Possible efficacy of temozolomide in a patient with gliomatosis cerebri

Gliomatosis cerebri is a rare primary brain tumor characterized by the proliferation of neoplastic glial cells that typically involve multiple brain areas. The morphology of tumor cells is diverse, taking on the appearance of astrocytes, oligodendrocytes, or Schwann cells with variable mitotic activity. A hallmark of this disease is the preservation of brain structures with the sparing of neurons. Clinical manifestations are not specific, including headache, seizures, visual disturbances, corticospinal tract deficits, dementia, and lethargy.1 Before the era of MRI, this diagnosis was generally established at autopsy or in rare patients undergoing several brain biopsies. With the current usage of MRI showing diffuse, poorly circumscribed T2-weighted lesions with swelling of involved areas, gliomatosis cerebri is now more often considered. The prognosis of this devastating neoplasm is generally poor, with a median survival time of approximately 12 months. Surgery is unrealistic considering the extent of the disease, standard chemotherapy (nitrosourea) is unsuccessful, and although brain irradiation can stabilize or improve neurologic function in some patients, its impact on survival has not been demonstrated.2 New approaches are warranted. In …