Treatment and outcomes for gangliogliomas brain tumor. While a subset of gangliogliomas are known to harbor the activating p. Ganglioglioma genetic and rare diseases information. Pdf fortytwo patients with lowgrade brain tumor and refractory epilepsy were studied. A rare presentation of a ganglioglioma with welldefined benign and malignant areas in the same patient provided an excellent opportunity to query whether there is a clonal progression of the tumor to a more malignant form and to further identify the potential genes involved in this progression. A case report of rare location of ganglioglioma egyptian. Focal or diffuse nonenhancing white matter wm mass cerebral hemispheres, supratentorial 23. Successful treatment with dabrafenib gsk2118436 in a patient with ganglioglioma. Ganglioglioma occurs most commonly in the cerebrum the part of the brain that.
Generally, they are benign growths that occur over a long clinical course and present with no specific radiologic characteristics. A focal area of cortical dysplasia is also identified. However, in some instances, the patients seizures can be attributed to a number of identifiable morphologic abnormalities including hippocampal sclerosis mesial temporal. Ganglioglioma with a glioblastomatous component and highgrade atypia of neuronal cells are extremely rare findings. It is a type of brain tumor that contains properties of both glial cells responsible for providing the structural support of the central nervous system and neuronal cells the functioning component of the central nervous system. Gangliogliomas occur when a single cell in the brain starts to divide into more cells, forming a tumor. This is only one of a few such cases observed thus far and contributes to our understanding of the characteristics of this rare posterior fossa tumor. Gangliogliomas may occur anywhere in the central nervous system but are not encountered commonly. This tumor can affect people of any age but most often shows up between the ages of 30 and 50.
Some of the worlds best neuroscientists, cancer biologists and doctors from the st. These cystic gangliogliomas were located, in order of decreas. Between 1998 and 2012, 16 patients, including 11 men and 5 women, with a median age of 12. Cytopathology of cns tumours is dealt with in the article cns cytopathology there are separate articles for peripheral nerve sheath tumours and pituitaryperipituitary lesions. Net s guide to childhood desmoplastic infantile ganglioglioma. Gangliogliomas are generally benign tumors, composed of transformed neuronal and glial elements, with. Ganglioglioma is a rare and slowly growing benign tumor. Gangliogliomas are rare neoplasms, with an incidence of only 1. Ganglioglioma treatment danafarberboston childrens. In 1930, courville defined the term ganglioglioma as tumors consisting of astrocytic neuronal components with rare mitotic figures see figure 8. Ganglioglioma is a rare, slowgrowing primary central nervous system cns tumor which most frequently occurs in the temporal lobes of children and young adults. Response to contemporary therapy is individual to each tumor type and malignancy grade. Pdf optic pathway ganglioglioma with intraventricular.
Ganglioglioma is a very rare brain tumor that affects the function of the central nervous system. Ganglioglioma arising from desmoplastic medulloblastoma. Center of brain tumor, beijing institute for brain disorders, beijing, pr china. For most childhood brain and spinal cord tumors, the optimal treatment regimen has not been determined. It accounts for approximately 1 percent of all brain tumors. Most previous studies on brain tumor and epilepsy analyzed patients with different lowgrade tu. Desmoplastic infantile ganglioglioma, childhood tumor. There is no current study that identifies fourth ventricular ganglioglioma with mixed features of the more common, but overall rare neurocytoma. They can occur anywhere in the central nervous system but are most commonly located in the temporal lobe and are mainly found in children. Gg is frequently found in patients younger than 30 years 80% with refractory epilepsy. Natural history of spinal ganglioglioma in neurofibromatosis type 1. Children who have brain and spinal cord tumors should be considered for enrollment in a clinical trial when an appropriate study is available. Glioneuronal tumor with features of ganglioglioma and.
