Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course

OBJECT Ganglioglioma (GG) is commonly recognized as a low-grade tumor located in the temporal lobe, often presenting with seizures. Most are amenable to complete resection and are associated with excellent oncological outcome. The authors encountered several GGs in various locations, which seem to have a less favorable clinical course than GGs in the temporal lobe. METHODS The authors performed a single-center retrospective review of all children with a histological diagnosis of GG who were treated at Children’s Hospital Colorado between 1997 and 2013. Each tumor was categorized by 2 pediatric neuroradiologists as typical or atypical based on preoperative MRI appearance. Typical lesions were cortically based, within a single cerebral lobe, well-circumscribed, and solid or mixed solid/cystic. The treatment and clinical course of each patient was analyzed. RESULTS Thirty-seven children were identified, with a median age at presentation of 8.2 years and median follow-up of 38.0 months. Eighteen tumors (48.6%) were typical and 19 (51.4%) were atypical. All typical lesions presented with seizures, whereas no atypical lesions did so. Sixteen (88.9%) typical lesions were located in the temporal lobe. In the atypical group, tumor location was variable, including 11 (57.9%) in the brainstem. Death during follow-up was statistically more common in the atypical group (31.6% vs 0%, p = 0.02). Gross-total resection (GTR) was achieved for 15 of 16 typical tumors (93.8%), compared with 3 atypical tumors (15.8%, p < 0.0001). Presentation with seizure or non-brainstem location were each associated with survival (p = 0.02 and 0.004, respectively). The presence of mutation in BRAF exon 15 did not differ between the 2 groups. CONCLUSIONS Pediatric GG with typical imaging features is associated with excellent rates of GTR and overall survival. Atypical GG is commonly encountered, less amenable to GTR, and associated with a worse outcome. This may relate to anatomical or biological characteristics and merits further investigation.

Download Full-text

Chronic epilepsy associated with temporal tumors: long-term surgical outcome

Neurosurgical FOCUS ◽

10.3171/2009.5.focus0998 ◽

2009 ◽

Vol 27 (2) ◽

pp. E6 ◽

Cited By ~ 22

Author(s):

Dmitry Ruban ◽

Richard W. Byrne ◽

Andres Kanner ◽

Michael Smith ◽

Elizabeth J. Cochran ◽

...

Keyword(s):

Temporal Lobe ◽

Intractable Epilepsy ◽

Complete Resection ◽

Low Grade ◽

Diffuse Astrocytoma ◽

Seizure Onset ◽

Chronic Epilepsy ◽

The Mean

Object The authors undertook a study to review the clinical features and outcome in patients who underwent surgery for intractable chronic epilepsy caused by temporal lobe tumors. Methods The Rush Surgical Epilepsy Database was queried to identify patients with chronic intractable epilepsy who underwent resection of temporal lobe tumors between 1981 and 2005 at Rush University Medical Center. Medical records were reviewed for age of the patient at seizure onset, delay to referral for surgery, seizure frequency and characteristics, preoperative MR imaging results, extent of resection, pathological diagnosis, complications, duration of follow-up period, and seizure improvement. Results Thirty-eight patients were identified, all with low-grade tumors. Gangliogliomas were the most common (36.8%), followed in descending order by dysembryoplastic neuroepithelial tumors (26.3%) and low-grade diffuse astrocytoma (10.5%). The mean duration between seizure onset and surgery was 15.4 years. Complex partial seizures were the most common presenting symptom. Detailed operative data were available for 28 patients; of these, 89.3% underwent complete resection of the amygdala, and 82.1% underwent partial or complete resection of hippocampus, in addition to lesionectomy. The mean follow-up duration was 7.7 years (range 1.0–23.1 years), with 78.9% of patients having seizure status that improved to Engel Class I, 15.8% to Engel Class II, and 5.3% to Engel Class III. Permanent complications were noted in 2.6% of patients. Conclusions The authors' examination of the long-term follow-up data in patients with temporal lobe tumors causing chronic intractable epilepsy demonstrated excellent results in seizure improvement after surgery.

