Anaplastic Ganglioglioma: A Very Rare Intramedullary Spinal Cord Tumor

Schneider, C.; Vosbeck, Jürg; Grotzer, Michael A.; Boltshauser, Eugen; Kothbauer, Karl F.

doi:10.1159/000339851

Cited by 11 publications

(3 citation statements)

References 31 publications

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“…This high proportion of patients with adjuvant treatment is amplified by the occurrence of 2 cases of a particularly rare tumor type, the anaplastic ganglioglioma in the highgrade group. 23 The occurrence of these rare tumors in our cohort may be due to a referral bias of complex cases to the senior author. The role of adjuvant radiotherapy is debated.…”

Section: Discussionmentioning

confidence: 88%

Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children

Schneider¹,

Hidalgo²,

Schmitt‐Mechelke

et al. 2014

PED

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Object Presently, the best available treatment for intramedullary spinal cord tumors (IMSCTs) in children is microsurgery with the objective of maximal tumor removal and minimal neurological morbidity. The latter has become manageable with the development and standard use of intraoperative neurophysiological monitoring. Traditionally, the perioperative neurological evaluation is based on surgical or spinal cord injury scores focusing on sensorimotor function. Little is known about the quality of life after such operations; therefore, this study was designed to investigate the impact of surgery for IMSCTs on the quality of life in children. Methods Twelve consecutive pediatric patients treated for IMSCT were included in this retrospective fixed cohort study. A multidimensional questionnaire-based quality of life instrument, the Pediatric Quality of Life Questionnaire version 4 (PedsQL 4.0), was chosen to analyze follow-up data. This validated instrument particularly allows for a comparison between a patient cohort and a healthy pediatric sample population. Results Of 11 mailed questionnaires (1 patient had died of progressive disease), 10 were returned, resulting in a response rate of 91%. There were 8 low-grade lesions (5 pilocytic astrocytomas, 1 ganglioglioma, 1 hemangioblastoma, and 1 cavernoma) and 4 high-grade lesions (2 anaplastic gangliogliomas, 1 glioblastoma, and 1 glioneuronal tumor). The mean age at diagnosis was 7.5 years, the mean follow-up was 4.2 years, and 83% of the patients were male. Total resection was achieved in 5 patients and subtotal resection in 7. Four patients had undergone 2 or more resections. The 4 patients with high-grade tumors and 2 with incompletely resected low-grade tumors underwent adjuvant treatment (2 chemotherapy and 4 both radiotherapy and chemotherapy). The mean modified McCormick Scale score at the time of diagnosis was 1.7; at the time of follow-up, 1.5. The mean PedsQL 4.0 total score in the low-grade group was 78.5; in the high-grade group, 82.6. There was no significant difference in PedsQL 4.0 scores between the patient cohort and the normal population. Conclusions In a small cohort of children who had undergone surgery for IMSCTs with a mean follow-up of 4.2 years, quality of life scores according to the PedsQL 4.0 instrument were not different from those in a normal sample population.

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Section: Discussionmentioning

confidence: 88%

Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children

Schneider¹,

Hidalgo²,

Schmitt‐Mechelke

et al. 2014

PED

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“…Patients are generally children or young adults at presentation [9–13], Caucasian [13], and more likely to be male, as there is slight gender predominance [13]. AGGs are most commonly unifocal, supratentorial in location, and located in the temporal lobes [13], but cases have also been reported within the spinal cord [14], as well as in intraventricular locations [15]. The present case series is a retrospective review of three unique cases of AGG treated at the Comprehensive Cancer Center of Wake Forest University (CCCWFU) between January 2011 and May 2014 (Supplementary Fig.…”

Section: Introductionmentioning

confidence: 99%

Anaplastic ganglioglioma: a report of three cases and review of the literature

et al. 2015

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Gangliogliomas are rare tumors of the central nervous system that are thought to arise from a glioneuronal precursor and consist of both neuronal and glial elements. Grade III, or anaplastic ganglioglioma (AGG), most commonly affects children and young adults, generally arises in a supratentorial location, is highly epileptogenic, and often results in diffuse local and distant failure within the craniospinal axis. Pathologically, these tumors are graded by the degree of malignancy in their glial portion and radiologic diagnosis is difficult due to the wide variation in its degree of solid and cystic components, contrast uptake, and calcification patterns. This report presents three cases of AGG, with initial treatment including subtotal resection followed by conformal radio-therapy. In the case where the AGG developed in the setting of an existent low-grade astrocytoma, the patient received no chemotherapy. Both of the other de novo cases were managed with adjuvant chemoradiotherapy with temozolomide. Recurrence occurred at 6, 16, and 20 months following therapy. Two of the three patients experienced symptomatic decline at recurrence, but experienced Karnofsky performance status (KPS) improvement after salvage therapy, including the reduction of cranial neuropathy and balance. All patients had a significant reduction in presenting symptoms following salvage therapy. Patients died at 23, 20, and 22 months following initial surgical management, respectively. A review of anaplastic and malignant gangliogliomas is presented in the context of these three cases.

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“…2,3 Primary spinal cord GGs usually follow a benign clinical course, with a 5-year progression-free survival rate of 67%, although aggressive behavior has been reported. 4–10 Transformation to a higher grade tumor may occur more frequently in adults. 11–13 Intracerebral, leptomeningeal, and intraventricular spread from primary spinal cord GG is exceedingly rare.…”

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Massive Dissemination From Spinal Cord Gangliogliomas Negative for BRAF V600E

Lummus

Aisner

Sams

et al. 2014

American Journal of Clinical Pathology

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Objectives Metastatic leptomeningeal spread from spinal cord gangliogliomas (GGs) is exceedingly rare. Methods Two adult women, aged 27 and 51 years, died of massive disseminations of cervicothoracic GGs 4 and 6 years, respectively, after initial diagnoses; full autopsies were performed. BRAF status was assessed by VE1 immunohistochemistry (IHC), Sanger sequencing, and single-nucleotide base extension assay (SNaPshot). Results The 27-year-old underwent two biopsies, chemotherapy, radiation, and ventriculoperitoneal shunt placement; she developed craniospinal and peritoneal dissemination. Autopsy confirmed shunt-mediated peritoneal metastases, microscopic bone marrow involvement, and profuse spinal and supratentorial leptomeningeal and parenchymal spread. The 51-year-old underwent two resections, radiation, and chemotherapy and developed pancytopenia with biopsy-proven bony metastases 15 months before death. Autopsy demonstrated leptomeningeal, subpial, and subependymal metastases. The tumors in both primary and metastatic sites were BRAF negative by VE1 IHC and two different mutational analyses. This compared with negative BRAF results for an additional four nonmetastatic adult nonsupratentorial GGs and in our study. Conclusions We document two rare cases of massively metastatic spinal cord GGs in adult patients who were negative for BRAF V600E mutations via multiple methods.

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Anaplastic Ganglioglioma: A Very Rare Intramedullary Spinal Cord Tumor

Cited by 11 publications

References 31 publications

Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children

Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children

Anaplastic ganglioglioma: a report of three cases and review of the literature

Massive Dissemination From Spinal Cord Gangliogliomas Negative for BRAF V600E

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