Astroblastoma

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Astroblastoma
Astroblastoma1.jpg
Axial T1 with contrast showing left frontal Astroblastoma
Tumor Class Astrocytoma
Cell of origin Astrocyte
WHO Grade Not defined
Survival unknown, thought to be less than one year
Treatment Surgery, Chemotherapy, Radiation
Histologic Features
Radiographic Features

Contents

Definition

Astroblastoma is a rare glioma of young adulthood, histologhically characterized by a typical perivascular pattern of GFAP-positive neoplastic astrocytes with broad, non-tapering processes radiating toward a central blood vessel. Astroblastoma has yet to be assigned a WHO grade.

Epidemiology

Reliable epidemiological data regarding astroblastoma are not available. These lesions are most common in young adults, but may occur in infants and children.

Localization

The cerebral hemispheres are most often affected, but these lesions may arise in the corpus callosum, cerebellum, optic nerves, brainstem, or cauda equina.

Clinical presentation

Patients most commonly present with focal neurological deficit consistent with the location of the tumor or with evidence of increased intracranial pressure.

Imaging

On MRI, astroblastomas are well-defined, cystic lesions with an enhancing mural component.

Macroscopy

Astroblastomas are well-circumscribed, solid lesions with a well-defined border with normal brain.

Histopathology

Astroblastoma is a homogeneous glial neoplasm characterized by a perivascular pseudorosette pattern of GFAP-positive neoplastic astrocytes with broad, non-tapering processes radiating toward a central blood vessel. These lesions do not contain foci of conventional, infiltrative, fibrillary or gemistocytic astrocytoma or ependymoma.

Molecular genetics

The molecular genetics of astroblastoma have not yet been elucidated, though this lesion has been reported to occur in the presence of LOH on chromosome 9p.

Prognosis

The prognosis of astroblastoma has not been characterized, though the presence of high-grade features is thought to be a poor prognostic finding.

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