Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The location of ganglioglioma (GG) within the infratentorial compartment is unusual. The authors report a rare case of GG in the cerebellar hemisphere. 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. After complete resection of the tumor, the patient became symptom free. Histological examination on the tumor disclosed glial cells and dysplastic ganglion cells. Although it is a rare tumor, in the appropriate clinical setting, a GG should be considered in the presence of a cerebellar mass with both solid and cystic components on magnetic resonance images in children.
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PMID:Cerebellar ganglioglioma. 1909 27

Here we report a rare case of cerebellar ganglioglioma accompanied by a large cyst, and present a review of the reported 28 cases with cerebellar ganglioglioma. An otherwise healthy 46-year-old woman complained of gradual headache and truncal ataxia. MRI revealed a huge cystic lesion with a mural nodule in the left cerebellar hemisphere. The tumor was resected totally. Histologically, it was composed of neuronal and glial elements, and was accordingly diagnosed as ganglioglioma.
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PMID:Ganglioglioma originating in the cerebellum with a large cyst--a case report and review of the literature. 1913 Jul 32

We present a rare case of an adult patient without seizures who is found to have a ganglioglioma occurring in the cerebellopontine angle. A 52-year-old woman with ataxia, headaches, and falling episodes underwent neuroimaging. Magnetic resonance imaging (MRI) revealed a smooth, somewhat lobulated mass in the left cerebellopontine angle. The mass was hypointense on T1-weighted imaging, hyperintense on T2-weighted imaging, and did not enhance after administration of gadolinium. Left retromastoid craniectomy was performed, and the mass was noted to be exophytic from the brain stem. The exophytic component was resected. Light microscopic findings were consistent with ganglioglioma. This was confirmed with immunohistochemical studies. Ganglioglioma is a rare tumor of the central nervous system that typically presents with seizures in children and young adults. Occurrence of this tumor in the cerebellopontine angle is extremely unusual; this rarity is magnified by its occurrence in an adult patient without a history of seizures. Our case illustrates that ganglioglioma should be considered in the differential diagnosis of cerebellopontine angle masses at any age. This appears to be especially true when dealing with masses that are non-enhancing on imaging.
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PMID:Ganglioglioma in the cerebellopontine angle of an adult without seizures. 1977 36

Desmoplastic gangliogliomas are mixed cerebral tumors traditionally reported in infants. However, a few non-infantile cases have been documented. A case of a desmoplastic ganglioglioma in a 16-year male is presented. The patient reported severe headaches. Radiological examination revealed a large mass occupying the right frontal lobe. The lesion was totally excised. Histopathological examination confirmed the diagnosis of a desmoplastic ganglioglioma. The postoperative course was excellent. At the 10(1/2) year follow-up there was no evidence of tumor recurrence. Although desmoplastic gangliogliomas have aggressive features, complete surgical removal is the treatment of choice obviating the need for adjuvant therapy.
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PMID:Long-term follow-up of a non-infantile desmoplastic ganglioglioma. 2002 87

We report a case of de novo malignant ganglioglioma. A 61-year-old male presented with a 12-day history of headaches and general malaise. Pre-operative magnetic resonance imaging revealed an irregular enhancing mass in the left temporal lobe with associated dural enhancement and subacute subdural hematoma. The findings at surgery were of a vascular tumor intimately involving the surrounding white matter, with central necrosis. Histological and immunohistochemical studies showed an anaplastic ganglioglioma with World Health Organization grade IV characteristics. Gangliogliomas of the central nervous system are rare and are typified by a pediatric predilection and indolent behavior. A review of the de novo anaplastic and malignant gangliogliomas is presented.
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PMID:Malignant ganglioglioma: case report and review of literature. 2052 41

With the exception of oligoastrocytoma, mixed gliomas are rarely encountered, and the astrocytic component of mixed oligoastrocytoma is almost always fibrillary and diffusely infiltrative. Pleomorphic xanthoastrocytoma (PXA) has occasionally been described in conjunction with ganglioglioma, as well as in 1 case of oligodendroglioma. In this latter case, described by Perry et al., 1p/19q codeletions were not detected. The authors report on a 25-year-old woman with a combined PXA/oligodendroglioma in which concurrent 1p/19q codeletions were detected in the oligodendroglial component only. The patient presented with a 1-month history of headaches. Neuroimaging revealed a heterogeneous left temporal mass with focal enhancement, cystic changes, hemorrhage, and left-to-right midline shift. The patient underwent a craniotomy and gross-total resection. Pathological examination revealed a glial tumor composed of 2 apparently distinct components. The largest component exhibited a prominent fascicular, reticulin-rich, spindle cell arrangement admixed with areas of highly pleomorphic cells, with bizarre cytological features reminiscent of PXA. A smaller component was composed of cellular sheets and lobules of oligodendroglial cells. Both components were characterized by anaplastic features. Dual-color fluorescence in situ hybridization for 1p/19q codeletions was performed. Only the oligodendroglial component showed the combined 1p/19q deletions. This case represents the first instance in which PXA has been reported in conjunction with an oligodendroglioma exhibiting the "molecular signature" characteristic of oligodendroglial neoplasms. The different genetic alterations seen in the 2 components of this neoplasm argue in favor of a "collision tumor" rather than a mixed glioma of the same genotype.
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PMID:Pleomorphic xanthoastrocytoma and oligodendroglioma: collision of 2 morphologically and genetically distinct anaplastic components. 2121 37

