The evolution of eloquent located low-grade gliomas surgical approaches, their natural history and molecular classification

Low-grade glioma is characterized by slow growth, infiltrative pattern through white matter tracts and progression to a malignant tumour type. The traditional classification is newly replaced by molecular stratification. This reorganisation gathers glioma with similar prognosis and treatment protocols. The preferential location of that tumour in eloquent areas constituted, over time, a real challenge regarding the best surgical approach. Because of the high risk of postoperative neurological deficits initially a more conservative management was adopted. Once with the development of preoperative and intraoperative functional assessment techniques, a higher degree of resection was possible in the limits of cortico-subcortical eloquence, being well known that this is a statistically significant factor for survival. We present in this paper the natural evolution of low-grade glioma, their new molecular classification, prognostic factors and the various approach proposed for eloquent ones.

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Doubling Recruitment of Pediatric Low-grade Glioma within Two Decades does not change Outcome – Report from the German LGG Studies

Klinische Pädiatrie ◽

10.1055/a-1471-5897 ◽

2021 ◽

Vol 233 (03) ◽

pp. 107-122

Author(s):

Astrid Katharina Gnekow ◽

Daniela Kandels ◽

Torsten Pietsch ◽

Brigitte Bison ◽

Monika Warmuth-Metz ◽

...

Keyword(s):

Standard Of Care ◽

Incidence Rates ◽

Observation Time ◽

Low Grade Glioma ◽

Low Grade ◽

Similar Distribution ◽

Multicenter Studies ◽

Histologic Diagnosis ◽

Treatment Protocols ◽

Therapy Algorithm

Abstract Background Successive multicenter studies for pediatric low-grade glioma (LGG) in Germany were accompanied by a doubling of annual recruitment over 2 decades. We investigated whether this increase conveyed a change of epidemiologic characteristics or survival. Methods and results Participating centers reported 4634 patients with the radiologic/histologic diagnosis of LGG (1996–2018), rising from 109 to 278/year. Relating these numbers to all pediatric CNS tumors registered at the German Childhood Cancer Registry, the LGG fraction and annual crude incidence rates increased (32% to 51%; 0.94 to 2.12/100,000 children/adolescents<15 years). The consecutive LGG studies recruited 899 (HIT-LGG 1996), 1592 (SIOP-LGG 2004), and 1836 (LGG-registry) patients with similar distribution of tumor-sites, histology, and dissemination. 5-year overall survival was 96%-98% at median observation time of 8.1 years. Acknowledging unequal follow-up periods, 589/899 (66%), 1089/1582 (69%), and 1387/1836 (76%) patients remained under observation, while 1252/4317 received adjuvant treatment with decreasing frequency of front-line radiotherapy from 16% to 5%. Conclusion Pediatric LGG incidence rates in Germany are now comparable to other European countries. The rise in patient numbers followed implementation of standard-of-care treatment protocols, but did not result in relevant changes of epidemiologic or clinical parameters or survival. Shifts in patient distribution between treatment arms reflect growing acceptance of the LGG therapy algorithm.

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Left Pan-Hippocampal Low Grade Glioma—2-Stage Transsylvian Transventricular and Paramedian Supracerebellar Transtentorial Approaches: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opy214 ◽

2018 ◽

Vol 16 (3) ◽

pp. E82-E82

Author(s):

Juan C Fernandez-Miranda

Keyword(s):

Tumor Resection ◽

Spatial Relationship ◽

Low Grade Glioma ◽

Parahippocampal Gyrus ◽

Surgical Approaches ◽

Low Grade ◽

Fiber Tractography ◽

High Definition ◽

Posterior Portion ◽

Fiber Tracts

Abstract The surgical goal for low-grade gliomas (LGGs) is to maximize resection while minimizing morbidity. Pan-hippocampal LGGs extend from the hippocampal head to the hippocampal tail, and involve the parahippocampal gyrus and uncus. Given their anteroposterior extension, they cannot be completely removed with 1 single approach, requiring a 2-stage front-to-back operation. In this video, we present the case of a 52-yr-old man with new onset of generalized seizures and a dominant-side, nonenhancing, pan-hippocampal infiltrative lesion compatible with a low-grade glioma. Preoperative high-definition fiber tractography (HDFT) showed the spatial relationship of the tumor with surrounding fiber tracts, such as the arcuate, inferior fronto-occipital, and middle longitudinal fascicles, and optic radiations. Surgical resection was planned in 2 separate stages. The first stage consisted of a transsylvian transinferior insular sulcus approach to the extra- and intraventricular aspects of the uncohippocampal region. The entire anterior and middle portions of the tumor were successfully removed with minimal morbidity, including transient naming difficulties and permanent superior quadrantanopia. Postoperative HDFT showed preservation of all fiber tracts, except for a portion of Meyer's loop and the inferior-most aspect of the inferior fronto-occipital fascicle. The second stage was completed 8 wk later and consisted of a paramedian supracerebellar-transtentorial approach on sitting position. The posterior portion of the tumor was entirely removed to achieve a complete macroscopic tumor resection. The final diagnosis was IDH1-positive LGG. Pan-hippocampal tumors remain a surgical challenge but accurate knowledge of surgical neuroanatomy and surgical approaches facilitates their safe and effective treatment. The patient signed an informed consent including the use of photographic and video material for educational or academic purposes.

