Rare sacral extradural grade II ependymoma: a comprehensive review of literature

2021 ◽  
Vol 14 (11) ◽  
pp. e246540
Author(s):  
Ricardo J Fernández-de Thomas ◽  
Natalie Amaral-Nieves ◽  
Orlando De Jesus ◽  
Emil A Pastrana
Keyword(s):  
Treatment Guidelines ◽  
Myxopapillary Ependymoma ◽  
Low Grade ◽  
Review Of Literature ◽  
Total Resection ◽  
Comprehensive Review ◽  
Sacral Region ◽  
Rare Tumours ◽  
Grade Ii ◽  
Sacral Spinal Cord

Sacral spinal cord ependymoma is an uncommon pathology. Most of the reported cases are consistent with a myxopapillary ependymoma histopathologic subtype. Non-myxopapillary ependymomas rarely occur in the sacral region. Most lesions are intradural; however, rare extradural cases can occur. We present the case of a 46-year-old female patient diagnosed with a grade II sacral extradural ependymoma, emphasising the importance of an interdepartmental case approach for diagnosis and management. Even though grade II ependymomas are considered low grade, the potential for recurrence and metastatic disease has been reported. There are no treatment guidelines for these rare tumours besides gross total resection.

Giant intradural myxopapillary ependymoma: review of literature

2021 ◽  
Vol 14 (1) ◽  
pp. e239453
Author(s):  
Orlando De Jesus
Keyword(s):  
Spinal Cord ◽  
Back Pain ◽  
Single Point ◽  
Myxopapillary Ependymoma ◽  
Review Of Literature ◽  
Significant Morbidity ◽  
Total Resection ◽  
Arm Pain ◽  
Progressive Paraparesis ◽  
Minimal Residual

This report presents the longest spanning intradural myxopapillary ependymoma consisting of 23 vertebral segments in the literature. An 11-year-old boy presented with right arm pain, mid back pain and progressive paraparesis associated with urinary retention. On MRI, the patient was found to have an intradural lesion extending from C5 to S3. The patient underwent T7 and T8 laminectomies with an almost total resection except for a minimal residual adhering to the spinal cord. The patient with the largest spanning spinal cord ependymoma was managed satisfactorily without significant morbidity. A small laminectomy may be used in some occasions despite the tumour’s extensive size because it may have a single point of attachment to the cord.

Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases

2012 ◽  
Vol 9 (6) ◽  
pp. 646-653 ◽  
Author(s):  
James H. Stephen ◽  
Angela J. Sievert ◽  
Peter J. Madsen ◽  
Alexander R. Judkins ◽  
Adam C. Resnick ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Protein Expression ◽  
Pediatric Patients ◽  
Pathological Diagnosis ◽  
Gross Total Resection ◽  
Low Grade ◽  
Total Resection ◽  
Significant Difference ◽  
Grade Ii

Object Primary spinal cord ependymomas (EPNs) are rare in children, comprising classical WHO Grade II and III tumors and Grade I myxopapillary ependymomas (MEPNs). Despite their benign histology, recurrences and neural-axis dissemination have been reported in up to 33% MEPNs in the pediatric population. Treatment options beyond resection are limited, and little is known about their tumorigenesis. The purpose of this study was to explore the tumor biology and outcomes in a consecutive series of pediatric patients treated at a single institution. Methods The authors performed a retrospective clinicopathological review of 19 patients at a tertiary referral children's hospital for resection of a spinal cord ependymoma. The population included 8 patients with a pathological diagnosis of MEPN and 11 patients with a pathological diagnosis of spinal EPN (10 cases were Grade II and 1 case was Grade III). The upregulation of the following genes HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was studied by immunohistochemistry from archived paraffin-embedded tumor samples of the entire cohort to compare the expression in MEPN versus EPN. Results Gross-total resection was achieved in 75% of patients presenting with MEPNs and in 100% of those with EPNs. The average follow-up period was 79 months for the MEPN subset and 53 months for Grade II/III EPNs. Overall survival for both subsets was 100%. However, event-free survival was only 50% for patients with MEPNs. Of note, in all cases involving MEPNs that recurred, the patients had undergone gross-total resection on initial surgery. In contrast, there were no tumor recurrences in patients with EPNs. Immunohistochemistry revealed no significant differences in protein expression between the two tumor types with the exception of EPHB3, which demonstrates a tendency to be positive in MEPNs (6 reactive tumors of 9) rather than in EPN (2 reactive tumors of 10). Conclusions The authors' experience shows that, following a gross-total resection, MEPNs are more likely to recur than their higher-grade counterpart, EPNs. This supports the recommendation for close long-term radiological follow-up of pediatric patients with MEPNs to monitor for recurrence, despite the tumor's low-grade histological feature. No significant difference in the protein expression of HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was present in this selected cohort of pediatric patients.

