Disseminated ependymomas of the central nervous system

1996 ◽  
Vol 85 (4) ◽  
pp. 618-624 ◽  
Author(s):  
Ali R. Rezai ◽  
Henry H. Woo ◽  
Mark Lee ◽  
Henry Cohen ◽  
David Zagzag ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Proliferation Index ◽  
Significant Variable ◽  
Low Grade ◽  
High Grade ◽  
Primary Tumor Site ◽  
Tumor Dissemination ◽  
Proliferative Indices ◽  
Disseminated Disease

✓ Ependymomas are rare central nervous system (CNS) neoplasms that occasionally disseminate along the neuraxis or to extraneural sites. Definitive criteria predictive of dissemination have yet to be determined. One hundred forty patients with CNS ependymoma (88 primary spinal and 52 primary intracranial tumors) were surgically treated by the senior author (F.J.E.) between 1986 and 1994. Sixteen patients (11.4%) demonstrated tumor dissemination. The disseminated group consisted of 11 (12.5%) of 88 primary spinal and five (9.6%) of 52 primary intracranial ependymomas. The authors retrospectively reviewed the patients with CNS ependymoma and have identified several characteristics associated with dissemination from the primary tumor site. The mean time from diagnosis to dissemination was 6.8 years. The patients with disseminated disease were younger (16.8 vs. 28.3 years old, p = 0.02), had fewer gross-total resections (29% vs. 68%, p = 0.015), and had tumors with higher proliferative indices (MIB-1 staining, 13.14% vs. 2.06%, p = 0.02). High-grade tumors had a mean proliferation index of 21%, versus 2.4% and 1.6% for myxopapillary and low-grade tumors, respectively (p = 0.0003). In contrast to previous studies, tumor histology was the most significant variable for time to dissemination as determined by multivariate analysis (p = 0.008). Myxopapillary and high-grade tumors were 3.6 and 5.6 times more likely to have a shorter time to dissemination than low-grade tumors. In addition, dissemination is associated with a worse prognosis. At follow-up review, 31% of patients with disseminated disease had died compared to 7% of patients without dissemination (p = 0.04). It is concluded that younger patients with subtotal resections, myxopapillary or high-grade histology, and tumors with high proliferative indices are at substantial risk for the development of disseminated disease during their clinical course.

scholarly journals Central nervous system tumors: Radiologic pathologic correlation and diagnostic approach

2015 ◽  
Vol 6 (02) ◽  
pp. 191-197 ◽  
Author(s):  
Ishita Pant ◽  
Sujata Chaturvedi ◽  
Deepak Kumar Jha ◽  
Rima Kumari ◽  
Samta Parteki
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Metastatic Tumor ◽  
Rural Setting ◽  
Complete Agreement ◽  
Histopathological Diagnosis ◽  
Low Grade ◽  
High Grade ◽  
Metastatic Tumors ◽  
Diagnostic Algorithms

ABSTRACT Objectives: This study was conducted to formulate location-wise radiologic diagnostic algorithms and assess their concordance with the final histopathological diagnosis so as to evaluate their utility in a rural setting where only basic facilities are available. Materials and Methods: A retrospective analysis to assess the concordance of radiology (primarily MRI) with final histopathology report was done. Based on the most common incidence of tumor location and basic radiology findings, diagnostic algorithms were prepared. Results: For supratentorial intraaxial parenchymal location concordance was seen in all high-grade astrocytomas, low- and high-grade oligodendrogliomas, metastatic tumors, primitive neuroectodermal tumors, high-grade ependymomas, neuronal and mixed neuro-glial tumors and tumors of hematopoietic system. Lowest concordance was seen in low-grade astrocytomas. In the supratentorial intraaxial ventricular location, agreement was observed in choroid plexus tumors, ependymomas, low-grade astrocytomas and meningiomas; in the supratentorial extraaxial location, except for the lack of concordance in the only case of metastatic tumor, concordance was observed in meningeal tumors, tumors of the sellar region, tumors of cranial and paraspinal nerves; the infratentorial intraaxial parenchymal location showed agreement in low- as well as high-grade astrocytomas, metastatic tumors, high-grade ependymoma, embryonal tumors and hematopoietic tumors; in the infratentorial intraaxial ventricular location, except for the lack of concordance in one case of low-grade astrocytoma and two cases of medulloblastomas, agreement was observed in low- and high-grade ependymoma; infratentorial extraaxial tumors showed complete agreement in all tumors of cranial and paraspinal nerves, meningiomas, and hematopoietic tumors. Conclusion: A location-based approach to central nervous system (CNS) tumors is helpful in establishing an appropriate differential diagnosis.

