, pathology, clinical, operative, and imaging findings; degree of epidural compression before and after sLITT; length of hospital stay; complications; and duration before subsequent oncological treatment. Independent-sample t-tests were used to compare means between pre- and post-sLITT treatments. Survival was estimated by the Kaplan-Meier method. Multivariate logistic regression was used to analyze predictive factors for local recurrence and neurological complications.RESULTSThere were 110 patients who underwent 120 sLITT procedures. Spinal levels treated included 5 cervical, 8 lumbar, and 107 thoracic. The pre-sLITT Frankel grades were E (91.7%), D (6.7%), and C (1.7%). The preoperative ESCC grade was 1c or higher in 92% of cases. Metastases were most common from renal cell carcinoma (39%), followed by non–small cell lung carcinoma (10.8%) and other tumors (35%). The most common location of ESCC was in the vertebral body (88.3%), followed by paraspinal/foraminal (7.5%) and posterior elements (4.2%). Adjuvant radiotherapy (spinal stereotactic radiosurgery or conventional external beam radiation therapy) was performed in 87 cases (72.5%), whereas 33 procedures (27.5%) were performed as salvage after radiotherapy options were exhausted. sLITT was performed without need for spinal stabilization in 87 cases (72.5%). Post-sLITT Frankel grades were E (85%), D (10%), C (4.2%), and B (0.8%); treatment was associated with a median decrease of 2 ESCC grades. The local control rate at 1 year was 81.7%. Local control failure occurred in 25 cases (20.8%). The median progression-free survival was not reached, and overall survival was 14 months. Tumor location in the paraspinal region and salvage treatment were independent predictors of local recurrence, with hazard ratios of 6.3 and 3.3, respectively (p = 0.01). Complications were observed in 22 cases (18.3%). sLITT procedures performed in the lumbar and cervical spine had hazard ratios for neurological complications of 15.4 and 17.1 (p < 0.01), respectively, relative to the thoracic spine.CONCLUSIONSsLITT is safe and provides effective local control for high-grade ESCC from vertebral metastases in the thoracic spine, particularly when combined with adjuvant radiotherapy. The authors propose considering sLITT as an alternative to open surgery in selected patients with spinal metastases.


2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 37-41 ◽  
Author(s):  
William F. Regine ◽  
Roy A. Patchell ◽  
James M. Strottmann ◽  
Ali Meigooni ◽  
Michael Sanders ◽  
...  

Object. This investigation was performed to determine the tolerance and toxicities of split-course fractionated gamma knife radiosurgery (FSRS) given in combination with conventional external-beam radiation therapy (CEBRT). Methods. Eighteen patients with previously unirradiated, gliomas treated between March 1995 and January 2000 form the substrate of this report. These included 11 patients with malignant gliomas, six with low-grade gliomas, and one with a recurrent glioma. They were stratified into three groups according to tumor volume (TV). Fifteen were treated using the initial FSRS dose schedule and form the subject of this report. Group A (four patients), had TV of 5 cm3 or less (7 Gy twice pre- and twice post-CEBRT); Group B (six patients), TV greater than 5 cm3 but less than or equal to 15 cm3 (7 Gy twice pre-CEBRT and once post-CEBRT); and Group C (five patients), TV greater than 15 cm3 but less than or equal to 30 cm3 (7 Gy once pre- and once post-CEBRT). All patients received CEBRT to 59.4 Gy in 1.8-Gy fractions. Dose escalation was planned, provided the level of toxicity was acceptable. All patients were able to complete CEBRT without interruption or experiencing disease progression. Unacceptable toxicity was observed in two Grade 4/Group B patients and two Grade 4/Group C patients. Eight patients required reoperation. In three (38%) there was necrosis without evidence of tumor. Neuroimaging studies were available for evaluation in 14 patients. Two had a partial (≥ 50%) reduction in volume and nine had a minor (> 20%) reduction in size. The median follow-up period was 15 months (range 9–60 months). Six patients remained alive for 3 to 60 months. Conclusions. The imaging responses and the ability of these patients with intracranial gliomas to complete therapy without interruption or experiencing disease progression is encouraging. Excessive toxicity derived from combined FSRS and CEBRT treatment, as evaluated thus far in this study, was seen in patients with Group B and C lesions at the 7-Gy dose level. Evaluation of this novel treatment strategy with dose modification is ongoing.


10.37206/88 ◽  
2005 ◽  
Author(s):  
Ellen Yorke ◽  
Rodica Alecu ◽  
Li Ding ◽  
Doracy Fontenla ◽  
Andre Kalend ◽  
...  

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