Letter to the Editor Regarding “Association Between Facility Volume and Overall Survival for Patients with Grade II Meningioma after Gross Total Resection”

2020 ◽  
Vol 142 ◽  
pp. 537
Author(s):  
Gustavo Correa Lordelo ◽  
Victor Salviato Nespoli ◽  
Iuri Santana Neville ◽  
Wellingson Silva Paiva
Keyword(s):  
Overall Survival ◽  
Gross Total Resection ◽  
Total Resection ◽  
Letter To The Editor ◽  
Grade Ii

scholarly journals Transcavernous Resection of a Giant Extensive Chondrosarcoma with Endoscopic Assistance

2021 ◽  
Author(s):  
Walid Elshamy ◽  
Burcak Soylemez ◽  
Sima Sayyahmelli ◽  
Nese Keser ◽  
Mustafa K. Baskaya
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Skull Base ◽  
Internal Carotid ◽  
Gross Total Resection ◽  
Petrous Apex ◽  
Left Internal Carotid Artery ◽  
Total Resection ◽  
Complete Occlusion ◽  
Grade Ii

AbstractChondrosarcomas are one of the major malignant neoplasms which occur at the skull base. These tumors are locally invasive. Gross total resection of chondrosarcomas is associated with longer progression-free survival rates. The patient is a 55-year-old man with a history of dysphagia, left eye dryness, hearing loss, and left-sided facial pain. Magnetic resonance imaging (MRI) showed a giant heterogeneously enhancing left-sided skull base mass within the cavernous sinus and the petrous apex with extension into the sphenoid bone, clivus, and the cerebellopontine angle, with associated displacement of the brainstem (Fig. 1). An endoscopic endonasal biopsy revealed a grade-II chondrosarcoma. The patient was then referred for surgical resection. Computed tomography (CT) scan and CT angiogram of the head and neck showed a left-sided skull base mass, partial destruction of the petrous apex, and complete or near-complete occlusion of the left internal carotid artery. Digital subtraction angiography confirmed complete occlusion of the left internal carotid artery with cortical, vertebrobasilar, and leptomeningeal collateral development. The decision was made to proceed with a left-sided transcavernous approach with possible petrous apex drilling. During surgery, minimal petrous apex drilling was necessary due to autopetrosectomy by the tumor. Endoscopy was used to assist achieving gross total resection (Fig. 2). Surgery and postoperative course were uneventful. MRI confirmed gross total resection of the tumor. The histopathology was a grade-II chondrosarcoma. The patient received proton therapy and continues to do well without recurrence at 4-year follow-up. This video demonstrates steps of the combined microsurgical skull base approaches for resection of these challenging tumors.The link to the video can be found at: https://youtu.be/WlmCP_-i57s.

scholarly journals A survival analysis of surgically treated incidental low-grade glioma patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Lingcheng Zeng ◽  
Qi Mei ◽  
Hua Li ◽  
Changshu Ke ◽  
Jiasheng Yu ◽  
...  
Keyword(s):  
Progression Free Survival ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Total Resection ◽  
Surgical Timing ◽  
Asymptomatic Group ◽  
Free Survival ◽  
Symptomatic Group ◽  
Significant Difference ◽  
Asymptomatic Phase