scholarly journals Oncology is changing: the challenge of effectively assessing response within clinical trials in low-grade gliomas

2011 ◽  
pp. 75-76
Author(s):  
Ilaria Imarisio ◽  
Chiara Fumagalli
Keyword(s):  
Central Nervous System ◽  
Clinical Trials ◽  
Nervous System ◽  
Who Classification ◽  
Low Grade ◽  
High Grade ◽  
Low Grade Gliomas ◽  
Microvascular Proliferation ◽  
Grade Iii

The current WHO classification of primary central nervous system (CNS) tumors recognizes four separate tumor grades (I–IV), which can be grouped into low-grade (I and II) or high-grade (III and IV), depending on the absence or presence of high-grade histopathological features, such as microvascular proliferation and necrosis...

Oncology is changing: the challenge of effectively assessing response within clinical trials in low-grade gliomas

2011 ◽  
Vol 5 (2) ◽  
pp. 75
Author(s):  
Ilaria Imarisio ◽  
Chiara Fumagalli
Keyword(s):  
Central Nervous System ◽  
Clinical Trials ◽  
Nervous System ◽  
Who Classification ◽  
Low Grade ◽  
High Grade ◽  
Low Grade Gliomas ◽  
Microvascular Proliferation ◽  
Grade Iii

The current WHO classification of primary central nervous system (CNS) tumors recognizes four separate tumor grades (I–IV), which can be grouped into low-grade (I and II) or high-grade (III and IV), depending on the absence or presence of high-grade histopathological features, such as microvascular proliferation and necrosis...

scholarly journals RONC-22. SECOND TUMORS IN PEDIATRIC PATIENTS TREATED WITH PROTON THERAPY TO THE CENTRAL NERVOUS SYSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii459-iii459
Author(s):  
Daniel J Indelicato ◽  
James Bates ◽  
Raymond Mailhot-Vega ◽  
Christopher Morris ◽  
Eric Sandler ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Proton Therapy ◽  
Cumulative Incidence ◽  
Pediatric Patients ◽  
Photon Radiation ◽  
Low Grade ◽  
High Grade ◽  
Second Tumors

Abstract BACKGROUND Previous institutional data suggests the 10-year cumulative incidence of second tumors is 3% in children treated with photon radiation for central nervous system (CNS) malignancy, with 90% of these tumors occurring in areas receiving ≤36 Gy. Comparative figures for children treated with proton therapy (PT) does not exist. METHODS 1056 consecutive pediatric patients with a median follow-up of 5.0 years were treated between 2006–2019 with double-scattered PT to a site within the craniospinal axis. 230 patients were ≤3 years old and 14 had neurofibromatosis. A second tumor was defined as any solid neoplasm with histologic features different from the original tumor that had arisen within the irradiated volume. RESULTS Five patients developed second tumors resulting in a 5- and 10-year cumulative incidence of 0.2% (95% CI: 0–1.2%) and 1.6% (95% CI: 0.6%-3.9%), respectively. Of those who developed second tumors, median age at radiation was 4.3 years old (range, 2.1 to 5.1 years old) and diagnoses consisted of medulloblastoma (n=2), ependymoma (n=2), and craniopharyngioma (n=1). The second tumors included high grade gliomas (n=3) and high grade sarcoma (n=1) that occurred in regions receiving at least 54 Gy. One patient with neurofibromatosis developed both a low-grade glioma and choroidal melanoma in craniospinal irradiation regions receiving 36 Gy. Four of five patients with second tumors are alive. CONCLUSION The reduction in moderate-to-low dose radiation exposure from proton therapy may be associated with a decreased incidence of second tumors in children treated for CNS neoplasms. More follow-up is needed to confirm these findings.

Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients

2003 ◽  
Vol 98 (6) ◽  
pp. 1182-1187 ◽  
Author(s):  
Robert D. Ecker ◽  
W. Richard Marsh ◽  
Bruce E. Pollock ◽  
Özlem Kurtkaya-Yapicier ◽  
Robyn McClelland ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Survival Rate ◽  
Mayo Clinic ◽  
Recurrent Disease ◽  
External Beam Radiation ◽  
Low Grade ◽  
High Grade ◽  
Content Type ◽  
Fine Print

Object. The authors reviewed the Mayo Clinic experience with the treatment of hemangiopericytoma in the primary central nervous system (CNS). Methods. A retrospective study of all patients at the Mayo Clinic revealed 38 who had been treated for hemangiopericytoma in the CNS. Twenty of these patients were diagnosed in the decade between 1990 and 2000; 18 were initially diagnosed and underwent surgery before 1990. In the patients treated since 1990, the 5-year Kaplan—Meier survival rate was 93%. The 5-year disease-free survival rate was 89%. Sixty percent of patients treated with the aid of stereotactic radiosurgery for recurrent disease were alive 4.4 years after their initial treatment. Salvage chemotherapy was not effective. No survival benefit was detected in patients who had received initial adjuvant external-beam radiation therapy. High-grade tumors recurred 6.7 years earlier than did low-grade lesions (p = 0.004). Conclusions. The 5-year survival rate in patients with hemangiopericytoma of the CNS has improved at the authors' institution during the last 10 years. Although the reason for this is not entirely clear, the authors suspect that the improved treatment of patients with cancer, a 0% intraoperative mortality rate, and the use of radiosurgery in the treatment of recurrent disease all likely contribute. High-grade tumors recurred statistically significantly earlier than low-grade lesions. Current chemotherapies are ineffective in the treatment of hemangiopericytoma of the CNS.

The Rare Phenomenon of Loss of INI1 Expression at Recurrence/Progression of Primary Central Nervous System Tumors: Report of 3 Cases

2019 ◽  
Vol 28 (3) ◽  
pp. 341-347 ◽  
Author(s):  
Akash Pramod Sali ◽  
Vishal Chaubey ◽  
Duhita Kodare ◽  
Ayushi Sahay ◽  
Sridhar Epari
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Protein Expression ◽  
Complete Loss ◽  
Glioneuronal Tumor ◽  
Low Grade ◽  
High Grade ◽  
Immunohistochemical Expression ◽  
Not Otherwise Specified ◽  
Expression Loss

It is extremely rare for loss of immunohistochemical expression of INI1 to occur primarily at recurrence/progression with retained expression at the primary/initial presentation of central nervous system (CNS) tumor. In this article, we present 3 such cases showing loss of INI1 expression primarily at recurrence. All patients were males, aged 7 years (case 1), 11 years (case 2), and 35 years (case 3), diagnosed with low-grade glial/glioneuronal tumor, not otherwise specified (case 1), craniopharyngioma (case 2), and glioblastoma (case 3); all showed retained INI1 protein expression. Case 1 at 12 months recurrence showed a high-grade tumor with relative undifferentiated morphology, case 2 after 104 months showed a sarcomatous progression, and case 3 recurred after 4 months with the presence of relative undifferentiated round cells. All these recurrences showed loss of INI1 expression. Loss of SMARCB1/INI1 gene function resulting in complete loss of INI1 protein expression is not a well-accepted genetic mechanism for transformation/progression as this series emphasizes.

scholarly journals Perfusion and permeability MRI in glioma grading

2020 ◽  
Vol 51 (1) ◽  
Author(s):  
Sonay Aydin ◽  
Erdem Fatihoğlu ◽  
Pınar Nercis Koşar ◽  
Elif Ergün
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sensitivity And Specificity ◽  
Perfusion Mri ◽  
Low Grade ◽  
High Grade ◽  
Brain Glioma ◽  
Grade Group ◽  
Low Grade Gliomas ◽  
The Central Nervous System

Abstract Background MRI is successful in showing the anatomy of probable pathologies of the central nervous system. Although it may not be sufficient to reveal physiological and metabolic changes, advanced MRI techniques, such as perfusion and permeability MRI, are the key to overcoming these limitations. The aim of this study was to detect the efficacy of permeability and perfusion MRI techniques. Results The study included 38 patients with a pathology result of primary brain glioma. The permeability MRI (Ktrans, Ve), perfusion MRI values (CBV, CBF), and pathology results were evaluated. The high-grade group included 22 patients, and the low-grade group, 16 patients. Mean CBV and CBF, median Ktrans, and Ve values were higher in the high-grade group. All parameters tended to elevate with grade and had a positive correlation. CBV > 2.25, with sensitivity and specificity of 100%, CBF > 2.02, with sensitivity and specificity of 100%, Ktrans > 0.043, with sensitivity of 81.82% and specificity of 100%, and Ve > 0.255, with sensitivity and specificity of 100%, can predict high grade. Conclusion Perfusion and permeability MRI can be used safely for the differentiation of high- and low-grade gliomas and for the prediction of glioma grades.

Sign in / Sign up

Export Citation Format

Share Document