Giant Cell Glioblastoma

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Definition

Giant cell glioblastoma is a histological variant of glioblastoma characterized by bizarre, multi-nucleated giant cells, an abundant stromal reticulin network, and a high frequency of TP53 (p53) mutations. Giant cell glioblastoma is considered a WHO Grade IV astrocytoma.

Epidemiology

Giant cell glioblastoma accounts for about 5% of all GBMs. The mean age of occurrence is 42 years. There is a male predominance (1.6:1).

Clinical presentation

Giant cell glioblastomas are de novo lesions with symptoms at presentation similar to those of GBM.

Imaging/Macroscopy

Because of their abundant stromal component , these lesions may appear both by imaging and surgery to be well-encapsulated lesions, and are often mistaken for a metastasis.

Histopathology

Giant cell glioblastoma is composed of numerous bizarre, multi-nucleated giant cells and small fusiform syncytial cells within an abundant stromal reticulin network.

Molecular genetics

TP53 mutations are present in nearly 75-90% of these lesions. PTEN mutations are present in a third of these tumors. Giant cell glioblastomas typically lack EGFR amplification/overexpression or LOH of p16.

Prognosis

Mean survival time following surgical intervention appears to be somewhat longer than that for other variants of GBM, possibly because of a less infiltrative behavior.