Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Desmoplastic infantile ganglioglioma is a rare intracranial tumor of early childhood with a usually excellent prognosis despite malignant features both radiologically and histologically. We present the case of a desmoplastic infantile ganglioglioma with histologically highly anaplastic features and both intracerebral and pial metastases. After partial resection the tumor was rapidly progressive and new metastases appeared. A combination of vincristine and carboplatinum was used according to the Low Grade Glioma Protocol of the International Society of Pediatric Oncology, with a temporary good response. When histologically characterized by highly anaplastic features, it seems the biologic behavior of this tumor remains uncertain. The aggressive behavior and the responsiveness to chemotherapy in this case may challenge the belief in the benign nature of these rare tumors.
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PMID:Desmoplastic infantile ganglioglioma: a potentially malignant tumor? 1240 29

Desmoplastic infantile ganglioglioma is a supratentorial tumor that typically occurs in infants below the age of 24 months. Rare tumors with the same radiological and histological characteristics have been described in older subjects. We report a case of desmoplastic ganglioglioma in a 12-year-old girl with a 13 years follow-up. The patient presented with an inaugural generalized seizure. CT scan demonstrated a large superficial parieto-occipital mass, attached to the dura with solid and cystic components. Surgical resection was macroscopically complete. No adjuvant treatment was given. Thirteen years after surgery, the patient is symptom free. Histological examination revealed a pleomorphic tumor involving the meningeal space and the cortex. Meningeal portion was made of neoplastic astrocytes enmeshed in a dense network of connective tissue. The cortical component showed abnormal neurons, tumoral astrocytes and small foci of poorly differentiated cells with rare mitoses. Our observation and the seven others found in the literature indicate that desmoplastic gangliogliomas can occur in children and even in young adults. Despite some worrisome radiological and histological features, these tumors should not be misdiagnosed as malignant glioma. Like infantile cases, non-infantile desmoplastic gangliogliomas seem to have a favorable prognosis without additional therapy, if a total surgical resection can be performed.
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PMID:A report of a desmoplastic ganglioglioma in a 12-year-old girl with review of the literature. 1620 62

Desmoplastic infantile ganglioglioma is a rare intracranial tumor of infancy, characterized by solid and cystic component, voluminous size and supratentorial location. These tumors are diagnosed usually below the age of 2 years. We report 1 case of desmoplastic ganglioglioma in 13-year-old male. Computed tomography and magnetic resonance imaging diagnosed supratentorial mixed cystic and solid tumor, which presented as a large cystic component with intense contrast enhancement of a mural nodule. The tumor was surgically removed, and histology revealed desmoplastic ganglioglioma. The patient had a good follow up. This observation emphasizes the possibility of desmoplastic ganglioglioma in older infants. It mustn't be considered as a specific entity of very young age infant and must be recognized in older infant because it may be misdiagnosed as malignant glioma. Despite the pseudo malignant appearance, these tumors have a good prognosis after surgery and when excision is complete they don't led to recurrences.
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PMID:[Magnetic resonance imaging features of desmoplastic cerebral ganglioglioma of infancy: report of 1 case]. 1636 14