Rosette-forming glioneuronal tumors of the posterior fossa

2010 ◽  
Vol 5 (1) ◽  
pp. 98-103 ◽  
Author(s):  
Manish N. Shah ◽  
Jeffrey R. Leonard ◽  
Arie Perry
Keyword(s):  
Posterior Fossa ◽  
Fourth Ventricle ◽  
Complete Resection ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Tumor Type ◽  
Who Grade ◽  
Pathological Characteristics ◽  
The Mean ◽  
Glioneuronal Tumors

Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a rare, recently described WHO Grade I neoplasm. The authors report 6 examples of RGNT arising primarily from the cerebellar vermis. All the patients were female, and the mean age of presentation was 24.8 years. The original diagnoses included pilocytic astrocytoma, ependymoma, cerebellar dysembryoplastic neuroepithelial tumor (DNT), and oligodendroglioma. The cases showed classic pathological characteristics, although in 2 cases the lesions included DNT-like “floating neurons” involving Purkinje cells, a feature which has not been previously reported to the authors' knowledge. The clinical outcome was excellent with no recurrences after complete resection. These cases expand the known clinical and histological spectrum of this rare tumor type. Given the lack of fourth ventricle involvement in most of these cases, the authors suggest revising the name to RGNT of the posterior fossa.

Fourth ventricle rosette-forming glioneuronal tumor

2006 ◽  
Vol 105 (1) ◽  
pp. 129-131 ◽  
Author(s):  
Mahlon Johnson ◽  
John Pace ◽  
Judy F. Burroughs
Keyword(s):  
Fourth Ventricle ◽  
Cranial Computed Tomography ◽  
Glioneuronal Tumor ◽  
Tumor Type ◽  
Optimal Management ◽  
Pilocytic Astrocytomas ◽  
Long Term Follow Up ◽  
Glioneuronal Tumors

✓ The authors describe a rosette-forming glioneuronal tumor of the fourth ventricle in a 29-year-old woman. She had been experiencing dizziness for 1 year and headaches for 1 month. Cranial computed tomography revealed a relatively circumscribed mass involving the inferior cerebellum and floor of the fourth ventricle with extension into the ventricle. Histologically, much of the tumor was piloid with Rosenthal fibers as well as telangiectatic blood vessels; other areas contained complete or incomplete neurocytic rosettes. This tumor type must be differentiated from pilocytic astrocytomas, other gliomas with a piloid glial component, and glioneuronal tumors arising from the floor of the fourth ventricle or inferior cerebellum. Recognition of, and long-term follow up for, this recently described pathological entity may clarify the nature of this lesion and strategies for its optimal management.

scholarly journals A giant solid cystic inferior fourth ventricle subependymoma – Case report and literature review

2016 ◽  
Vol 30 (4) ◽  
pp. 455-460
Author(s):  
A. Giovani ◽  
Narcisa Bucur ◽  
Ana Gheorghiu ◽  
Lena Papadopol ◽  
R.M. Gorgan
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Posterior Fossa ◽  
Fourth Ventricle ◽  
Precursor Cells ◽  
Benign Tumors ◽  
Total Resection ◽  
Who Grade ◽  
Slow Growing ◽  
Glial Precursor

Abstract Subependymomas are a rare subtype of ependymomas, slow growing WHO grade I tumors that develop either intracranial from the subependymal glial precursor cells layer of the ventricles or intramedullary. These tumors originate in the undifferentiated Subependymal layer of cells that can become either ependymocytes or astrocytes. Most of the subependymomas are located inside the fourth ventricle (50-60%). We reviewed the case of a 40 years old woman with a giant solid cystic fourth ventricle ependymoma. The patient underwent total resection of the tumor through a subociipital transvermian approach. We discussed the characteristics of these benign tumors and reviewed the literature on this subject and concluded that total resection is the treatment of choice for symptomatic Subependymomas localized in posterior fossa.