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Surgery for low-grade glioma infiltrating the central cerebral region: location as a predictive factor for neurological deficit, epileptological outcome, and quality of life

Journal of Neurosurgery ◽

10.3171/2013.5.jns122235 ◽

2013 ◽

Vol 119 (2) ◽

pp. 318-323 ◽

Cited By ~ 37

Author(s):

Philippe Schucht ◽

Fadi Ghareeb ◽

Hugues Duffau

Keyword(s):

Quality Of Life ◽

Return To Work ◽

Seizure Control ◽

Extent Of Resection ◽

Low Grade Glioma ◽

Neurological Deficits ◽

Main Concern ◽

Low Grade ◽

Who Grade

Object A main concern with regard to surgery for low-grade glioma (LGG, WHO Grade II) is maintenance of the patient's functional integrity. This concern is particularly relevant for gliomas in the central region, where damage can have grave repercussions. The authors evaluated postsurgical outcomes with regard to neurological deficits, seizures, and quality of life. Methods Outcomes were compared for 33 patients with central LGG (central cohort) and a control cohort of 31 patients with frontal LGG (frontal cohort), all of whom had had medically intractable seizures before undergoing surgery with mapping while awake. All surgeries were performed in the period from February 2007 through April 2010 at the same institution. Results For the central cohort, the median extent of resection was 92% (range 80%–97%), and for the frontal cohort, the median extent of resection was 93% (range 83%–98%; p = 1.0). Although the rate of mild neurological deficits was similar for both groups, seizure freedom (Engel Class I) was achieved for only 4 (12.1%) of 33 patients in the central cohort compared with 26 (83.9%) of 31 patients in the frontal cohort (p < 0.0001). The rate of return to work was lower for patients in the central cohort (4 [12.1%] of 33) than for the patients in the frontal cohort (28 [90.3%] of 31; p < 0.0001). Conclusions Resection of central LGG is feasible and safe when appropriate intraoperative mapping is used. However, seizure control for these patients remains poor, a finding that contrasts markedly with seizure control for patients in the frontal cohort and with that reported in the literature. For patients with central LGG, poor seizure control ultimately determines quality of life because most will not be able to return to work.

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PEDT-02 DIAGNOSIS, TREATMENT AND CLINICAL OUTCOME OF ATYPICAL BRAINSTEM TUMOUR IN CHILDHOOD

Neuro-Oncology Advances ◽

10.1093/noajnl/vdz039.071 ◽

2019 ◽

Vol 1 (Supplement_2) ◽

pp. ii16-ii16

Author(s):

Takaaki Yanagisawa ◽

Takaya Honda ◽

Masatada Yamaoka ◽

Masaharu Akiyama ◽

Kohei Fukuoka ◽

...

Keyword(s):

Clinical Outcome ◽

Survival Data ◽

Treatment Strategy ◽

Retrospective Chart Review ◽

Malignant Tumour ◽

Diffuse Intrinsic Pontine Glioma ◽

Clinical Findings ◽

Low Grade Glioma ◽

Pathological Diagnosis ◽

Low Grade

Abstract BACKGROUND Brainstem tumours account for 10–15% of brain tumors in childhood. Diffuse intrinsic pontine glioma (DIPG) accounts for 60–80% of them and are diagnosed based on clinical findings and radiologic features. All the rest of tumours excluding DIPG are very rare, heterogeneous group of tumours including low-grade glioma and malignant embryonal tumors. It is often difficult to diagnose and decide treatment strategy for their rarity. METHODS To present our experience with atypical brainstem tumours, a retrospective chart review was conducted to identify eligible cases treated over a ten-year period. All tumors involving brainstem, felt not to be DIPGs for absence of clinical/neuroimaging features were included. Demographic information, pathological findings, neuroimaging characteristics, surgical and nonsurgical management plans, and survival data were collected for analysis. RESULTS Between April 2007 and March 2017, 16 patients (14 initial and 2 recurrent) aged from 3 to 20 years were identified. 14 of them were symptomatic and 4 of them were asymptomatic at reference. Of 10 symptomatic cases, 10 were biopsied and pathological diagnosis was low-grade glioma in 8, glioblastoma in 2 cases. They had treatment depending on the pathological diagnosis. Of 4 asymptomatic cases, one with small focal tumour, with no findings suggesting malignant tumour with 11C-methioninePET or MRS, progressed to show typical clinical and image findings of DIPG in a year. For other three, they remain asymptomatic without progression with no treatment for 25months, 60months, and 65 months respectively. Malignant transformation was observed in one with biopsy-conformed oligoastrocytoma with no K27M-H3 mutations treated with chemotherapy and another with pilocytic astrocytoma treated with chemotherapy and radiotherapy. CONCLUSIONS Though molecular findings such as K27M-H3 mutations can predict clinical outcome in some cases, it still remains difficult to diagnose and find treatment strategy of atypical brainstem tumours. The need and usefulness of nationwide registry study is warranted.

