Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database

Terrier, Louis-Marie; Bauchet, Luc; Rigau, Valérie; Amelot, Aymeric; Zouaoui, Sonia; Filipiak, Isabelle; Caille, Agnès; Almairac, Fabien; Aubriot-Lorton, Marie-Hélène; Bergemer-Fouquet, Anne-Marie; Bord, E.; Cornu, Philippe; Czorny, A.; Hieu, P. Dam; Debono, Bertrand; Delisle, Marie–Bernadette; Emery, Évelyne; Farah, Walid; Gauchotte, Guillaume; Godfraind, Catherine; Guyotat, Jacques; Irthum, B.; Janot, Kévin; Reste, Pierre-Jean Le; Liguoro, Dominique; Loiseau, Hugues; Lot, Guillaume; Lubrano, V.; Mandonnet, Emmanuel; Menei, Philippe; Métellus, Philippe; Milin, Serge; Muckenstrum, Bertrand; Roche, Pièrre-Hugues; Rousseau, Audrey; Uro‐Coste, Emmanuelle; Vital, Anne; Voirin, Jimmy; Wager, Michel; Zanello, Marc; François, P.; Velut, Stéphane; Varlet, Pascale; Figarella‐Branger, Dominique; Pallud, Johan; Zemmoura, Ilyess

doi:10.1093/neuonc/now186

Cited by 27 publications

(37 citation statements)

References 39 publications

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“…These data suggest that the BRAF V600E mutation may influence long-term survival if associated with the H3.3 K27M mutation. Anaplastic GGs (grade III), a controversial and poorly characterized entity with a poor prognosis (24,40,45), can arise de novo or secondary to the malignant transformation of WHO grade I GG, but the rate of malignant transformation is unclear, with reported values of 0.6% to 14.5% depending on the study (25,26,44). Three patients in our series presented secondary malignant transformation, whatever the mutational status.…”

Section: Discussionmentioning

confidence: 75%

Co‐occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma

Pagès

Beccaria²,

Boddaert³

et al. 2017

Brain Pathology

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Ganglioglioma (GG) is a grade I tumor characterized by alterations in the MAPK pathway, including BRAF V600E mutation. Recently, diffuse midline glioma with an H3 K27M mutation was added to the WHO 2016 classification as a new grade IV entity. As cooccurrence of H3 K27M and BRAF V600E mutations has been reported in midline tumors and anaplastic GG, we searched for BRAF V600E and H3 K27M mutations in a series of 54 paediatric midline grade I GG (midline GG) to determine the frequency of double mutations and its relevance for prognosis. Twenty-seven patients (50%) possessed the BRAF V600E mutation. The frequency of the co-occurrence of H3F3A/BRAF mutations at diagnosis was 9.3%. No H3 K27M mutation was detected in the absence of the BRAF V600E mutation. Double-immunostaining revealed that BRAF V600E and H3 K27M mutant proteins were present in both the glial and neuronal components. Immunopositivity for the BRAF V600E mutant protein correlated with BRAF mutation status as detected by massARRAY or digital droplet PCR. The median follow-up of patients with double mutation was 4 years. One patient died of progressive disease 8 years after diagnosis, whereas the four other patients were all alive with stable disease at the last clinical follow-up (at 9 months, 1 year and 7 years) without adjuvant therapy. We demonstrate in this first series of midline GGs that the H3 K27M mutation can occur in association with the BRAF V600E mutation in grade I glioneuronal tumors. Despite the presence of H3 K27M mutations, these cases should not be graded and treated as grade IV tumors because they have a better spontaneous outcome than classic diffuse midline H3 K27M-mutant glioma. These data suggest that H3 K27M cannot be considered a specific hallmark of grade IV diffuse gliomas and highlight the importance of integrated histomolecular diagnosis in paediatric brain tumors.

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Section: Discussionmentioning

confidence: 75%

Co‐occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma

Pagès

Beccaria²,

Boddaert³

et al. 2017

Brain Pathology

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“…Gross total resection should be attempted whenever possible with the preservation of neurological function. Some studies have advocated the role of gross total resection along with adjuvant radiochemotherapy in achieving good survival rates [20,21].…”

Section: Discussionmentioning

confidence: 99%

A case report of rare location of ganglioglioma

et al. 2019

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Background: Gangliogliomas are rare tumors of the central nervous system. They can occur anywhere in the central nervous system but are most commonly located in the temporal lobe and are mainly found in children. Anaplastic ganglioglioma can result from either de novo or transformation of a pre-existing lesion. Case presentation: We report a case of de novo anaplastic ganglioglioma in the parieto occipital region, which is a rare location. A 34-year-old lady presented with features of raised intracranial pressure (ICP) with right side hemiparesis. Contrast-enhanced magnetic resonance imaging (CEMRI) of the brain showed welldefined intense heterogenously enhancing solid cystic mass lesion 5.3 × 5.2 cm in the left parieto occipital region with mass effect and midline shift. Intraoperatively, a cystic mass lesion with reddish brown nodule was seen in the left occipital lobe. Complete tumor excision was done. Microscopic and IHC examination was suggestive of anaplastic ganglioglioma. The post-operative period was uneventful. The patient received 60-Gy radiotherapy with temozolamide as adjuvant therapy, and repeat imaging showed no tumor recurrence. Conclusion: Anaplastic gangliogliomas are rare tumors with parieto occipital as rare location.

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“…[ 5 11 21 ] Their incidence is estimated at approximately 0.02 cases per million people per year. [ 19 23 ]…”

Section: Discussionmentioning

confidence: 99%

“…Prognostic factors of poor survival are older age at diagnosis, male sex, and malignant glial features. [ 23 ]…”

Section: Discussionmentioning

confidence: 99%

A case of adult anaplastic cerebellar ganglioglioma

Bouali¹,

Maatar²,

Zehani³

et al. 2018

Surg Neurol Int

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Background:Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity.Case Description:A 40-year-old man presented with a history of headaches and dizziness and progressive gait disturbance and was diagnosed with anaplastic ganglioglioma in the posterior fossa.Conclusions:Although rare, our case demonstrates that anaplastic ganglioglioma should be considered in the differential diagnosis of infratentorial tumors in adult patients.

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Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database

Cited by 27 publications

References 39 publications

Co‐occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma

Co‐occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma

A case report of rare location of ganglioglioma

A case of adult anaplastic cerebellar ganglioglioma

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