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INTRODUCTION

The focus of this chapter is to provide you with a general overview of tumors involving the nervous system. It will be important to know their prognosis and treatment options.

Also, keep in mind some of the “buzz-words” that describe histopathological hallmarks of each type of tumor.

PRIMARY CNS TUMORS

Metastatic brain tumors are 10 times more common than primary malignant brain tumors and therefore the most common brain tumor overall.

Pilocytic Astrocytoma (PA)

  • Patients: more common in childhood

  • Location: cerebellum, optic chiasm (associated with NF1), hypothalamus, periventricular

  • Imaging: enhancing mural nodule with a cystic component

  • Microscopy: pilocytic elongated cells, Rosenthal fibers (Fig. 12-1)

  • Treatment: surgical resection

image   KEY FACT

Optic glioma is a grade 1 PA that arises from glial cells of optic pathway. Surgical resection if symptomatic; radiotherapy if unresectable. Patients will continue to have visual impairment.

FIGURE 12-1.

Rosenthal fibers seen in Pilocytic Astrocytoma. Reproduced with permission from Kemp WL, Burns DK, Brow TG: Pathology: The Big Picture. New York, NY: McGraw Hill; 2008.

Glioblastoma Multiforme

  • Patients: adults (peak incidence—64-year-old)

  • Location: cerebral hemispheres

  • Imaging: heterogeneously enhancing lesion with vasogenic edema and necrotic core; may cross midline (butterfly glioma)

  • Microscopy: pseudopalisading with necrosis

  • Treatment: radiation and chemotherapy (temozolomide), resection to delay disease progression

image   WARDS TIP

GBM is a fast growing and invasive brain tumor. More common in men, survival is less than 20% after 2 years.

Oligodendroglioma

  • Patients: adults (around age 40)

  • Location: frontal lobes (seizures, personality change)

  • Imaging: variable

  • Microscopy: “fried egg” appearance with clear cytoplasm (Fig. 12-2)

  • Treatment: resection and radiation

image   WARDS TIP

GBM is the most common form of malignant primary brain tumor.

FIGURE 12-2.

“Fried egg” appearance with clear cytoplasm seen in oligodendroglioma. (Used with permission from Dr. Stephen Cohle, Spectrum Health-Blodgett Campus, Grand Rapids, MI.)

Ependymoma

  • Patients: more common in children, associated with NF2

  • Location: posterior fossa/ventricular system (children), spinal cord (adults) arises from ependymal lining of ventricles

  • Imaging:

    • Intracranial tumors—heterogeneous, slightly enhancing

    • Spinal tumors—homogeneous, brightly enhancing

  • Microscopy: perivascular pseudorosettes, GFAP+ (Fig. 12-3)

  • Treatment: resection

image   WARDS TIP

If ependymoma affects affecting ventricular system, expect noncommunicating hydrocephalus.

image   WARDS TIP

Von Hippel-Lindau syndrome is an autosomal dominant cancer syndrome associated with cerebellar and spinal cord hemangioblastomas, renal cell carcinoma, pheochromocytoma, and retinal angiomas.

FIGURE 12-3.

Perivascular pseudorosettes seen in Ependymoma. WebPathology

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