AbstractTo evaluate the surgical effect on survival in patients with incidental low-grade glioma (LGG) through comparison between asymptomatic and symptomatic patients. The medical records of surgically treated adult cerebral incidental LGG (iLGG) patients in our department between January 2008 and December 2015 were retrospectively reviewed. The survival of patients was calculated starting from the initial imaging diagnosis. Factors related to progression-free survival (PFS), overall survival (OS) and malignant progression-free survival (MPFS) were statistically analyzed. Seventy-five iLGG patients underwent surgery: 49 in the asymptomatic group, who underwent surgery in the asymptomatic period, and 26 in the symptomatic group, who underwent surgery after the tumor had grown and the patients had developed tumor-related symptoms. Significantly more tumors were initially located adjacent to the functional area in the symptomatic group than in the asymptomatic group (P < 0.05), but there was no significant difference in the total resection rate between the two groups. The incidence of postoperative complications (15.4%) and postoperative epilepsy (23.1%) was higher in the symptomatic group than in the asymptomatic group (4.1% and 10.2%, respectively). Multivariate analysis showed that surgical timing, namely, surgery performed before or after symptom occurrence, had no significant effect on PFS, OS or MPFS, while total resection significantly prolonged PFS, OS and MPFS, and the pathology of oligodendroglioma was positively correlated with PFS and OS (P < 0.05). Surgical timing for iLGGs should facilitate total resection. If total resection can be achieved, even after symptom occurrence, patients can achieve comparable survival benefits to those treated with surgery in the asymptomatic phase.

scholarly journals RTID-05. INDIGO: A GLOBAL, RANDOMIZED, DOUBLE-BLIND, PHASE 3 STUDY OF VORASIDENIB (AG-881) VS PLACEBO IN PATIENTS WITH RESIDUAL/RECURRENT GRADE II GLIOMA WITH AN ISOCITRATE DEHYDROGENASE 1/2 (IDH1/2) MUTATION

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii194-ii194
Author(s):  
Ingo Mellinghoff ◽  
Martin van den Bent ◽  
Jennifer Clarke ◽  
Elizabeth Maher ◽  
Katherine Peters ◽  
...  
Keyword(s):  
High Risk ◽  
Dose Escalation ◽  
Objective Response ◽  
Data Monitoring Committee ◽  
Progression Free Survival ◽  
High Risk Population ◽  
Low Grade ◽  
Who Grade ◽  
Free Survival ◽  
Grade Ii

Abstract BACKGROUND Low-grade gliomas (LGGs; WHO grade II) are incurable and ultimately progress to high-grade gliomas. The current treatment options are surgery followed by observation (“watch and wait”) for patients with lower risk for disease progression or postoperative chemoradiotherapy (high-risk population). There are no approved targeted therapies. IDH1 and IDH2 mutations (mIDH1/2) occur in approximately 80% and 4% of LGGs, respectively, and promote tumorigenesis via neomorphic production of D-2-hydroxyglutarate. Vorasidenib, an oral, potent, reversible, brain-penetrant pan-inhibitor of mIDH1/2, was evaluated in 76 patients with glioma in two phase 1 studies (dose escalation and perioperative) and was associated with a favorable safety profile at daily doses below 100 mg. Preliminary clinical activity was observed in non-enhancing glioma patients in both studies, with an objective response rate (ORR) of 18.2% and median progression-free survival of 31.4 months in the dose escalation study. METHODS Approximately 366 patients will be randomized 1:1 to vorasidenib (50 mg QD) or matched placebo and stratified by 1p19q status (intact vs co-deleted). Key eligibility criteria: age ≥ 12 years; grade II oligodendroglioma or astrocytoma (per WHO 2016 criteria) not in need of immediate treatment and without high-risk features; centrally confirmed mIDH1/2 status; ≥ 1 surgery for glioma with most recent ≥ 1 year but ≤ 5 years before randomization, and no other anticancer therapy; Karnofsky performance status ≥ 80%; and centrally confirmed measurable, non-enhancing disease evaluable by magnetic resonance imaging. Crossover from placebo to the vorasidenib arm is permitted upon centrally confirmed radiographic progression per RANO-LGG criteria. Primary endpoint: progression-free survival assessed by independent review. Secondary endpoints: safety and tolerability, tumor growth rate assessed by volume, ORR, overall survival, and quality of life. Clinical data will be reviewed regularly by an independent data monitoring committee. The study is currently enrolling patients in the US, with additional countries planned (NCT04164901).

scholarly journals Occurrence of a Clonal T-Cell Population in a Case of Chronic Myelomonocytic Leukemia

2021 ◽  
Vol 14 ◽  
pp. 263485352199150
Author(s):  
Anupama Patil ◽  
Balasaheb Wanve ◽  
Pradeep Kar ◽  
Shanthi Velusamy
Keyword(s):  
T Cell ◽  
Cell Population ◽  
De Novo ◽  
Treatment Guidelines ◽  
Myeloproliferative Neoplasm ◽  
Genetic Mutation ◽  
Pcr Analysis ◽  
Low Grade ◽  
Monocytic Leukemia ◽  
Laboratory Findings