Jude neurobiology and brain tumor divisions combine their efforts to seek cures. Ganglioglioma and ganglion cell tumors springerlink. Since the tumor extended from the right optic nerve to the right geniculate body, it was diagnosed as an optic glioma before operation. We report a case of a hypothalamic chiasmatic ganglioglioma in a 21yearold woman who presented with hyperprolactinemia and developed visual field defects. There has muchliterature in recent years examining the pathology of chronic, medically intractable epilepsy. Brain tumor basics living creatures are made up of cells. Gangliocytomas are rare indolent cns tumors who grade i, primarily encountered in children, and frequently discovered as the cause of epilepsy. Childhood brain and spinal cord tumors treatment overview. Primary brain tumor of astrocytic origin with intrinsic tendency for malignant progression, degeneration into anaplastic astrocytoma aa. Brain tumor syllabus american society of neuroimaging. Gangliogliomas are rare tumors of the central nervous system. Although rare, gangliogliomas should be included in the differential diagnosis of lesions occurring. Mr findings jong won kwon, inone kim, jungeun cheon, woo sun kim, je geun chi, kyuchang wang, and kyung mo yeon summary.
Therefore, there are a limited number of patients and data involving the use or role of adjuvant therapy after subtotal resections strs of gangliogliomas. Gangliogliomas are rare tumors occurring in both children and. We present a case of cerebellopontine cp angle ganglioglioma in a young child with developmental delay and no trigeminal nerve symptoms. Gangliogliomas are generally benign who grade i tumors. I are rare tumors of the central nervous system cns that account for approximately 1% of all cns. Ganglioglioma was first described by courville in 1930 as a central nervous system neoplasm containing both astrocytic and neuronal components. The mri image shows a ganglioglioma in the right temporal lobe arrow. We investigated the outcomes of patients who underwent different combination treatments. Ganglioglioma is usually considered as a lowgrade tumor with the negligible prospect to spread to other parts, but in the rare instance, it can transform to highgrade tumor having to expandability. They differ from gangliogliomas by the absence of neoplastic glial cells, although both tumors are defined by the presence of displaced ganglion cells large mature neurons that show cytological or architectural abnormalities. Ganglioglioma gg is one of the commonest causes of tumorrelated refractory epilepsy in young patients 1, and together with other lowgrade brain neoplasm s, comprises 1030% of the pathological substrate in patients with chronic intractable partial epilepsy 2. Ganglioglioma and gangliocytomas together comprise 0.
The tumor is often slow growing and noncancerous benign. Ganglioglioma comparison with other lowgrade brain. Cemri of the brain was done 14 and 24 months after surgery which showed post radiotherapy changes with gliosis with no recurrent lesion fig. Overall, mixed features of ganglioglioma and neurocytoma were appreciated in both cases and the diagnosis of glioneuronal tumor with mixed features of ganglioglioma and neurocytoma was rendered.
A case of adult anaplastic cerebellar ganglioglioma. This can occur when the cell randomly acquires changes mutations in genes that regulate how a cell divides. Epilepsy is a common clinical presentation and this tumor has a typical occurrence in the temporal lobes, although they have been described in all parts of the central nervous system. Analysis of braf v600e mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extracerebellar pilocytic astrocytoma. Gangliogliomas comprise less than 1% of all brain tumors and occur most often in children. The adult body normally forms new cells only when they. The desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy are rare neoplasms arising in the cerebral hemispheres within the first two years of life which are defined by a combination of distinctive clinicopathologic features. Pdf ganglioglioma comparison with other lowgrade brain tumors. Unfortunately, it is a rapidly growing, destructive tumor that may lead to death within months. The treatment for hemangioblastoma is surgical excision of the tumor. A ganglioglioma is a rare type of brain tumor, accounting for approximately 1% of all brain tumors. On a scan, hemangioblastoma shows as a welldefined, low attenuation region in the posterior fossa with an enhancing nodule on the wall.
The primary diagnosis is made with a computed tomography scan ct scan. This circumscribed cystic lesion with an enhancing mural nodule was radiologically indistinguishable from a pilocytic astrocytoma. Gangliogliomas are rare benign mixed neuronal and glial tumors of the brain, likely arising from transformation of a. Gangliogliomas in adults american cancer society journals. Such clinical trials are carried out by institutions and cooperative groups. However, optic nerve biopsy showed that the lesion was a. Jude brain tumor program has made great advances in treatments for brain tumor patients. In many of these cases, the etiology or morphologic basis for seizures continues to be elusive. A paraganglioma is a rare tumor that begins in certain nerve cells that are dispersed throughout the body. We describe a case of medulloblastoma maturating into ganglioglioma during therapy. A 10monthold boy was diagnosed with a desmoplastic medulloblastoma and was treated with gross total resection followed by induction chemotherapy.
