Malignant cerebellar ganglioglioma. A case report and review of the literature

Ganglioglioma is usually a well differentiated slowly growing mixed neuronal and glial neoplasm corresponding to WHO grade I or II. However, some gangliogliomas are considered to be WHO grade III because they exhibit anaplastic features in their glial component. Finally there are exceptionally rare cases of newly diagnosed gangliogliomas with grade IV changes in the glial component. We report a case of a 25-year-old woman with a family history of neurofibromatosis who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell multiform tumor glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. Histologically, two cell populations were noted: a predominant glial component consisting in a multiform glioblastoma and ganglion cells supporting a diagnosis of ganglioglioma. Immunohistochemical analysis clearly distinguished the two tumor cell populations. Although other cases of grade III gangliogliomas and twelve cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the second case of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histological findings in a multiform glioblastoma and a ganglioglioma. It also documents the aggressive biologic behavior of this complex neoplasm.     

Ganglioglioma revealed by spontaneous intracerebral hematoma: A cohort study

BackgroundGangliogliomas are rare tumors of the central nervous system. Intracerebral hemorrhage (ICH) is a frequent pathological event, rarely related to intracranial tumor. Here we report a cohort of 14 gangliogliomas, 3 of which were revealed by ICH. We also performed a systematic review of the literature on gangliogliomas revealed by ICH.Patients and methodsWe retrospectively collected data for all gangliogliomas operated on in our department between January 2015 and December 2019: clinical history, radiological data and outcome, with a special focus on gangliogliomas revealed by spontaneous ICH. All cases had pathological validation. For the systematic review, relevant studies were identified by systematic search of the scientific literature in PubMed.ResultsFifteen patients underwent surgery for ganglioglioma during the study period. Six cases were revealed by seizures, 3 by headache, 1 by hydrocephaly and 1 by developmental disorder. Three patients (21%) had hemorrhagic presentation. All patients were male, aged 30 to 48 years, diagnosed with atypical ICH without vascular abnormality on cerebral angiogram. Conservative management was first decided. Diagnosis of ganglioglioma was made within 3 months for 2 patients and 9 years later for 1, after surgical removal and histological analysis. All hemorrhagic gangliogliomas were BRAF wild type. The systematic review of the literature identified 8 other cases of ganglioglioma revealed by ICH.ConclusionICH may be a mode of revelation of ganglioglioma, and ganglioglioma is a possible etiology of atypical ICH in young patients. Long-term imaging follow-up is mandatory in patients with unexplained ICH.     

Multiple gangliogliomas of the optic pathway

We report a case of multiple gangliogliomas of the optic pathway in an 18-year-old boy. He presented with visual disturbance mainly in his left eye, non-specific headaches, and episodes of sensory disturbance in his left arm. Visual acuity was 6/9 and 6/24 in his right and left eye respectively. He did not have any Lisch nodules. Optic atrophy was noted in his left eye. MRI scan revealed multiple enhancing lesions involving the optic chiasm, left optic tract, right lateral geniculate body, and right optic radiation in the temporal lobe. Stereotactic biopsy of the temporal tumour confirmed ganglioglioma. Multiple gangliogliomas of the optic pathway has not been hitherto described.     

Ganglioglioma presenting as a meningioma: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Gangliogliomas are intra-axial, avascular masses located predominantly in the temporal lobe. A ganglioglioma that mimics a meningioma in that it is extra-axial and has a significant extracranial vascular supply has not been reported previously. CLINICAL PRESENTATION: A 12-year-old girl presented with a right temporoparietal mass. A neurological examination revealed nothing abnormal, and the girl's symptoms were limited to headaches. INTERVENTION: Magnetic resonance imaging revealed an extra-axial mass, and cerebral catheter angiography revealed a blood supply mainly from the posterior division of the right middle meningeal artery. Intraoperative findings confirmed the extra-axial location of the tumor, and histological analysis revealed that the tumor was a ganglioglioma. CONCLUSION: This report confirms that gangliogliomas can present as extra-axial, vascular masses that are similar to meningiomas.     