Download Full-text

Optimizing Postoperative Surveillance of Pediatric Low-Grade Glioma Using Tumor Behavior Patterns

Neurosurgery ◽

10.1093/neuros/nyz072 ◽

2019 ◽

Vol 86 (2) ◽

pp. 288-297 ◽

Cited By ~ 3

Author(s):

Mohamed A Zaazoue ◽

Peter E Manley ◽

Mohammed Al Mehdar ◽

Nicole J Ullrich ◽

Hormuzdiyar H Dasenbrock ◽

...

Keyword(s):

Extent Of Resection ◽

Tumor Location ◽

Subtotal Resection ◽

Low Grade ◽

Total Resection ◽

Detection Rates ◽

Postoperative Surveillance ◽

Low Grade Gliomas ◽

Mri Scans

Abstract BACKGROUND Pediatric low-grade gliomas are among the most common childhood neoplasms, yet their post-treatment surveillance remains nonstandardized, relying on arbitrarily chosen imaging intervals. OBJECTIVE To optimize postoperative magnetic resonance imaging (MRI) surveillance protocols for pediatric low-grade gliomas. METHODS Patients aged 0 to 21 yr with pediatric low-grade gliomas, treated between 1990 and 2016 were retrospectively analyzed. The timing of surveillance imaging and radiologic tumor outcomes were extracted, and the effect of patient age, tumor location, histology, and extent of resection as prognostic factors was studied. An algorithm was developed to analyze the detection efficacy and cost of all possible surveillance protocols. RESULTS A total of 517 patients were included with a median follow-up of 7.7 yr (range: 2-25.1 yr) who underwent 8061 MRI scans (mean 15.6 scans per patient). Tumor recurrence was detected radiologically in 292 patients (56.5%), of whom, 143 underwent reoperation. The hazards ratio (HR) of recurrence was higher in patients who underwent biopsy (HR = 3.60; 95% confidence interval (CI): 2.45-5.30; P < .001), subtotal resection (HR = 2.97; 95% CI: 2.18-4.03; P < .001), and near-total resection (HR = 2.03; 95% CI: 1.16-3.54; P = .01), compared to patients with gross total resection (GTR). For all patients, an 8-image surveillance protocol at 0, 3, 6, 12, 24, 36, 60, and 72 mo (total cost: $13 672 per patient) yielded comparative detection rates to the current 15-image protocol ($25 635). For patients who underwent GTR, a 6-image protocol at 0, 3, 9, 24, 36, and 60 mo ($10 254) is sufficient. CONCLUSION Our data suggest that postoperative surveillance of pediatric low-grade gliomas can be effectively performed using less frequent imaging compared to current practice, thereby improving adherence to follow-up, and quality-of-life, while reducing costs.

Download Full-text

Profound hearing loss following surgery in pediatric patients with posterior fossa low-grade glioma

Neuro-Oncology Practice ◽

10.1093/nop/npx025 ◽

2017 ◽

Vol 5 (2) ◽

pp. 96-103 ◽

Cited By ~ 1

Author(s):

Yahya Ghazwani ◽

Ibrahim Qaddoumi ◽

Johnnie K Bass ◽

Shengjie Wu ◽

Jason Chiang ◽

...

Keyword(s):