Complex Dysembryoplastic Neuroepithelial Tumor With Pilocytic Astrocytoma Component

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S44-S45
Author(s):  
Sandhyarani Dasaraju ◽  
Krutika Patel ◽  
Shalla Akbar ◽  
Osama Elkadi
Keyword(s):  
Contrast Enhancement ◽  
Pilocytic Astrocytoma ◽  
Early Recognition ◽  
Complex Form ◽  
Surgical Excision ◽  
Biological Behavior ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Who Grade ◽  
Complete Surgical Excision

Abstract Objectives Dysembryoplastic neuroepithelial tumor (DNET) is an uncommon mixed glioneuronal tumor. DNET is classified as WHO grade I neoplasm and commonly seen in the temporal lobe of children and young adults with features of pharmacoresistant seizures. The term complex DNET defines those tumors associated with additional features, including a pilocytic astrocytoma (PA) component, a finding that is rarely described in the literature. Methods Here we report a 16-year-old male who presented with seizures. On imaging, a 5.5 × 2.2 × 2.1-cm multiloculated mass was centered in the left frontoparietal gray matter with solid and cystic components and focal areas of contrast enhancement. Results Microscopic examination revealed a noninfiltrative glioneuronal neoplasm composed of a bland population of round to oval cells arranged around pools of mucinous material. Occasional large neuronal cells were noted. Focal areas composed of elongated astrocytic cells demonstrating piloid morphology were also present. No high-grade features were demonstrated. The findings are consistent with DNET, complex form in addition to a second component with piloid features resembling pilocytic astrocytoma. Conclusion The early recognition of DNET is important as it has an excellent prognosis on complete surgical excision without use of adjunct therapies. Unresected or incompletely removed DNET and secondary radiochemotherapy may give rise to recurrence and malignancy. The development of the secondary neoplasm challenges the stability in terms of biological behavior and highlights the importance of complete surgical excision and close follow-up. Our patient had neither undergone previous surgery nor received any adjuvant therapy, and hence this case is a de novo development of PA in a DNET. The association of contrast enhancement with DNET is an unusual finding in this case.

MULTIFOCAL COMPLEX GLIONEURONAL TUMOR IN AN ELDERLY MAN

Neurosurgery ◽  
2009 ◽  
Vol 64 (6) ◽  
pp. E1193-E1195 ◽  
Author(s):  
Jian-Qiang Lu ◽  
Bernd W. Scheithauer ◽  
Pranshu Sharma ◽  
James N. Scott ◽  
Ian F. Parney ◽  
...  
Keyword(s):  
Obstructive Hydrocephalus ◽  
External Ventricular Drain ◽  
Third Ventricle ◽  
Glioneuronal Tumor ◽  
Third Ventriculostomy ◽  
Magnetic Resonance Imaging Scan ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Histological Features ◽  
The Third ◽  
Glioneuronal Tumors

Abstract OBJECTIVE The clinicopathological spectra of a dysembryoplastic neuroepithelial tumor (DNT) and a rosette-forming glioneuronal tumor (RGNT) are expanding. We report here the autopsy findings of a case of complex glioneuronal tumor with combined histological features of both a DNT and an RGNT. CLINICAL PRESENTATION A 79-year-old man presented with a 1-month history of confusion and gait difficulties. A magnetic resonance imaging scan revealed obstructive hydrocephalus attributed to a mass in the posterior third ventricle. INTERVENTION A third ventriculostomy was performed. Postoperatively, the mass remained unchanged in size for more than 14 months. Thirty-eight months after his initial manifestations, he experienced minor head trauma and was then hospitalized. Despite placement of an external ventricular drain and other supportive treatment, he deteriorated and died. A full autopsy was performed, with emphasis on the brain. The mass lesion and a few independent microfoci situated primarily around the third ventricle showed histological features of pilocytic astrocytoma with recurrent hemorrhage. Far more numerous were microfoci with histological features of a DNT, including floating neurons, as well as typical RGNT-associated, synaptophysin-positive rosettes and perivascular pseudorosettes. CONCLUSION The advanced age of the patient, the coexisting histological features of the DNT and RGNT, and the distinctive anatomic distribution of the lesions, being centered on the third ventricle, may lend insight into the histogenetic relationship of a DNT, an RGNT, and mixed glioneuronal tumors.

