1/18. spinal cord ganglioglioma in a child with neurofibromatosis type 2. Case report and literature review.Gangliogliomas of the spinal cord are rare disease entities that occur in early childhood. Their occurrence in association with neurofibromatosis Type 2 (NF2) has not been described. The authors describe the unique case of a 2-year-old child with stigmata of NF2 who harbored a spinal cord ganglioglioma that presented as a rapidly growing, exophytic intramedullary mass lesion at the cervicomedullary junction. Treatment consisted of complete surgical resection. Histopathological analysis of the lesion demonstrated a mixed population of neoplastic cells, of both neuronal and glial lineage, that supported the diagnosis of ganglioglioma.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/18. Extracranial metastasis of anaplastic ganglioglioma through a ventriculoperitoneal shunt: a case report.Extracranial metastasis of an intracranial anaplastic ganglioglioma through a ventriculoperitoneal shunt is reported. A 53-year-old woman was treated by surgery, radiation and chemotherapy and died 2 years later. At autopsy, multiple metastatic lesions were found in the spinal cord, and the abdominal and pleural cavities. Histologically, all the metastatic lesions were composed of atypical cells which resembled primitive glial elements found in intracranial anaplastic ganglioglioma, suggesting that anaplastic glial elements have a metastatic potential. Extracranial metastasis of ganglioglioma is a rare occurrence; however, the spread of glial elements through the shunt further suggests that caution is required in therapy and indicates a need for protective filters in the shunt system.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
3/18. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
4/18. Intramedullary spinal cord ganglioglioma: a report of five cases.We report five cases of ganglioglioma in various locations. The tumours were removed with gross total resection in most cases without any further neurological deterioration. Without any additional treatment, no evidence of recurrence or regrowth of tumours was observed in the mean follow-up period of 4.1 years. We suggest that intramedullary spinal cord gangliogliomas be resected totally due to the high risk of recurrence and regrowth rate of the tumours with subtotal resection in the long term considering especially that these tumours affect predominantly young patients.- - - - - - - - - - ranking = 2.5keywords = spinal (Clic here for more details about this article) |
5/18. Malignant transformation of a spinal cord ganglioglioma--case report and review of the literature.Gangliogliomas are tumors of mixed glial and neuronal phenotype that usually have a benign clinical course. Rare cases display anaplastic features at the time of first presentation or progress to anaplastic gliomas over extended times. We report on a ganglioglioma of the spinal cord that recurred as a malignant glioma one and a half years after resection. The initial neoplasm was composed of a mixture of well-differentiated ganglionic and astrocytic cells. The recurrent tumor was an anaplastic small-cell glioma. The sole unusual aspect in the initial neoplasm was an abundance of small vessels with calcified walls, which mimicked a vascular malformation.- - - - - - - - - - ranking = 2.5keywords = spinal (Clic here for more details about this article) |
6/18. Intramedullary spinal cord ganglioglioma presenting with abnormal abdominal wall movement. Case report.The authors present a case of intramedullary ganglioglioma in a 6-year-old girl. Since the age of 4 months the patient had experienced a spontaneous wavy undulating movement of her anterior abdominal wall resembling a severe peristalsis. The movement was continuous even during sleep, and this symptom was named "belly dance." Magnetic resonance images revealed an intramedullary tumor with ill-defined borders, and the lesion was partially resected. The patient made a good recovery, although 4 years postsurgery her scoliosis had progressed.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
7/18. spinal cord ganglioglioma presenting as acute paraparesis.A 17-year-old male presented with acute onset paraparesis in the lower limbs. urinary retention was present and the patient required catheterisation. Clinical examination confirmed severe bilateral lower limb weakness and a sensory level at T8. magnetic resonance imaging (MRI) revealed a haemorrhagic intramedullary tumour extending from T8 to the conus. Microsurgical excision of the tumour was performed and the patient made a good functional recovery. The histology of the tumour demonstrated a ganglioglioma of the spinal cord. Acute paraparesis has not previously been reported with a spinal cord ganglioglioma. We discuss the clinical, diagnostic and pathological features of spinal cord gangliogliomas.- - - - - - - - - - ranking = 1.5keywords = spinal (Clic here for more details about this article) |
8/18. Desmoplastic infantile ganglioglioma: a questionably benign tumour.Desmoplastic infantile ganglioglioma is a rare intracranial tumour of childhood that involves the cerebral cortex and the leptomeninges. We report two patients with desmoplastic infantile gangliogliomas and multiple cerebrospinal metastases. To our knowledge, only two similar cases have been reported in the published literature. Pathologically, this rare intracranial tumour shows glial and ganglionic differentiation, accompanied by an extreme desmoplastic reaction. These are low-grade neoplasms that are questionably benign.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
9/18. Case of desmoplastic infantile ganglioglioma secreting ceruloplasmin.Desmoplastic infantile ganglioglioma is a rare world health organization (WHO) grade I tumor commonly arising in early infancy and usually presenting with both solid and cystic components. We report a case of a large midline-enhancing desmoplastic infantile ganglioglioma in which newly formed cysts in communication with lateral ventricles contained highly proteinaceous fluid. Proteomic analysis of the fluid showed three proteins not normally found in cerebrospinal fluid. Immunohistochemical analysis of the tumor sample showed that the desmoplastic infantile ganglioglioma produced a high concentration of ceruloplasmin, which probably accounts for most of the 30- to 40-fold increase in protein compared with normal cerebrospinal fluid. To our knowledge, this is the first report of ceruloplasmin secretion by a brain tumor, and ongoing studies on the mechanism might yield novel approaches to reducing cyst production and protein content in an otherwise stable solid tumor.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
10/18. Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease).Dysplastic gangliocytoma of the cerebellum is a rare disorder requiring surgical resection. During surgical exploration, the margin with normal cerebellar tissue is often not distinct. We present here a case in which we surgically treated a patient with Lhermitte-Duclos disease, and discuss radical resection of the tumour. The patient was first admitted at the age of 34 years. A MRI scan revealed a large left cerebellar mass typical of dysplastic gangliocytoma. Postoperatively the patient developed severe cerebellar syndrome with only partial recovery after 4 years. MRI revealed no impairment of cerebrospinal fluid circulation; the mass in the left cerebellum had been removed, and no recurrent disease was visible. Clinical problems after gross total or complete removal of Lhermitte-Duclos lesions have not been reported in the literature, but herein we stress the importance of extreme caution in removing these lesions in cerebellar areas that have no distinct border between the tumour and normal tissue.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
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