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IDH/MGMT-driven molecular classification of low-grade glioma is a strong predictor for long-term survival

Neuro-Oncology ◽

10.1093/neuonc/nos317 ◽

2013 ◽

Vol 15 (4) ◽

pp. 469-479 ◽

Cited By ~ 109

Author(s):

S. Leu ◽

S. von Felten ◽

S. Frank ◽

E. Vassella ◽

I. Vajtai ◽

...

Keyword(s):

Strong Predictor ◽

Molecular Classification ◽

Low Grade Glioma ◽

Low Grade ◽

Term Survival ◽

Long Term Survival

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Paediatric Embryonal Tumours in Multilayered Rosettes Presenting as a Low Grade Glioma- An Unusual Case Report

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2020/45038.14089 ◽

2020 ◽

Author(s):

Sibhi Ganapathy ◽

Nikunj Godhani

Keyword(s):

Case Report ◽

Low Grade Glioma ◽

World Health ◽

Neurological Deficits ◽

Histopathological Analysis ◽

Low Grade ◽

Low Grade Gliomas ◽

Rare Differential Diagnosis ◽

Embryonal Tumours ◽

The Brain

Paediatric Embryonal Tumours in Multilayered Rosettes (ETMR) are rare aggressive tumours with poor survival statistics, defined in 2016 by World Health Organisation (WHO) classification of brain tumours. The tumours have a characteristic radiological appearance on Magnetic Resonance Imaging (MRI) of the brain, which is easily decipherable. This combined with a clinical picture of raised intracranial pressure symptoms, seizures and rapidly progressive new onset neurological deficits make the diagnosis fairly obvious. The final confirmation of the diagnosis is done by immunohistochemical analysis of the C19Myc gene alteration. Rarely, certain radiological presentations are uncharacteristic and resemble other more benign pathologies with overlapping clinical presentations. This can be misleading, as ETMRs require aggressive surgery followed by adjuvant chemotherapy and radiation to ensure best possible survival. We present such a case report of what appeared to be a low-grade glioma in the frontal lobe. This tumour presented with one episode of generalised tonic clonic seizures not unusual as a presenting complaint in low-grade gliomas per se. Surgical debulking under ultrasonic guidance was done and the specimen was sent to histopathological analysis. The histopathological analysis showed a surprise ETMR diagnosis which was sent for confirmation to two other centers. This case report highlights the need to keep ETMRs as a rare differential diagnosis for even low-grade gliomas of the brain, thereby allowing accurate prognostication only after histopathological and immunohistochemical assessment. We present a brief literature review on unusual presentations of ETMRs reported in literature to further illustrate the chimeric nature of this rare disease.

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Neurocognitive Function in Newly Diagnosed Low-grade Glioma Patients Undergoing Surgical Resection With Awake Mapping Techniques

Neurosurgery ◽

10.1227/neu.0000000000000779 ◽

2015 ◽

Vol 77 (3) ◽

pp. 371-379 ◽

Cited By ~ 36

Author(s):

Caroline A. Racine ◽

Jing Li ◽

Annette M. Molinaro ◽

Nicholas Butowski ◽

Mitchel S. Berger

Keyword(s):

Surgical Resection ◽

Cognitive Deficits ◽

Neuropsychological Testing ◽

Case Series ◽

Low Grade Glioma ◽

Neurological Deficits ◽

Low Grade ◽

Newly Diagnosed ◽

Cognitive Screening ◽

Mapping Techniques

Abstract BACKGROUND: Low-grade glioma (LGG) patients have increased life expectancy, so interest is high in the treatments that maximize cognition and quality of life. OBJECTIVE: To examine presurgical baseline cognitive deficits in a case series of LGG patients and determine cognitive effects of surgical resection with awake mapping. METHODS: We retrospectively assessed neurological deficits, subjective concerns from patient or caregiver, and cognitive deficits at baseline and postsurgery for 22 patients with newly diagnosed LGG who underwent baseline neuropsychological evaluation and magnetic resonance imaging before awake surgical resection with mapping. Twelve of the 22 patients returned for postoperative evaluation approximately 7 months after surgery. RESULTS: At baseline, 92% of patients/caregivers reported changes in cognition or mood. Neurological examinations and Montreal Cognitive Assessment Scale scores were largely normal; however, on many tests of memory and language, nearly half of individuals showed deficits. After surgery, 45% had no deficits on neurological examination, whereas 55% had only transient or mild difficulties. Follow-up neuropsychological testing found most performances stable to improved, particularly in language, although some patients showed declines on memory tasks. CONCLUSION: Most LGG patients in this series presented with normal neurological examinations and cognitive screening, but showed subjective cognitive and mood concerns and cognitive decline on neuropsychological testing, suggesting the importance of comprehensive evaluation. After awake mapping, language tended to be preserved, but memory demonstrated decline in some patients. These results highlight the importance of establishing a cognitive baseline before surgical resection and further suggest that awake mapping techniques provide reasonable language outcomes in individuals with LGG in eloquent regions.

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