Mixed Glioneuronal Tumors: Recently Described Entities

2007 ◽  
Vol 131 (2) ◽  
pp. 228-233
Author(s):  
Mark A. Edgar ◽  
Marc K. Rosenblum
Keyword(s):  
Fourth Ventricle ◽  
Data Sources ◽  
Glioneuronal Tumor ◽  
Cancer Center ◽  
Glial Tumors ◽  
Papillary Glioneuronal Tumor ◽  
Pathologic Findings ◽  
Glioneuronal Tumors

Abstract Context.—Several distinctive mixed glioneuronal tumors that warrant recognition as clinicopathologic entities have been recently described by neuropathologists. Objective.—To summarize important clinical, radiologic, and pathologic findings for 3 novel glioneuronal tumors (papillary glioneuronal tumor, rosetted glioneuronal tumor, and rosette-forming glioneuronal tumor of the fourth ventricle). Data Sources.—Recent reports in the pathology literature and the authors' experience with mixed glioneuronal tumors at a major cancer center. Conclusions.—Histologic features enabling recognition of these recently described glioneuronal tumors are presented along with remarks concerning the classification of mixed neuronal and glial tumors exhibiting unconventional appearances.

Epilepsy Surgery for Glioneuronal Tumors in Childhood

Neurosurgery ◽  
2014 ◽  
Vol 74 (6) ◽  
pp. 648-657 ◽  
Author(s):  
Georgia Ramantani ◽  
Navah Ester Kadish ◽  
Constantin Anastasopoulos ◽  
Armin Brandt ◽  
Kathrin Wagner ◽  
...  
Keyword(s):  
Cognitive Functioning ◽  
Epilepsy Surgery ◽  
Visual Memory ◽  
Refractory Epilepsy ◽  
Full Scale ◽  
Glioneuronal Tumor ◽  
Group Level ◽  
Verbal Iq ◽  
The Mean ◽  
Glioneuronal Tumors

ABSTRACT BACKGROUND: In contrast to the abundance of seizure outcome reports in epilepsy surgery for glioneuronal tumors in childhood and adolescence, there is a dearth of information regarding cognitive outcomes. OBJECTIVE: To investigate the seizure and cognitive outcome of children and adolescents that underwent resective surgery for glioneuronal tumor-associated refractory epilepsy and determine their predictive factors. METHODS: We retrospectively analyzed the presurgical findings, resection types, and outcomes over 1.3 to 12.3 years (mean, 7.3) of 29 consecutive patients, who underwent resection in 2000 to 2011. The mean age at epilepsy onset was 7.9 years (range, 0-15.4), the mean age at surgery was 11.7 years (range, 2.6-17.3), and the mean epilepsy duration to surgery was 3.8 years (range, 0.3-15.3). Etiology comprised 13 dysembryoplastic neuroepithelial tumors and 16 gangliogliomas, with additional focal cortical dysplasia in 5 cases. RESULTS: Eighty-six percent of children were seizure free 12 months after surgery; at final follow-up, 76% remained seizure free and 62% had discontinued antiepileptic drugs. Gross total resection was related to significantly higher rates of seizure freedom. Higher presurgical cognitive functioning (full-scale IQ, verbal IQ) was related to shorter epilepsy duration to surgery independent of age at epilepsy onset, thus determining postsurgical functioning. Improvements in verbal IQ, performance IQ, and visual memory as well as a trend toward improvement in full-scale IQ were established after surgery. Despite individual losses in full-scale IQ, verbal or visual memory, no deterioration was noted in any cognitive variable on a group level. CONCLUSION: Completeness of resection predisposes to favorable outcomes regarding seizure alleviation. Whereas cognitive functioning deteriorates with time in glioneuronal tumor-related refractory epilepsy, surgery is linked to improvement rather than to deterioration on a group level.

