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2.1
Ganglioglioma

Images

WIKIDATA, Public Domain

Presentation

METHODS: In this report, we describe an atypical presentation of a cerebellar GG in a 20-month-old male who initially presented with syncope and emesis in the absence of electrographic evidence of seizures, radiographic evidence of hydrocephalus, or elevated [ncbi.nlm.nih.gov]

Clinical presentation Symptoms are site-specific, but are stereotypically long-standing. Ganglioglioma is the most common tumor associated with chronic temporal lobe epilepsy. Seizures are the most common presenting symptom. [prod.wiki.cns.org]

Entire Body System

  • Weakness

    Neurological examination revealed mild motor weakness of the right lower extremity and slightly decreased sensation in the left lower extremity. Hyperhidrosis was observed in the right C3-T8 dermatomes. [ncbi.nlm.nih.gov]

    He was discharged for outpatient systemic polychemotherapy, but worsening headaches, vomiting, weakness, confusion, bradylalia and incoherent phrases led to his readmission a month later. [elsevier.es]

  • Fatigue

    He presented with headache, fatigue and occasional nausea and vomiting. MRI revealed three large, distinct tumours with striking cyst formation. Stereotactic craniotomy and excision of the temporal and occipital tumours confirmed ganglioglioma. [ncbi.nlm.nih.gov]

    We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019 [patientslikeme.com]

    Other symptoms are associated with increased pressure in the brain, including: headache — generally upon awakening in the morning nausea and vomiting – often worse in the morning and improving throughout the day fatigue weakness on one side of the body [childrenshospital.org]

Gastrointestinal

  • Nausea

    He presented with headache, fatigue and occasional nausea and vomiting. MRI revealed three large, distinct tumours with striking cyst formation. Stereotactic craniotomy and excision of the temporal and occipital tumours confirmed ganglioglioma. [ncbi.nlm.nih.gov]

    […] provide support and protection of neurons) Epidemiology of Ganglioglioma especially in children and young adults represent less than 2% of all primary brain tumours Symptoms of Ganglioglioma epileptic seizures signs of intracranial pressure (headache, nausea [hirntumorhilfe.org]

    Deling (2013) 15/M 1 year Headache, nausea, unsteady gait GTR + − No recurrence (19 months) 5. Deling (2013) 27/M 2 years Headache TR + − No recurrence (8 months) 6. [jstage.jst.go.jp]

    A young adult female presents with generalized headaches of several months duration, which recently progressed in intensity with associated nausea and vomiting. She was in previously good health without significant past medical history. [arkanalabs.com]

  • Constipation

    During this period, lie also developed numbness and weakness of both legs along with retention of urine and constipation. [jpma.org.pk]

Neurologic

  • Headache

    However, neuroimaging should be rationalized in children with headaches, as it is reported to be frequently used in patients with migraines, chronic headaches or headaches without red flags. 11 The head MRI findings reported a greater impact on one lobe [elsevier.es]

    A 61-year-old male presented with a 12-day history of headaches and general malaise. [ncbi.nlm.nih.gov]

    A 12-year-old boy suffered from headache and gait disturbance. Neuroimaging studies demonstrated a large enhancing cerebellar mass with cystic components compressing the forth ventricle. [jkns.or.kr]

  • Dizziness

    Reduced hearing, dizziness and weakness of both lower limbs were found in 1 case. Intracranial hypertension were detected in 5 cases, including 1 case complicating by decreased visual acuity. [ncbi.nlm.nih.gov]

    Case Report : We present a case of a 36 year–old female patient with an 18–month history of headache and dizziness. Imaging studies showed a cerebellar lesion, with compression of the fourth ventricle. [austinpublishinggroup.com]

    CASE REPORT A 45-yr-old female patient presented with a history of headache and dizziness, both of which progressed slowly. A neurological examination showed nothing remarkable. [synapse.koreamed.org]

    Case Description: In this report, we describe a 36-year-old woman who presented with a seizure followed by worsening headache, dizziness, confusion, and intermittent left facial numbness over the next few weeks. [surgicalneurologyint.com]

    The most common presenting signs and symptoms are seizures (temporal lobe and other supratentorial locations), followed by headache, dizziness, ataxia (posterior fossa), and progressive weakness (spinal cord). [ejns.springeropen.com]

  • Convulsions

    We describe a case of a 24-year-old male presenting with convulsions and a calcified parieto-occipital mass. [ncbi.nlm.nih.gov]

    Subsequently, she had convulsive status epilepticus and a repeated MRI demonstrated the reappearance of the contrast-enhancing mass. Biopsy demonstrated a ganglioglioma. [n.neurology.org]

    […] neuroimaging, with tumors being the main differential diagnosis. 6–10 This was seen in this case study patient, who began with progressive, oppressive bilateral occipital headaches, altered visual acuity, projectile vomiting, dysarthria, somnolence, weakness, convulsions [elsevier.es]

  • Paresthesia

    He began to experience photophobia, dizziness, left-sided facial paresthesias, and a self-limited bifrontal headache. [elsevier.es]

    The prime symptom of Ganglioglioma is episodic epileptic seizure attack and others are as follows: Headaches High intracranial pressure Nausea Vomiting Blurry vision Paresthesia or one-sided body weakness The above mentioned clinical presentations are [cancerwall.com]

    Materials and Methods Clinical Summary A 20-year-old healthy woman presented with a three-week progressive history of headaches, nausea and vomiting, blurry vision, and left-sided paresthesia. [ncbi.nlm.nih.gov]

