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24-09: Intracranial & Spinal Mass Lesions

Vanja C. Douglas; Michael J. Aminoff

Key Clinical Updates in Intracranial & Spinal Mass Lesions

In patients with glioblastoma with methylated methylguanine- DNA methyltransferase (MGMT) promoter, combination therapy with lomustine and temozolomide improved median survival from 31 to 48 months in a randomized controlled trial.

1. PRIMARY INTRACRANIAL TUMORS

ESSENTIALS OF DIAGNOSIS

  • Generalized or focal disturbance of cerebral function, or both.

  • Increased intracranial pressure in some patients.

  • Neuroradiologic evidence of space-occupying lesion.

General Considerations

Roughly one-third of all primary intracranial neoplasms (Table 24–4) are meningiomas, one-quarter are gliomas, and the remainder are pituitary adenomas (see Chapter 26), neurofibromas, and other tumors. Certain tumors, especially neurofibromas, hemangioblastomas, and retinoblastomas, may have a familial basis, and congenital factors bear on the development of craniopharyngiomas. Tumors may occur at any age, but certain gliomas show particular age predilections.

Table 24–4.Primary intracranial tumors (listed by major histology grouping and by incidence within each group).
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Table 24–4. Primary intracranial tumors (listed by major histology grouping and by incidence within each group).
Tumor Clinical Features Treatment and Prognosis
Tumors of meninges
Meningioma Originates from the dura mater or arachnoid; compresses rather than invades adjacent neural structures. Increasingly common with advancing age. Tumor size varies greatly. Symptoms vary with tumor site—eg, unilateral proptosis (sphenoidal ridge); anosmia and optic nerve compression (olfactory groove). Tumor is usually benign and readily detected by CT scanning; may lead to calcification and bone erosion visible on plain radiographs of skull. Treatment is surgical. Tumor may recur if removal is incomplete.
Tumors of neuroepithelial origin
Glioblastoma multiforme Presents commonly with nonspecific complaints and increased intracranial pressure. As it grows, focal deficits develop. O6-methylguanine-DNA methyltransferase promoter methylation positivity (seen in 40% of cases) and isocitrate dehydrogenase 1/2 mutations (seen in 10% of cases) carry better prognosis. Course is rapidly progressive, with poor prognosis (< 20% survival at 2 years). Total surgical removal is usually not possible. Radiation therapy and temozolamide may prolong survival. Tumor treatment fields added to temozolamide after completion of radiation therapy prolong survival.
Astrocytoma Presentation similar to glioblastoma multiforme but course more protracted, often over several years. Cerebellar astrocytoma may have a more benign course. Isocitrate dehydrogenase 1/2 mutations (seen in a majority of cases) carry better prognosis in grade II and III tumors. Prognosis is variable. By the time of diagnosis, total excision is usually impossible; tumor may be radiosensitive and temozolamide is also helpful in grade II and III tumors. In cerebellar astrocytoma, total surgical removal is often possible.
Ependymoma Glioma arising from the ependyma of a ventricle, especially the fourth ventricle; leads to early signs of increased intracranial pressure. Arises also from central canal of cord. Tumor is best treated surgically if possible. Radiation therapy may be used for residual tumor.
Oligodendroglioma Slow-growing. Usually arises in cerebral hemisphere in adults. Calcification may be visible on skull radiograph. Co-deletion ...

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