Chronic myelo-monocytic leukemia (CMML) is an aggressive myeloid neoplasm with some features of a myelodysplastic syndrome (MDS) and others of a myeloproliferative neoplasm (MPN). Rarely, patients with CMML have a co-existing lympho-proliferative disorder (LPD). In most cases, the lymphoid neoplasm is diagnosed first, and the CMML is considered to be a secondary therapy-induced form of leukemia. We report herein a unique case of de-novo CMML, with an underlying clonal T-cell population and describe its clinical presentation and laboratory findings. A 70-year old male presented with a 3-month history of cough, dsypnea, abdominal distension, and low-grade fever. Physical and radiological examination revealed hepatosplenomegaly but no lymphadenopathy. Peripheral blood had absolute monocytosis with marrow showing CMML with 10% blasts along with dysplasia in myeloid and erythroid lineages. Flow cytometry indicated possibility of chronic myelo-monocytic leukemia with 13% monocytic cells along with an additional clonal population of gamma/delta T cells (15%) with aberrant immunophenotype. Polymerase chain reaction (PCR) analysis was positive for clonal T-cell rearrangement. A diagnosis of CMML with an underlying clonal T-CLPD was made. The synchronous occurrence of CMML and T-cell neoplasm may be attributed to a genetic mutation common to both. Currently, there are no treatment guidelines for group of patients; hence individualized therapeutic strategies should be implemented to enable symptomatic improvement and provide optimum care.

scholarly journals A 25-year retrospective, single center analysis of 343 WHO grade II/III glioma patients: implications for grading and temozolomide therapy

2021 ◽  
Author(s):  
Eike Steidl ◽  
Katharina Filipski ◽  
Pia S. Zeiner ◽  
Marlies Wagner ◽  
Emmanouil Fokas ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Molecular Markers ◽  
Treatment Time ◽  
Real Life ◽  
Patient Characteristics ◽  
Low Grade ◽  
Who Grade ◽  
Idh1 R132h ◽  
Treatment Data ◽  
Grade Ii

Abstract Purpose Classification and treatment of WHO grade II/III gliomas have dramatically changed. Implementing molecular markers into the WHO classification raised discussions about the significance of grading and clinical trials showed overall survival (OS) benefits for combined radiochemotherapy. As molecularly stratified treatment data outside clinical trials are scarce, we conducted this retrospective study. Methods We identified 343 patients (1995–2015) with newly diagnosed WHO grade II/III gliomas and analyzed molecular markers, patient characteristics, symptoms, histology, treatment, time to treatment failure (TTF) and OS. Results IDH-status was available for all patients (259 mutant, 84 IDH1-R132H-non-mutant). Molecular subclassification was possible in 173 tumors, resulting in diagnosis of 80 astrocytomas and 93 oligodendrogliomas. WHO grading remained significant for OS in astrocytomas/IDH1-R132H-non-mutant gliomas (p < 0.01) but not for oligodendroglioma (p = 0.27). Chemotherapy (and temozolomide in particular) showed inferior OS compared to radiotherapy in astrocytomas (median 6.1/12.1 years; p = 0.03) and oligodendrogliomas (median 13.2/not reached (n.r.) years; p = 0.03). While radiochemotherapy improved TTF in oligodendroglioma (median radiochemotherapy n.r./chemotherapy 3.8/radiotherapy 7.3 years; p < 0.001/ = 0.06; OS data immature) the effect, mainly in combination with temozolomide, was weaker in astrocytomas (median radiochemotherapy 6.7/chemotherapy 2.3/radiotherapy 2.0 years; p < 0.001/ = 0.11) and did not translate to improved OS (median 8.4 years). Conclusion This is one of the largest retrospective, real-life datasets reporting treatment and outcome in low-grade gliomas incorporating molecular markers. Current histologic grading features remain prognostic in astrocytomas while being insignificant in oligodendroglioma with interfering treatment effects. Chemotherapy (temozolomide) was less effective than radiotherapy in both astrocytomas and oligodendrogliomas while radiochemotherapy showed the highest TTF in oligodendrogliomas.

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