  • Focal Seizure

    Infratentorial GGs typically present with signs and symptoms of increased intracranial pressure (ICP), cranial nerve deficits, or focal cerebellar findings; rarely, these tumors have been associated with focal seizures. [ncbi.nlm.nih.gov]

    Clinical vignette LT, a 30-year-old, right-handed mechanical engineer, had a history of focal seizures since 12 years of age; these seizures were characterized by odd smell, spreading discomfort, and “déjà-vu” lasting less than a minute, with postictal [medlink.com]

    While gangliogliomas can be found throughout the central nervous system, from the cerebral hemispheres to the spinal cord, most tumors occur in the temporal lobe and present with focal seizures. [surgicalneurologyint.com]

Workup

Extensive workup for an inflammatory or infectious etiology was unfruitful and two brain biopsies were inconclusive. [ncbi.nlm.nih.gov]

As with all neoplasms, the workup for ganglioglioma includes cerebral imaging (CT or MRI) followed by surgical resection. Imaging characteristics are highly variable due to the various pattern of growth and possible locations of these tumors. [medlink.com]

Other Pathologies

  • Lymphocytic Infiltrate

    Neuropathology of the frontal lobe lesion (figure 2A) demonstrated a moderately cellular proliferation of mixed dysplastic neuronal/ganglionic and astrocytic glial cells, with eosinophilic granular bodies and focal lymphocytic infiltrates. [casereports.bmj.com]

    No conspicuous perivascular lymphocytic infiltrate was seen. [cancerjournal.net]

    Fibrovascular stroma confined to the neuronal component, perivascular lymphocytic infiltrates, and small foci of calcification are common, as is immunopositivity for synaptophysin, neuron-specific enolase, and chromogranin A. [en.wikipedia.org]

Treatment

Treatment with Vemurafenib as single agent was started. For the first time, a radiological and clinical response was obtained after 3 months of treatment and sustained after 6 months. [ncbi.nlm.nih.gov]

Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment. [revistaneurocirugia.com]

Patients usually present with seizures refractory to medical treatment. Association with neurofibromatosis type I and tuberous sclerosis has been reported. [orpha.net]

Prognosis

Its prognosis and relationship with complicating hydrocephalus are unclear. [ncbi.nlm.nih.gov]

Desmoplastic medulloblastoma is more common in children younger than 3 years of age and has a better prognosis. 14 As in neuroblastoma, maturation of medulloblastoma cells may be associated with a better prognosis. 11 This case suggests that some types [pediatrics.aappublications.org]

Local resection is the treatment of choice and determines prognosis. [radiopaedia.org]

Prognosis A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. [braintumour.ca]

Etiology

Extensive workup for an inflammatory or infectious etiology was unfruitful and two brain biopsies were inconclusive. [ncbi.nlm.nih.gov]

They may co-occur with adjacent focal cortical dysplasia (FCD IIIb), suggesting a common developmental etiology for both structural abnormalities. [epilepsydiagnosis.org]

The etiology of gangliogliomas is controversial as is their biological potential. Some lesions display neoplastic features including the potential to enlarge and behave in a malignant fashion. [path.upmc.edu]

[…] lead to activation of the MAPK pathway and therefore cause increased cell proliferation Gangliocytoma: Data not available but likely closely resembles that of ganglioglioma References: Acta Neuropathol Commun 2020;8:30, Folia Neuropathol 2013;51:283 Etiology [pathologyoutlines.com]

Epidemiology

MRI Ongoing Trials at Clinical Trials.gov US National Guidelines Clearinghouse NICE Guidance FDA onGanglioglioma epidemiology and demographics CDC on Ganglioglioma epidemiology and demographics Ganglioglioma epidemiology and demographics in the news [wikidoc.org]

Through this case and literature review, the authors discuss some epidemiological, histological, clinical, radiological and management features of this very rare tumor. [ncbi.nlm.nih.gov]

Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database. Neurosurgery. 2015 ;76: 313 – 319. Google Scholar | Crossref | Medline 6. [journals.sagepub.com]

Pathophysiology

Ganglioglioma Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Epidemiology and Demographics Risk Factors Screening Differentiating Ganglioglioma from other Diseases Natural History, Complications [wikidoc.org]

[…] incidence in epilepsy related surgical resections is 1 - 3% (Brain Pathol 2012;22:350, N Engl J Med 2017;377:1648) Sites Can occur throughout the CNS; temporal lobe is most common (~70%) (Acta Neuropathol 1994;88:166, J Neuropathol Exp Neurol 2002;61:575) Pathophysiology [pathologyoutlines.com]

Operation To clarify the pathophysiologic features of the lesion and relieve symptoms, the patient was placed in the prone position and given a partial resection of lesion in the medulla oblongata. [tcr.amegroups.com]

However, in ganglioglioma, the extent of the epileptogenic zone is unclear and the pathophysiological mechanisms underlying epileptogenicity are not well characterized. [aepi.biomedcentral.com]

Prevention

The patient is scheduled for adjuvant radiotherapy with the hope of prevention of progression. [ncbi.nlm.nih.gov]

There are no known risk factors or ways to prevent the disease. The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems DIG can cause. Use the menu to choose a different section to read in this guide. [cancer.net]

Therefore, currently, no guidelines or methods are available towards preventing tumor formation Active research is currently being performed to explore the possibilities for treatment and prevention of tumors such as Anaplastic Ganglioglioma Regular medical [dovemed.com]

Radiation therapy Radiation therapy uses X-rays to kill the cells and prevent them from growing. External radiation therapy, a type of radiation therapy uses machines outside the body to deliver the dose. [isitcurable.com]

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