Hearing Loss ◽

Posterior Fossa ◽

Extent Of Resection ◽

Tumor Location ◽

Low Grade Glioma ◽

Gross Total Resection ◽

Subtotal Resection ◽

Low Grade ◽

Total Resection ◽

Profound Hearing Loss

Abstract Background Hearing loss may occur in patients with posterior fossa low-grade glioma who undergo surgery. Methods We retrospectively reviewed 217 patients with posterior fossa low-grade glioma, including 115 for whom results of hearing tests performed after surgery and before chemotherapy or radiation therapy were available. We explored the association of UHL with age at diagnosis, sex, race, tumor location, extent of resection, posterior fossa syndrome, ventriculoperitoneal shunt placement, and histology. Results Of the 115 patients, 15 (13.0%: 11 male, 6 black, 8 white, 1 multiracial; median age 7 years [range, 1.3–17.2 years]) had profound UHL after surgery alone or before receiving ototoxic therapy. Median age at tumor diagnosis was 6.8 years (range, 0.7–14.1 years), and median age at surgery was 6.8 years (range, 0.7–14.1 years). Patients with UHL had pathology characteristic of pilocytic astrocytoma (n = 10), ganglioglioma (n = 4), or low-grade astrocytoma (n = 1). Of these 15 patients, 4 underwent biopsy, 1 underwent gross total resection, 1 underwent near-total resection, and 9 underwent subtotal resection. UHL was more frequent in black patients than in white patients (OR 7.3, P = .007) and less frequent in patients who underwent gross total resection or near-total resection than in those who underwent subtotal resection (OR 0.11, P = .02). Conclusions Children undergoing surgery for posterior fossa low-grade glioma are at risk for UHL, which may be related to race or extent of resection. These patients should receive postoperative audiologic testing, as earlier intervention may improve outcomes.

Download Full-text

P14.27 The significance of multicentric noncontrast-enhancing lesions distant from surgically resected glioblastoma: Case series of 3 patients

Neuro-Oncology ◽

10.1093/neuonc/noz126.262 ◽

2019 ◽

Vol 21 (Supplement_3) ◽

pp. iii72-iii73

Author(s):

J Hwang ◽

H An ◽

S Yoon ◽

K Park

Keyword(s):

Temporal Lobe ◽

Multidisciplinary Treatment ◽

Case Series ◽

Tumor Location ◽

Low Grade Glioma ◽

Low Grade ◽

Post Contrast ◽

Poor Prognostic Factor ◽

Prognostic Features ◽

18Fdg Pet

Abstract BACKGROUND Glioblastoma is the most malignant primary brain tumor. The tumor location and multiplicity plays an important role in surgical and further treatment. The incidence of multiple lesions at the time of diagnosis was known as 1–20%, which showed a poor prognostic factor. Most researches has focused on multiple contrast-enhancing lesions, however, multicentric non-enhancing lesions distant from glioblastoma has been rarely evaluated. The authors reported the case series of the patient who showed multicentric non contrast-enhancing lesions without connection to histologically-proven glioblastoma. MATERIAL AND METHODS Multicentric non contrast-enhancing lesions were defined as areas of FLAIR hyperintensity and mass effect without post-contrast enhancement, separated from the histologically-proven glioblastoma in a newly diagnosed disease. Three patients who showed distant non-enhancing lesions with appearance of a multicentric low-grade glioma were included in this study. The typical enhancing lesions were surgically resected and standard chemo-radiotherapy was followed in all patients. RESULTS All patients were male and their age was 38, 60 and 65 years old respectively. Multicentric tumor location was as follows: Case 1, left frontal lobe with non-enhancing lesion in left parahippocampal gyrus; Case 2, left parietal with non-enhancing lesion in left anteromedial temporal lobe; Case 3, left thalamus with non-enhancing lesions in both basal frontal and right temporal lobe. Pathologically, the resected enhancing tumor revealed glioblastoma in 2 patients and diffuse midline glioma in 1. All tumors were IDH-wild type. The resected enhanced lesion showed no progression but all non-enhancing lesions developed contrast-enhancing tumors at 3, 13 and 17 months after initial treatment, with high tracer uptake on 18FDG-PET or 18FDOPA-PET. Despite multidisciplinary treatment, two patients died from disease progression at 30 and 32 months after diagnosis and one patient is still alive with overall survival of 15 months. CONCLUSION The appearance of multicentric non-enhancing lesions distant from a typically enhancing tumor showed an uncommon finding in glioblastoma and poor prognostic features. These lesions progress faster than expected for a low-grade glioma. These lesions should be distinguished from typical low-grade glioma and should be considered more advanced lesions than their appearances suggest.