Neuroblastomatous recurrence of ganglioglioma. Case report

This case is believed to be the first reported recurrent intracranial ganglioglioma with purely neuroblastomatous malignant transformation. A complete macroscopic resection of a right frontal lobe tumor in an 18-year-old woman revealed differentiated ganglioglioma. Seven years later a large, well-demarcated recurrent tumor was again macroscopically totally resected in the same patient. Histological analysis showed malignant transformation in only the neuronal component of the original tumor. A review of the literature on recurrent gangliogliomas and their malignant transformation is included.     

Intraventricular ganglioglioma: a short illustrated review

The following review of the literature describes the ganglioglioma, an uncommon mixed glioneuronal neoplasm, most often of low-grade histology, with a small, albeit well-documented, malignant potential. These tumors exhibit a strong epileptogenic propensity and most often present as new onset seizures or are discovered after a long history of refractory epilepsy. Despite their indolent course, the importance of gross total resection is well recognized to prevent anaplastic and malignant degeneration. Morphologically, the neoplasm is often cystic with an enhancing mural nodule, but can also be entirely solid. They are most often found in the temporal lobe but have been found throughout the neuraxis. An exceedingly rare location of the ganglioglioma is within the lateral ventricle. A systematic literature search revealed only eight reports documenting the occurrence of a ganglioglioma within the lateral ventricle. We describe an illustrative case of an intraventricular ganglioglioma with a prominent cystic component and enhancing mural nodule, which represents the classic radiographic appearance of gangliogliomas described in other locations. A superior parietal lobule approach offered excellent surgical access for tumor removal and the patient has remained free of neurological deficits following surgery. Regardless of location within the central nervous system, ganglioglioma should be on the differential diagnosis for any cystic mass with a mural nodule, particularly in the setting of epilepsy.     

Desmoplastic non-infantile ganglioglioma. Case report

Desmoplastic gangliogliomas are rare mixed glial and neuronal cerebral tumors, especially described in infants below 4 years of age but exceptional cases have been reported in young adults. These tumors are generally localised in parietal or temporal lobes, present as a large cystic lesion with peripheral contrast enhancement. They also have characteristic histological features: extensive desmoplasia and tumoral cells of variable size exhibiting immunohistochemical and ultrastructural features of glial and neuronal differentiation. Total surgical removal is sufficient for the treatment of these tumors and no radiotherapy or chemotherapy are indicated if complete resection is achieved. We report a case of desmoplastic ganglioglioma in a 19-year-old male. This tumor presented as a large parieto- temporal cystic lesion with rimmed contrast enhancement. At histological examination, this tumor exhibited extensive desmoplasia and comprised 2 types of tumoral cells: small cells with round nuclei, positive for NSE, neurofilaments and synaptophysin and sometimes presenting typical morphological features of neuronal differentiation, and large cells with abundant eosinophilic strongly staining for GFAP. This observation emphazises on the fact that desmoplastic ganglioglioma can no more be considered as a specific entity of infancy and must be well recognised even in young adults because it may be misdiagnosed as malignant glioma.     

Malignant transformation of a gangliocytoma/ganglioglioma into a glioblastoma multiforme: a molecular genetic analysis. Case report.

A gangliocytoma/ganglioglioma with no atypical or malignant features was subtotally resected from the right temporal lobe of a 16-year-old woman. A second resection was performed 8 years later to treat a locally recurrent lesion with increased cellularity that was diagnosed as a World Health Organization Grade II ganglioglioma on the basis of neuropathological examination. Molecular analysis of the recurrent tumor revealed a TP53 gene mutation, but no amplification of the epidermal growth factor receptor (EGFR) gene. Radiotherapy (60 Gy) was administered after the second resection. The patient returned 1 year later with a second focal recurrence. The specimen obtained during the third resection of tumor exhibited exclusively astrocytic differentiation, cellular pleomorphism with multinucleated cells, high mitotic activity, and endothelial proliferation. Therefore, the tumor was diagnosed to be a glioblastoma multiforme (GBM). Molecular analysis of tumor DNA from the second recurrent tumor demonstrated the presence of the TP53 mutation, which previously had been observed in the first recurrent tumor, but again no evidence of EGFR amplification. Findings demonstrate that the presence of TP53 mutation in progressed gangliogliomas should be interpreted as a progression-associated mutation rather than a consequence of treatment. This is the first report to indicate that the molecular pathways of gangliocytomas/gangliogliomas progressing to become GBMs may parallel those of diffuse astrocytomas progressing to become GBMs.     