Case report natural history of spinal ganglioglioma in. In this interactive program, discoveries about the biology of both normal and. Most gangliogliomas grow slowly and are considered benign. Discussion ganglioglioma is an infrequent tumor of the central nervous system, initially characterized by perkins in 1926. The free booklet is available as a pdf, so it is easy to print out. The patient is asymptomatic 26months after surgery. The tumor causes muscle spasms, sharp pain, and numbness in the legs, arms, feet, and. Fortytwo patients with lowgrade brain tumor and refractory epilepsy were studied.
Gangliogliomas are uncommon, usually lowgrade, cns tumors. V600e mutation in the braf oncogene, the genetic alterations responsible for the remainder are largely unknown, as is the spectrum of any additional cooperating gene mutations or copy number. Successful treatment with dabrafenib gsk2118436 in a. The pdq childhood brain tumor treatment summaries are organized primarily according to the who classification of nervous system tumors. The resection histologically demonstrated distinct areas of ganglioglioma and dysembryoplastic neuroepithelial tumor. The malignancy grade is an estimation of the degree of malignancy usually encountered in each type of tumor, where grade i is the least and grade iv the most malignant. We report the case of keywords ganglioglioma optic pathway suprasellar a 6yearold boy with a large, partially cystic, suprasellar tumor endoscopy hydrocephalus tumor originating in the chiasm and bilaterally involv ing the entire pregeniculate optic pathway, that was identi fied as a ganglioglioma by histopathological evaluation. Cerebrum, the motor and sensory action controlling brain part mostly affected with ganglioglioma 1,2. A ganglioglioma is rare brain tumor that commonly causes seizures. After multiple surgeries it is now very hard to remove the tumor without causing significant damage. A recurrence in the tumor bed during therapy was managed with focal radiation therapy and consolidation chemotherapy. Gangliogliomas world health organization who grade. This article reports a case of a composite ganglioglioma and dysembryoplastic neuroepithelial tumor occurring in a 36yearold woman in the left temporal lobe region.
A case of ganglioglioma of the optic pathway associated with congenital exophthalmos and strabismus is presented. In my case, this tumor is located on the lower spinal cord. Hematoxylineosin stained slides revealed a complex tumor with features of glioblastoma and marked atypia of neuronal. The combination of the aforementioned imaging findings with the cytohistomorphological and immunohistochemical findings are consistent withlead to the diagnosis of a desmoplastic noninfantile ganglioglioma. About brain tumorste brain tumor uide a primer for patients and caregiversfor newly diagnosed patients and their families about brain tumors 7 chapter 1. Under the microscope, the tumor has the features of an anaplastic astrocytoma with the addition of areas of dead tissue necrosis. Brainstem gangliogliomas usually become symptomatic in the first and second decades of life and involve the medulla and pons. Ganglioglioma comparison with other lowgrade brain tumors paulo thadeu brainerlima1, alessandra mertens brainerlima2, hildo rocha azevedofilho3 abstract method. Using precise microsurgical techniques and aided by intraoperative mri, an imagecomplete resection was successfully accomplished and the patient had fewer seizures. The present work demonstrates that anaplastic ganglioglioma need to be considered in the differential diagnosis of malignant primary infratentorial brain tumors in adult patients. Low grade ganglioglioma rapidly progressing to a who grade iv tumor showing malignant transformation in both astroglial and neuronal cell components. Ganglioglioma is an uncommon neoplasm of the central nervous system, most frequently seen in the temporal lobe, and usually associated with medically refractory epilepsy in children and young adults.
721 167 1316 130 55 1207 1229 1294 578 1262 1432 1268 986 1463 771 1248 814 907 218 816 1144 934 1300 673 1244 1345 1294 632 170 1380 377 474 501 1413 657 67 600 200 134 129 1367 236 1398