We describe a rare case of desmoplastic infantile ganglioglioma that appears to have transformed into a glioblastoma multiforme tumor in a 5-year-old girl. The patient was initially treated with total removal of the tumor, without subsequent radiation therapy or chemotherapy. She was in good health for 3 years, but manifested a sudden onset of seizures, followed by severe headache, vomiting, and left-sided weakness. Cranial magnetic resonance imaging revealed a large mass with rim enhancement at the previously operated site. The mass was completely removed, and the pathology report revealed glioblastoma multiforme. Postoperative chemotherapy and radiation treatment were administered. She has been followed for 11 years, and is alive without recurrence.
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PMID:Malignant transformation of a desmoplastic infantile ganglioglioma. 2176 58

Ganglioglioma is one of the rare mixed neuronal glial tumors of the central nerve system. It is responsible for 0.4 - 2% of the intracranial tumors observed in infants and young matures. Its most common localization is the supratentorial region. Typically, the first symptom is epilepsy. Due to the glial structure, that rare tumor can exhibit a malign transformation. Growing slowly through several months or years, it forms neurological dysfunction. The standard treatment of that supratentorial tumor is usually total resection. If an anaplastic quality is observed, the patient undergoes radiotherapy after the surgical intervention. In this article, we presented a 53-year-old patient who presented with headache and dysphasia. The patient was operated for the cystic mass in the left parietal lobe reported as an abscess. The pathology was reported as ganglioglioma and we discussed the case according to the literature.
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PMID:Ganglioglioma mimicking the cerebral abscess in advanced age: a case report. 2410 Dec 83

Ganglioglioma is a rare central nervous system neoplasm representing 0.4% to 1.7% of all brain tumors and most frequently occurs in the pediatric population with an incidence of 7.6%. These tumors are usually slow-growing and well-circumscribed solid or cystic lesions. Gangliogliomatosis infrequently occurs in the frontal lobe, pineal gland, basal ganglia, hypothalamus, and optic chiasm, with very few reports of brainstem ganglioglioma. We report a case of a 35-year-old female who initially presented with headache, vertigo, ataxia, saccadic dysfunction, dysarthria, and dysmetria for several years due to an unknown etiology. Her brain imaging showed multiple lesions in the pons and the cerebellum with cystic changes and size reduction and enlargement over the next few years while her neurological symptoms continued to worsen. The patient received courses of steroid treatment that improved her neurological symptoms, suggesting an inflammatory component of her disease. Extensive workup for an inflammatory or infectious etiology was unfruitful and two brain biopsies were inconclusive. A third biopsy showed atypical glial nuclei, binucleated cells, and Rosenthal fibers and the presence of BRAF V600E mutation was detected. The diagnosis of gangliogliomatosis was consequently established. This case illustrates that gangliogliomatosis may present with the waxing-and-waning neurological signs and symptoms. It can masquerade inflammatory processes in the central nervous system on brain imaging and deserves careful consideration in the diagnosis of patients with an indolent course of neurological deterioration.
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PMID:An enigmatic brainstem posterior fossa ganglioglioma in an adult. 2440 63

In this retrospective study we evaluated the efficacy of interstitial brachytherapy (IBT) using (125)Iodine seeds ((125)I) for intracranial ganglioglioma WHO I. Between October 1994 and March 2010, eight patients (m/f = 5/3, median age 30.4 years, age range 7-42.5 years) with intracranial ganglioglioma WHO I were treated with IBT using stereotactically implanted (125)I seeds. The median follow-up time was 41.5 months (range 16.7-140.1 months). Prior to interstitial brachytherapy one patient underwent microsurgical resection for three times; seven patients were treated with IBT primarily. In all patients we implanted the (125)I seeds stereotactically guided. The cumulative tumor surface dose ranged between 50 and 65 Gy (permanent implantation) and the median tumor volume was 5.6 ml (range 0.9-26 ml). After brachytherapy, follow-up MR imaging revealed complete remission in one patient, partial remission in three and stable disease in the remaining four patients. Five of eight patients presented with seizures were either seizure-free (1/5) or improved (4/5). Temporary treatment-related morbidity occurred in one patient only (headache, nausea/vomiting) and resolved completely under steroid medication after 4 weeks. No treatment-related mortality was observed. This study indicates that interstitial brachytherapy for the treatment of intracranial ganglioglioma WHO I is safe and provides a high rate of local tumor control. Due to the limited number of cases, it is not possible to conduct a rigorous statistical evaluation. Thus, larger numbers of patients are required.
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PMID:Intracranial ganglioglioma WHO I: results in a series of eight patients treated with stereotactic interstitial brachytherapy. 2477 Dec 87


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