Cerebral Gangliogliomas: Clinical Characteristics, CT and MRI

Summary  Eight patients with ganglioglioma who received surgical treatment at our institute between January 1989 and January 1997 were reviewed to determine their clinical, CT and MRI characteristics. Tumours were located in the temporal lobe (four patients), trigone of the lateral ventricle (two patients), basal ganglia (one patient) and fronto-temporal lobe (one patient). On imaging, two types of tumours were seen, a solid mass in 5 patients (62.5%) and a cystic mass in three patients (37.5%). Six complete tumours (75%) and all of the solid components of the cystic tumours were enhanced by contrast medium. Seven tumours (88%) had no peripheral oedema. On CT, the tumours being studied appeared as iso-(62.5%) or low density (37.5%) intra-axial tumours. Four tumours (50%) contained calcification. On MRI, the tumours appeared as well-circumscribed, iso- (62.5%) or low intensity (37.5%) intra-axial tumours on T1 weighted images, and as high (75%) on T2 weighted images. Three underwent total resection, 2 subtotal resection and 3 partial resection. No patients had have any further treatment such as radiation therapy or chemotherapy. Postoperative sudies were conducted on all patients with an average follow-up period 56 months (range 4–147 months) after surgery. There was no evidence of recurrence of tumours or of growth of residual tumours. We observed gangliogliomas which were located in unusual regions such as the trigone in two of the patients. To our knowledge, our series is the first report to describe trigonal gangliogliomas. We conclude, therefore, that ganglioglioma should be included as a possibility in the differential diagnosis of intracranial masses, even when they are located in the trigone.     

Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature

Desmoplastic gangliogliomas (DIG) are rare primary neoplasms that comprise 0.5–1.0% of all intracranial tumors. Clinically, there are two forms of DIG, the infantile and the non-infantile. These tumors invariably arise in the supratentorial region and commonly involve more than one lobe, preferentially the temporal and frontal. On neuroimaging are seen as large hypodense cystic masses with a solid isodense or slightly hyperdense superficial portion. The histologic diagnosis is characterized by the presence of three different cell lines: astrocytic, neuronal, and primitive neuroectodermal marker sites, which were demonstrable. The treatment of choice is radical surgical excision, and if this is done, achieved complete healing of the patient does not require additional treatment. A literature review of DIG was compiled through Medline/Ovid using the keywords “desmoplastic infantile ganglioglioma”, “desmoplastic non-infantile ganglioglioma” covering the years 1984–2009. We present a review of a total of 113 cases of infantile (94) and non-infantile gangliogliomas (19) published to date, examining the clinical, radiologic, surgical, and pathological aspects, as well as the outcome. Desmoplastic gangliogliomas represent a rare tumor group with two well-defined age groups, the children and non-children. Desmoplastic infantile gangliogliomas are the most common and occur in children below 5 years of age, and the large majority of them present within the first year of life. Surgery is the treatment of choice and no complementary treatment is needed in cases of complete tumor resection.     

A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature

Desmoplastic infantile gangliogliomas (DIG) are uncommon supratentorial brain tumors with a usually good prognosis despite an aggressive radiological appearance that typically occurs in infants below the age of 24 months. DIGs are exclusively supratentorial, generally have a voluminous size, and are partially cystic. Total surgical removal is sufficient for the treatment of these tumors, and no chemotherapy or radiotherapy is indicated if complete resection is achieved. Except for age difference, DIG and desmoplastic non-infantile gangliogliomas are radiologically and histologically similar. Non-infantile variants of this biologically benign intracranial neoplasm are rare; only 15 cases of non-infantile DIGs have been reported in the literature. As far as we know, this case is the 16th in literature. We reported the magnetic resonance imaging and histological findings of desmoplastic ganglioglioma in a 6-year-old boy.     

Ganglioglioma of the medulla oblongata.

