2 Hospital Physician Board Review Manual www.turner-white.com
Primary Brain Tumors
Jai Grewal, MD, Harpreet Kaur Grewal, MD, and Santosh Kesari, MD, PhD
Primary central nervous system tumors are relatively rare, but they can cause significant morbidity.
They are also among the most lethal of all neoplasms. Brain tumors are the second most common cause of death due to intracranial disease,
second only to stroke. The estimated annual incidence of primary brain tumors is approximately 21
per 100,000 individuals in the United States. 1 The
incidence of brain tumors varies by gender, age,
race, ethnicity, and geography and has increased
over time.2 Gliomas and germ cell tumors are more
common in men, whereas meningiomas are twice
as common in women. The only validated environmental risk factor for primary brain tumors is exposure to ionizing radiation. Other etiologies include
inherited cancer syndromes and primary central
nervous system lymphoma associated with AIDS. 3
Approximately 5% to 10% of gliomas are associated with a family history. 4 Inherited cancer syndromes involving brain tumors are listed in Table 1.
Primary brain tumors are classified according to
the World Health Organization 2007 classification, 5
as shown in Table 2. The TNM staging system is
not used for primary brain tumors. Primary brain
tumors are rarely metastatic outside the central
nervous system, with some exceptions (embryo-
nal tumors, malignant meningiomas). Tumors are
graded from I to IV based on histological grading
and prognosis, with grade IV being the most ag-
gressive. Notable features of selected nervous
system tumors are shown in Table 3. Gliomas
account for 78% of all primary malignant central
nervous system tumors, 1 and glioblastoma (WHO
grade IV) is the most common malignant primary
brain tumor in adults. Despite advances in the field
of oncology, the prognosis of these aggressive tu-
mors remains poor.
This review focuses on the evaluation and management of primary brain tumors, in particular glioblastoma (the most common malignant brain tumor
in adults), and highlights new advances in treatment
options. Management of common complications associated with brain tumors will also be discussed.
A 40-year-old right-handed nonsmoking
man presents to the emergency depart-
ment (ED) with the sudden onset of right-sided
weakness. His family reports that he has had
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