Download Full-text

JUVENILE INTRADURAL CHORDOMA

Neurosurgery ◽

10.1227/01.neu.0000316022.74162.00 ◽

2008 ◽

Vol 62 (2) ◽

pp. E525-E527 ◽

Cited By ~ 20

Author(s):

Steven W. Chang ◽

Pankaj A. Gore ◽

Peter Nakaji ◽

Harold L. Rekate

Keyword(s):

Clinical Presentation ◽

Radiation Treatment ◽

Complete Resection ◽

Magnetic Resonance Imaging Scan ◽

Resonance Imaging ◽

Total Resection ◽

Surgical Cure ◽

Neurologically Intact ◽

Resonance Imaging Scan

Abstract OBJECTIVE We report the youngest known case of a prepontine intradural chordoma. These tumors are exceedingly rare. Unlike their more common extradural counterparts, no recurrence of an intradural chordoma has been reported. CLINICAL PRESENTATION A 9-year-old boy underwent diagnostic imaging for evaluation of headaches. Although neurologically intact, a magnetic resonance imaging scan revealed a large prepontine mass with focal enhancement. INTERVENTION Endoscopic-assisted gross total resection was attained with staged bilateral retrosigmoid approaches. There were no additional adjuvant therapies. At the time of the 1-year follow-up evaluation, the patient had no recurrence. CONCLUSION By using an endoscopic-assisted procedure, we achieved complete resection of an intradural chordoma offering a potential for surgical cure. Resection is particularly advantageous because it spares the young child the need for radiation treatment. Close follow-up is warranted because we postulate that this tumor exists in a biological continuum between benign notochordal hamartomatous remnants and typical invasive chordomas.

Download Full-text

Gastrointestinal stromal tumors (GISTs) of the esophagus and gastroesophageal junction (GEJ).

Journal of Clinical Oncology ◽

10.1200/jco.2017.35.4_suppl.179 ◽

2017 ◽

Vol 35 (4_suppl) ◽

pp. 179-179

Author(s):

Andrew M. Briggler ◽

Rondell P Graham ◽

Gustavo Figueiredo Marcondes Westin ◽

Andrew Folpe ◽

Dawn E. Jaroszewski ◽

...

Keyword(s):

Median Time ◽

Mutational Analysis ◽

Gastroesophageal Junction ◽

Tumor Location ◽

Tnm Staging ◽

Low Grade ◽

Anastomotic Site ◽

Adjuvant Imatinib ◽

Kit Mutation

179 Background: GISTs arise from interstitial cells of Cajal anywhere within the gastrointestinal tract, but those of the esophagus and GEJ are exceedingly rare ( < 1% of cases). Our aim was to characterize clinicopathological features and outcomes of esophageal and GEJ GISTs and compare survival with a population-based registry (SEER). Methods: 28 cases were identified using the Mayo Clinic Cancer Registry from 1997 to 2016, and 378 cases from the SEER 18 registry from 2000 to 2013. Mayo cases were re-reviewed by Mayo pathologists. We analyzed patient characteristics, tumor location, TNM staging, mitotic index, molecular diagnostics, IHC staining, and histomorphology. JMP software was used to calculate time to recurrence (TTR) and overall survival (OS) utilizing Kaplan-Meier and log-rank. Results: At Mayo, 60% of tumors arose in the distal esophagus. Mean tumor size was 5.73 cm. 42% of cases were low grade. None had nodal involvement. Five cases were metastatic at diagnosis, all to the liver. 86.7% had spindle cell morphology. IHC staining was positive for KIT in 92%, followed by CD34 (52%), DOG-1 (24%), and actin (16%). Mutational analysis was done on 10 cases and KIT mutation was found in 8 cases; 2 were wild-type. Two-thirds of patients underwent surgery, mostly esophagectomy. 14 patients received adjuvant imatinib. Median time to diagnosis from symptom onset was 2 months; median time to surgery from diagnosis was 1 month. Of 10 patients with complete follow-up, 2 had recurrences: 1at the anastomotic site and 1 in the liver. Median TTR from surgery was 36.5 months. Median follow-up time was 31.5 months. Median OS from diagnosis for the Mayo cohort was 129.5 months (95% CI 55.7-x). SEER cohort median OS was 81 months (95% CI 63-101), and females had superior OS compared to males (HR 0.67; 95% CI 0.48-0.93, p = 0.016). Conclusions: Patients undergoing surgical resection for esophageal and GEJ GISTs had a favorable prognosis, but recurrences occurred. The superior OS seen in the Mayo cohort suggests early resection and adjuvant imatinib may improve outcomes.