A patient with signs and symptoms suggestive of symptomatic Arnold-Chiari malformation was encountered. Neuroradiologic investigation revealed the presence of a medullary tumor, which later proved to be a ganglioglioma. A review of the literature suggests that this presentation is typical of medullary gangliogliomas. It is proposed that the pathophysiology of this lesion renders it amenable to decompressive therapy via suboccipital craniotomy.     

Cerebellar ganglioglioma associated with a huge cyst: case report

Gangliogliomas represent approximately 0.2% of all the intracranial tumors. Ganglioglioma arising from the cerebellum is rare, with a rate of 1.5-9% of CNS gangliogliomas. The authors report a case of cerebellar ganglioglioma with a huge cyst. A 28-year-old man presented headache and ataxia. Computerized tomography (CT) demonstrated a huge cyst at the vermian region with calcification located at the peripheral side of the cyst. Magnetic resonance imaging (MRI) with administration of Gd-DTPA showed a slightly enhanced small mass at the left side of the cyst. Preoperative diagnosis of the lesion seemed to be a cystic astrocytoma. The tumor was removed subtotally through the midline suboccipital approach. Pathological examination of the tumor specimen revealed a ganglioglioma. The postoperative course was uneventful with no sign of tumor regrowth on repeated MRI. According to the previous 17 reports of cerebellar ganglioglioma including our case, the shorter interval from onset to the diagnosis and clinical symptoms such as increased intracranial pressure were conspicuous as compared with supratentorial ganglioglioma because of the anatomically narrow space of the posterior fossa. Neuroradiological findings showed tumor enhancement in 86% of the cases, calcification in 67%, and cyst formation in 53%.     

Ganglioglioma of conus medullaris

Summary Gangliogliomas are glioneuronal tumours of the young, and occur more frequently supratentorially. Among those uncommon cases in the spinal cord, the conus medullaris is an extremely rare site. We reviewed the literature of such cases and report another case of a ganglioglioma of the conus in a 13-year-old boy with an insidious sensory sensitive and motor deficit of the right lower limb. Subtotal resection was accomplished with no recurrence after two years of follow-up. As for the supratentorial locations, the extent of surgical resection is the main prognostic factor for gangliogliomas of the spinal cord, and they should be considered in the differential diagnosis of intramedullar neoplasm.     

Multiple intracranial ganglioglioma. Case report

Multiple intracranial ganglioglioma (GG) is an extremely rare condition, generally associated with van Recklinghausen neurofibromatosis. We report the case of a 26-year-old woman who presented with progressive tetraparesis, raised intracranial pressure and visual loss. The neuroradiologic workup demonstrated a multiple lesion involving the temporal lobe, the diencephalomesencephalon and the optical pathways. Computed tomography-guided stereotactic biopsy of a right temporal lesion led to the diagnosis of GG. In light of this case and the literature review, we discuss morphologic aspects, differential diagnosis and therapeutic options of GG. In patients with multiple cerebral tumors, search for multiple ganglioglioma is particularly important because of the therapeutic implications and the good prognosis of this tumor.     

Prognostic Factors in Supratentorial Ganglioglioma

Summary  Although gangliogliomas are often associated with long survival, efforts to identify specific prognostic factors in these tumors have been largely unsuccessful. To identify factors associated with long survival, we retrospectively reviewed 42 cases of supratentorial ganglioglioma surgically treated at our institution since 1985. Data analysis included Kaplan-Meier survival curves and log-rank tests of the effects of individual variables. The Cox proportional hazards method was used to fit a model incorporating several variables simultaneously.  The 42 cases included 21 male and 21 female patients with an average age at surgery of 31 years. Length of follow-up averaged 48 months. Ten patients died an average of 38 months after surgery. Factors found to have a significant correlation with mortality were older age at diagnosis (P=0.012), male gender (P=0.034), and malignant glial features (P=0.020). Presenting symptoms, location of tumor, adjuvant radiation therapy, and extent of surgical resection were not significantly related to survival.  These results are the first to demonstrate an association between prognostic factors and outcome in patients with supratentorial ganglioglioma.     

Ganglioglioma of the brainstem: report of three cases and review of the literature.

