Pediatric Primary Anaplastic Ganglioglioma: A Case Report and Review of the Literature

Scoccianti, Silvia; Agresti, Benedetta; Detti, Beatrice; Cipressi, Samantha; Franceschini, Davide; Greto, Daniela; Mussa, Federico; Sardi, Iacopo; Buccoliero, Annamaria; Genitori, Lorenzo; Biti, Giampaolo

doi:10.1159/000340067

Cited by 15 publications

(6 citation statements)

References 52 publications

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“…In Scoccianti et al review of 19 pediatric AGGs, 10 (53 %) patients experienced tumor progression, and of these, five progressed distantly [34, 35]. In our series, all patients experienced out-of-field, leptomeningeal failures.…”

Section: Discussionmentioning

confidence: 50%

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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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Section: Discussionmentioning

confidence: 50%

“…These findings suggest that chemotherapy may be warranted as an upfront treatment modality to minimize the risk of neuraxis failure. In spite of these distant failures, most authors continue to recommend conformal, partial brain irradiation to minimize the morbidity of extended fields [30, 34]. …”

Section: Discussionmentioning

confidence: 99%

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

et al. 2015

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“…Their 5 year event free survival (EFS) was 63±17% and overall survival was 88±12%. In a case serial by Lucas et al 17 3 patients with AGGs experienced leptomeningeal failures, also in Scieocianti et al's 18 review of 19 pediatric AGGs, 10 (53%) patients experienced tumor progression and of these, five progressed distantly. In the data of SEER 11 which is the largest series of the literature, adjuvant chemotherapy was not examined as a survival factor because of the limited information about the chemotherapy usage, however, it is thought that chemotherapy may be warranted as an upfront treatment modality on the basis of neuroaxis failure and recurrences.…”

Section: Discussionmentioning

confidence: 94%

Pediatric primary anaplastic ganglioglioma with malignant neuronal component

et al. 2018

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Kaymak-Cihan M, Erdiş E, Bozkurt S, Ünver-Korğalı E. Pediatric primary anaplastic ganglioglioma with malignant neuronal component. Turk J Pediatr 2018; 60: 102-106. Gangliogliomas (GGs) represent approximately 0.4%-1.0% of all brain tumors. Anaplastic gangliogliomas (AGGs) form 5-10% of all GGs. They are a mixed neuronal-glial tumor of central nervous system and composed by two cell lines; neuronal (ganglionic) and glial cells. Anaplastic component of AGGs is usually glial cells. Malignant neuronal component is a rare condition. Here we report an 8-year-old male patient who was diagnosed with primary AGG with malignant neuronal component and was treated with surgery, adjuvant radiotherapy and chemotherapy.

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“…2,5,6 Moreover, some authors use the presence of anaplastic histologic features or a MIB-1 labeling index > 10% to define a high-grade tumor. 2,4,6,7 The diagnosis of ganglioglioma by using neuroimaging (computed tomography and MRI) is mainly dependent on the delineation of a solid or partially cystic mass. 8,9 There are focal calcifications in 35% of tumors and enhancement with contrast material in 50%.…”

Section: Discussionmentioning

confidence: 99%

“…However, there is insufficient data supporting the indications and timing for radiotherapy and chemotherapy. 2,6 Scoccianti et al 6 reported an indication for conformal radiotherapy to the operative bed plus a 10-mm safety margin. In the present case, we employed adjuvant therapy based on current treatment standards for other high-grade gliomas aimed at an extended recurrence-free survival.…”

Section: Discussionmentioning

confidence: 99%

Anaplastic Ganglioglioma of the Pineal Region – A Case Report

Costa

Centeno

Neto³

et al. 2015

Arq Bras Neurocir

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Introduction Gangliogliomas are tumors commonly found in the temporal lobe and related to seizures; their appearance in the pineal region is rarely described. This report characterizes the first case of anaplastic ganglioglioma of the pineal region. Case Report The authors describe the case of a 32-year-old woman that developed progressive headache. An MRI investigation revealed a pineal tumor. The patient tested negative for biomarkers and underwent surgery through supracerebellar infratentorial approach and achieved gross total resection of the tumor in a challenging location. Pathological analysis revealed a biphasic neoplasm with the following two distinct phenotypes in separate fields: an immature neuronal component with several atypical mitoses and a mature astrocytic component with bipolar cells, microcysts, and eosinophilic bodies. The Ki67/MIB1 proliferation index was 20-30% in localized hotspots. Based on the pathological findings, the tumor was defined as an anaplastic ganglioglioma World Health Organization (WHO) grade III. Discussion/Conclusion Gangliogliomas are classified as glioneural neoplasms based on the histologic findings described as a mixture of neoplastic astrocytes and neurons. Moreover, these tumors represent 0.4-1.3% of tumors of the central nervous system. Authors describe de novo anaplastic ganglioglioma as 1% of the largest series. Gross total resection and adjuvant treatment may play important role in patients' prognostic. In this case, due to the malignant anaplastic component of her tumor, the patient received treatment with temozolamide and radiotherapy after gross total resection of the lesion.

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Pediatric Primary Anaplastic Ganglioglioma: A Case Report and Review of the Literature

Cited by 15 publications

References 52 publications

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

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

Pediatric primary anaplastic ganglioglioma with malignant neuronal component

Anaplastic Ganglioglioma of the Pineal Region – A Case Report

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