Download Full-text

Control of temporal lobe epilepsy following en bloc resection of low-grade tumors

Journal of Neurosurgery ◽

10.3171/jns.1993.78.1.0019 ◽

1993 ◽

Vol 78 (1) ◽

pp. 19-25 ◽

Cited By ~ 142

Author(s):

Peter J. Kirkpatrick ◽

Minal Honavar ◽

Ivan Janota ◽

Charles E. Polkey

Keyword(s):

Temporal Lobe ◽

Age At Onset ◽

Tumor Resection ◽

Temporal Lobectomy ◽

Dysembryoplastic Neuroepithelial Tumor ◽

Low Grade ◽

En Bloc ◽

Content Type ◽

Incomplete Removal

✓ Thirty-one patients with a mean age of 18.9 years (range 3 to 53 years) who underwent temporal lobe surgery for tumor-related epilepsy over a 14-year period are presented. All had suffered chronic drug-resistant temporal lobe seizures (mean age at onset 6.9 years, range 0 to 30 years; mean duration of condition 11.9 years, range 3 to 39 years). Preoperative interictal scalp electroencephalography tracings indicated unilateral localized epileptic foci in 90% of patients, and computerized tomography scans showed abnormalities within the temporal lobe in 87%. All patients underwent en bloc temporal lobectomy. No patient received adjuvant radiotherapy or chemotherapy. Review of the histological material showed dysembryoplastic neuroepithelial tumor in 27 (87%) of the specimens and microscopic evidence of incomplete removal of tumor in 22 (71%). At long-term follow-up evaluation (mean duration 5.8 years, range 1 to 14 years), 81% of patients were completely free of seizures (Engel grade I) and 10% were almost seizure free (Engel grade II) with no deaths reported in either early or late follow-up review. Only one patient in the series failed to benefit from the surgery. Four patients suffered permanent neurological deficit causing a mild disability. Psychological assessment showed no significant fall in verbal or performance intelligent quotient for the group, but a mild memory impairment was evident in 32%. Behavioral and social aspects improved in nearly all (94%) cases. Relief of seizures could not be predicted by intraoperative electrocorticography, and outcome was independent of the completeness of tumor resection. Postoperative electroencephalographic findings identified epileptiform potentials in 65% of patients, which were associated with a worse seizure-control outcome grade.

Download Full-text

Cervical meningioma resection including the inner dura through an open-door laminoplasty using hydroxyapatite spacers: A case report

Surgical Neurology International ◽

10.25259/sni_409_2020 ◽

2020 ◽

Vol 11 ◽

pp. 317

Author(s):

Naohisa Miyakoshi ◽

Michio Hongo ◽

Yuji Kasukawa ◽

Yoshinori Ishikawa ◽

Daisuke Kudo ◽

...

Keyword(s):