BACKGROUND: Brainstem gangliogliomas are rare low-grade tumors that usually have a long clinical history. However, they may cause sudden death. There are only 31 cases of brainstem ganglioglioma reported in the literature, and only one has been studied with magnetic resonance (MR). We present three new cases of brainstem ganglion cell tumor studied with computed tomography (CT) (3 cases) and MR (2 cases) and discuss the clinical presentation, diagnostic imaging and treatment of these tumors. CASE DESCRIPTION: Age at presentation ranged from 19 to 59 years old. Two patients were female and 1 male. Duration of symptoms before diagnosis ranged from 1 year to nearly 14 years. Presenting complaints included syncope spells, cranial nerve deficits, headache, and gait instability. Imaging studies revealed well-circumscribed lesions involving the brainstem; the lesion was cystic in one case and calcified in one. They were iso- or hyperdense on CT scan, isodense on T1-weighted and hyperdense on T2-weighted MRI and frequently showed contrast enhancement.All tumors were operated through a posterior fossa craniectomy. Using microsurgical techniques only partial resection could be achieved, as there was no sharp delineation from the surrounding tissue in any case. Two of our patients had increased neurological deficits after surgery. Radiotherapy was not given. Follow-up of tumoral remnants has not shown clear tumor growth after 1, 3.5, and 10 years. CONCLUSIONS: Imaging characteristics of brainstem gangliogliomas do not seem to differ from those in other locations and are not specific. Radical surgery is rarely if ever possible, nor is it advisable because of the risk of functional deterioration. However, because of their benign histology, partial resection seems to carry a similar prognosis as tumors in other locations that are amenable to complete resection.     

Non-enhancing de novo glioblastoma: report of two cases

Malignant gliomas arise from two distinct pathways, as de novo lesions or from secondary transformation from low-grade lesions. Herein, we describe the cases of two patients to illustrate the proposition that de novo malignant gliomas can originate as non-enhancing tumors and rapidly progress to a pattern of ring enhancement characteristic of a glioblastoma. Both patients presented with new-onset seizures (simple partial and generalized). Their neurological examinations were unremarkable. Initial MRI evaluations revealed a right precentral gyrus and right medial temporal lobe lesions in each case, respectively. These lesions demonstrated increased T2 signal changes without contrast enhancement. The biopsy of the right frontal lesion in the first patient was consistent with an anaplastic astrocytoma; the second patient was followed expectantly. Repeat MRI for both patients within 17 weeks disclosed ring-enhancing lesions, consistent with an unusually rapid evolution to glioblastoma multiforme (GBM). Subsequent resection of the right medial temporal lesion in the second patient revealed a GBM. Neither tumor displayed abnormal overexpression of P53 by immunohistochemistry. Early MRI of de novo glioblastomas may demonstrate a non-enhancing tumor suggestive of a low-grade lesion. These tumors can rapidly evolve into ring-enhancing lesions more consistent with the traditional imaging findings.     

Anaplastic ganglioglioma in an infant: case report and review of the literature

A 6-month-old girl had a gradually increasing head circumference. A preoperative computed tomographic (CT) scan of the head revealed an enhancing calcified partially cystic right frontal mass that was removed through a right frontotemporal craniotomy. On microscopic examination, the tumor was composed of sheets of neurons in a glial background alternating with highly cellular anaplastic areas. The diagnosis of anaplastic ganglioglioma was made. The child has done well for the 20 months since the operation without any evidence of tumor recurrence on subsequent CT scans. Because of the immaturity of the child's developing central nervous system, we have elected not to initiate radiotherapy at this time. The pertinent literature regarding gangliogliomas is reviewed.     

Anaplastic ganglioglioma: case report and review of the literature

A 10-year-old girl underwent radical decompression of a right temporal tumour extending to the insular cortex which proved to be a differentiated ganglioglioma. One year later, she presented with a bleeding right frontal tumour extending to the insula, the histology of which showed it to be the anaplastic variant of ganglioglioma. She underwent postoperative radiotherapy but died 6 months after the second operation. Anaplastic ganglioglioma is a rare variant of ganglioglioma and progression of the latter to anaplastic ganglioglioma is an extremely rare occurrence. The literature regarding ganglioglioma is reviewed.