Desmoplastic infantile astrocytoma/ganglioglioma
Synonyms: DIA/DIG
Desmoplastic infantile astrocytoma/ganglioglioma are mixed neuronal-glial tumors representing a histological spectrum of the same tumor. They are usually supratentorially located large cystic masses with a peripheral solid component characterized by prominent desmoplastic stroma and pleomorphic populations of neoplastic cells with either astrocytic or ganglionic differentiation and poorly differentiated cells in variable proportions. They usually present in the first 18 months of age with rapid head growth bulging anterior fontanel and bone structures over the tumor signs of raised intracranial pressure (headache vomiting papilledema) focal neurological signs and sometimes seizures.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
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Desmoplastic infantile astrocytoma/ganglioglioma?
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