Neuroradiology Case of the Week

Case 1

Matthew Cham, MD, Manoj Ketkar, MD, and P-L Westesson, MD, PhD, DDS

Clinical Presentation: A 17-year-old male presented with new symptoms of headache, difficulty swallowing, drooling and left-sided weakness.

Radiologic Findings: There is diffuse increased T2 and FLAIR signal involving nearly the entire right hemisphere (Figs. 1-3) without any significant enhancement. There are post-surgical changes in the temporal lobe on the right side (Fig. 2). There is no midline shift. The cortical sulci and ventricular systems are prominent for the age of seventeen. Spectroscopy (Fig. 4) demonstrates marked elevation of choline.

Figure 1	Figure 2
Figure 3		Figure 4

Diagnosis: Gliomatosis cerebri

Discussion: Gliomatosis cerebri is a rare primary brain tumor characterized by diffuse infiltration of the brain with 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. It represents an extreme form of diffusely infiltrating glioma [1,2]. It is difficult to distinguish gliomatosis cerebri from highly infiltrate anaplastic astrocytoma or GBM. This patient had a biopsy that confirmed the diagnosis of gliomatosis cerebri.
    It can occur at any age but is usually found in the third and fourth decades of life [3]. It may affect any part of the brain or even the spinal cord, optic nerve and compact white matter.
   Clinical manifestations are non-specific, and include headache, seizures, visual disturbances, corticospinal tract deficits, lethargy, and dementia [4].
   Before the advent of MRI imaging, diagnosis was generally not established until autopsy. On MRI, it typically appears as a diffuse, poorly circumscribed, infiltrating non-enhancing lesion that is hyperintense on T2-weighted images and expands the cerebral white matter.
   The prognosis for gliomatosis cerebri is generally poor, with a median survival time of only 12 months. Surgery is not practical considering the extent of the disease, standard chemotherapy (nitrosourea) has been unsuccessful, and although brain irradiation can stabilize or improve neurologic function in some patients, its impact on survival has yet to be proven [5].

References:

1. Nevin S. Gliomatosis cerebri. Brain 1938;61:170-191.
2. Mineura K, Sasjima T, Kowada M et al: Innovative approach in the diagnosis of gliomatosis cerebri using carbon-11-L-methionine position emission tomography, J Nuc Med 1991;32:726-728.
3. Koslow SA, Claassen D, Hirsch WL, Jungreis CA: Gliomatosis cerebr: a case report with autopsy correlation. Neuroradiol 1992;34:331-333.
4. Kim DG, Yang HJ, Park IA, et al. Gliomatosis cerebri: clinical features, treatment, and prognosis. Acta Neurochir 1998; 140: 755–762.
5. Cozad SC, Townsend P, Morantz RA, Jenny AB, Kepes JJ, Smalley SR. Gliomatosis cerebri: results with radiation therapy. Cancer 1996; 78: 1789–1793.