Symptomatic subependymoma of the fourth ventricle

1981 ◽  
Vol 55 (5) ◽  
pp. 841-844 ◽  
Author(s):  
Angelo Gandolfi ◽  
Riccardo E. Brizzi ◽  
Filomeno Tedeschi ◽  
Paolo Paini ◽  
Pellegrino Bassi
Keyword(s):  
Differential Diagnosis ◽  
Computerized Tomography ◽  
Posterior Fossa ◽  
Fourth Ventricle ◽  
Frozen Section ◽  
Surgical Removal ◽  
Tumor Type ◽  
Content Type ◽  
Direct Involvement ◽  
Fine Print

✓ Symptomatic subependymomas of the fourth ventricle are rare and usually not included in the preoperative differential diagnosis of tumors in this region. The case of a 63-year-old man with fourth ventricle subependymoma is described. For several years he had suffered with nausea and vomiting, and now presented signs of direct involvement of the posterior fossa. He was investigated preoperatively with computerized tomography, but the ultimate diagnosis was not suspected at that time. The tumor was diagnosed as an subependymoma at operation and was totally excised. This tumor type can easily be recognized on intraoperative frozen section, and its diagnosis should always lead to an attempt at complete surgical removal.

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Craniocervical Junction ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Younger Age ◽  
Curve Magnitude ◽  
The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

scholarly journals The Relationship between Inflammation Markers (CRP, IL-6, sCD40L) and Colorectal Cancer Stage, Grade, Size and Location

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1382
Author(s):  
Olga Martyna Koper-Lenkiewicz ◽  
Violetta Dymicka-Piekarska ◽  
Anna Justyna Milewska ◽  
Justyna Zińczuk ◽  
Joanna Kamińska
Keyword(s):  
Colorectal Cancer ◽  
Linear Regression ◽  
Tumor Size ◽  
Distal Colon ◽  
Proximal Colon ◽  
Model Parameters ◽  
Grade Group ◽  
Who Grade ◽  
The Mean ◽  
Mean Concentration

The aim of the study was the evaluation whether in primary colorectal cancer (CRC) patients (n = 55): age, sex, TNM classification results, WHO grade, tumor location (proximal colon, distal colon, rectum), tumor size, platelet count (PLT), mean platelet volume (MPV), mean platelet component (MCP), levels of carcinoembryonic antigen (CEA), cancer antigen (CA 19-9), as well as soluble lectin adhesion molecules (L-, E-, and P-selectins) may influence circulating inflammatory biomarkers: IL-6, CRP, and sCD40L. We found that CRP concentration evaluation in routine clinical practice may have an advantage as a prognostic biomarker in CRC patients, as this protein the most comprehensively reflects clinicopathological features of the tumor. Univariate linear regression analysis revealed that in CRC patients: (1) with an increase in PLT by 10 × 103/μL, the mean concentration of CRP increases by 3.4%; (2) with an increase in CA 19-9 of 1 U/mL, the mean concentration of CRP increases by 0.7%; (3) with the WHO 2 grade, the mean CRP concentration increases 3.631 times relative to the WHO 1 grade group; (4) with the WHO 3 grade, the mean CRP concentration increases by 4.916 times relative to the WHO 1 grade group; (5) with metastases (T1-4N+M+) the mean CRP concentration increases 4.183 times compared to non-metastatic patients (T1-4N0M0); (6) with a tumor located in the proximal colon, the mean concentration of CRP increases 2.175 times compared to a tumor located in the distal colon; (7) in patients with tumor size > 3 cm, the CRP concentration is about 2 times higher than in patients with tumor size ≤ 3 cm. In the multivariate linear regression model, the variables that influence the mean CRP value in CRC patients included: WHO grade and tumor localization. R2 for the created model equals 0.50, which indicates that this model explains 50% of the variance in the dependent variable. In CRC subjects: (1) with the WHO 2 grade, the mean CRP concentration rises 3.924 times relative to the WHO 1 grade; (2) with the WHO 3 grade, the mean CRP concentration increases 4.721 times in relation to the WHO 1 grade; (3) with a tumor located in the rectum, the mean CRP concentration rises 2.139 times compared to a tumor located in the distal colon; (4) with a tumor located in the proximal colon, the mean concentration of CRP increases 1.998 times compared to the tumor located in the distal colon; if other model parameters are fixed.

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