Tumor Resection ◽

Complete Resection ◽

Csf Leak ◽

Open Door ◽

Spinal Cord Tumors ◽

Total Resection ◽

Watertight Closure ◽

Intradural Extramedullary ◽

The Ideal

Background: The ideal surgery for spinal cord tumors is complete resection to prevent recurrence. However, it should be accomplished safely/effectively without risking increased morbidity. Here, we report a cervical meningioma that was totally resected, including the inner dura, through a laminoplasty performed with hydroxyapatite (HA) spacers. Case Description: A 61-year-old Asian male presented with a symptomatic intradural extramedullary C4-C6 cervical meningioma. At surgery, this required resection of the inner dural layer through an open-door laminoplasty. Preservation of the outer dural layer facilitated a watertight closure and the avoidance of a postoperative cerebrospinal fluid (CSF) fistula. Notably, the laminoplasty utilized HA spacers which were magnetic resonance (MR) compatible allowing for future follow-up studies to evaluate for tumor recurrence. At 5-year follow-up, the tumor had not recurred, the patient was asymptomatic, and alignment was maintained. Conclusion: Gross total resection of an intradural extramedullary C4-C6 cervical meningioma was performed with removal of just the inner dural layer. Preservation of the outer dural layer allowed for a watertight closure and the avoidance of a postoperative CSF leak. Further, laminoplasty using HA spacers allowed for successful tumor resection, adequate fusion/stabilization, while not interfering with future MR studies (e.g., HA MR compatible).

Download Full-text

Epidemiological Study of Adamantinoma from US Surveillance, Epidemiology, and End Results Program: III Retrospective Analysis

Journal of Oncology ◽

10.1155/2020/2809647 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Mahmut Nedim Aytekin ◽

Recep Öztürk ◽

Kamil Amer

Keyword(s):

Marital Status ◽

Survival Time ◽

Survival Rates ◽

Age Groups ◽

Tumor Location ◽

Cancer Surveillance ◽

Survival Duration ◽

Low Grade ◽

The Mean

Objective. Adamantinomas are rare low-grade malignant bone tumors. This study aims to describe the demographic characteristics and survival rates of patients suffering from adamantinomas. Methods. The National Institute of Cancer Surveillance, Epidemiology, and Recent Results (SEER) database was used, and patients diagnosed with adamantinoma between 1973 and 2016 were screened. Patients were classified according to sex, age, race/ethnicity, and marital status, and also tumors were classified according to year of diagnosis, laterality, type of treatment, and follow-up. Results. The mean age of patients was 30.8 ± 16.7 (range: 4–75). A total of 92 patients were identified; of these, 43 were females and 49 were males. The mean follow-up period was 138.1 ± 90.3 (range: 1–156) months. Mean survival duration was 287.8 ± 15.4 (95% CI: 257.7–317.9) months. Five- and ten-year survival rates were 98.8% and 91.5%, respectively. Besides, survival time was also observed to be independent of gender, age groups, race, marital status, tumor location, and year of diagnosis. Conclusion. Adamantinoma is a very rare bone tumor that affects the long bones in lower extremities and is more common in men. Five- and 10-year survival prognoses are reasonably satisfactory. Also, survival time is independent of variables such as gender, age, and tumor location.

Download Full-text

Microsurgical resection of brainstem cervicomedullary ganglioglioma: operative video and technique of creating a surgical pseudoplane for near-total resection

Neurosurgical Focus: Video ◽

10.3171/2019.10.focusvid.19413 ◽

2019 ◽

Vol 1 (2) ◽

pp. V13

Author(s):

James K. Liu ◽

Vincent N. Dodson

Keyword(s):

Neural Tissue ◽

Residual Tumor ◽

Complete Resection ◽

Neurological Function ◽

Neurological Injury ◽

Low Grade ◽

Total Resection ◽

Sharp Dissection ◽

Microsurgical Resection ◽

Eloquent Location

Cervicomedullary gangliogliomas are rare low-grade neoplasms of the brainstem. They can be challenging lesions to resect due to the eloquent location in the brainstem. In some instances, the absence of a clear surgical plane between the tumor and normal neural tissue can prohibit a complete resection. Therefore, it is important to leave a thin rim of residual tumor at the tumor-brainstem interface in order to avoid irreversible neurological injury. In this operative video, the authors demonstrate the technique to develop a surgical pseudoplane using sharp microdissection for a cervicomedullary brainstem ganglioglioma without a clear interface between the tumor and normal neural tissue. This strategy allowed for radical near-total resection of the tumor, thereby maximizing the extent of removal while preserving neurological function. Postoperatively, the patient had normal neurological function and returned to work without any disability. In summary, due to the lack of a clear surgical dissection plane, a pseudoplane near the surgical interface can be performed using sharp dissection to facilitate radical near-total resection.The video can be found here: https://youtu.be/biD4G1Hh0yk.

Download Full-text