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Brain tumors

Last updated: September 22, 2021

Summarytoggle arrow icon

Brain tumors are masses of abnormal cells within the brain. They can be primary or metastatic, benign or malignant. Common tumors in children are pilocytic astrocytomas, medulloblastomas, ependymomas, and craniopharyngiomas. Adults most often develop glioblastoma multiforme, meningiomas, hemangioblastomas, schwannomas, oligodendrogliomas, and pituitary adenomas. Clinical features and radiological findings vary according to the type, location, and onset of the tumor. Magnetic resonance imaging (MRI) is the primary diagnostic method. Removal of the entire tumor is a prerequisite for remission. The histological grade of the tumor, which is determined postoperatively, is an important factor in determining the prognosis. Malignant tumors usually require additional treatment with radiotherapy and/or chemotherapy.

Meningiomas, pituitary adenomas, and schwannomas are discussed in separate articles.

Overview

Primary brain tumors arise within the CNS and are classified based on the growth characteristics cell type from which the tumor arises.

By the spreading potential

Types of brain tumors by growth characteristics
Low-grade brain tumors High-grade brain tumors
WHO grade I–II III–IV
Borders Well-defined Poorly-defined
Spreading potential Low High
Recurrence Rare Frequent

By the cell type

Epidemiology

  • Approx. 30% of brain tumors are primary brain tumors, while approx. 70% are metastases.
  • Approx. 40% of primary brain tumors are benign.
  • Sex: > (except meningiomas, which occur more frequently in women) [1]

Clinical features

Symptoms depend on the type and location of the tumor (see “Primary brain tumors according to age” below). Onset is usually insidious.

Metastasis

Overview

Astrocytomas are neuroepithelial tumors (gliomas) that arise from astrocytes, which are a specific type of glial cell.

Classification of astrocytomas
Grade Type Description Epidemiology Location Prognosis
I Pilocytic astrocytoma
  • Predominantly in children and young adults (< 20 years)
  • Favorable; long-term survival
  • Completely curable with complete surgical resection
  • Median survival: > 10 years
II Diffuse astrocytoma
  • Slow growing, low-grade tumor that has the potential to progress to higher-grade tumors
  • Peak age: 20–40 years of age
  • Cerebral hemisphere
  • Incurable
  • Median survival: 2–12 years
III Anaplastic astrocytoma [3][4]
  • Peak age: 30–50 years of age
  • Incurable
  • Median survival between 18 months and 10 years
IV Glioblastoma multiforme (GBM) [5][6][7]
  • Malignant tumor with rapid growth, abrupt symptom onset, and short disease course (death within weeks)
  • Peak age: 60–70 years of age
  • Most common malignant primary brain tumor
  • Incurable
  • Median survival of 15 months

Pilocytic astrocytoma [8][9][10][11]

  • Description: slow-growing, circumscribed, non-invasive tumor
  • Epidemiology
    • Most commonly affects children and young adults (< 20 years)
    • The most common primary brain tumor of childhood
  • Associated conditions: neurofibromatosis type I
  • Typical location
  • Clinical features
  • Diagnostics
    • MRI findings
      • Well-demarcated cystic lesion
      • Bright contrast-enhancing solid nodule in the wall of the cyst
    • Histopathology
      • Microcysts
      • Bipolar cells; hair-like projections
      • Eosinophilic fibers with corkscrew appearance (Rosenthal fibers)
  • Treatment: Surgical resection
  • Prognosis
    • Favorable prognosis: high rates of long-term survival
    • Curable with complete resection of the tumor
    • Median survival: > 10 years

Optic glioma [12]

Diffuse astrocytoma

  • Description: slow growing, infiltrative tumor that arises from glial cells in the CNS and has the potential to progress to higher-grade tumors
  • Epidemiology: peak age 20–40 years
  • Location: cerebral hemispheres
  • Clinical features
    • Insidious onset and slow progression of symptoms
    • Initially manifest with nonspecific features such as headaches; , seizures
    • Focal symptoms may develop (e..g, progressive paralysis, aphasia)
  • Diagnostics
  • Treatment
    • Complete surgical resection often not possible resection until definable margins can be attempted
    • Percutaneous radiotherapy for very diffuse, unresectable tumors
  • Prognosis: incurable; median survival: 2–12 years

Anaplastic astrocytoma [3][4]

Glioblastoma multiforme

Glial fibrillary acidic protein (GFAP) is an important diagnostic marker for astrocytomas; it is almost always positive in glioblastoma multiforme!

Brainstem glioma [19]

Pediatric primary brain tumors [41]

  • Most pediatric brain tumors are primary.
  • Brain tumors are the second most common cause of pediatric cancer; after leukemia, accounting for approx. 20% of all cases of pediatric cancer and the primary cause of pediatric cancer deaths in the US.
Overview of pediatric primary brain tumors [27][42]
Tumor Precursor Typical location [25] Typical histology [29]
Supratentorial
Craniopharyngioma [34]
  • Suprasellar region
Infratentorial
Pilocytic astrocytoma
  • Rosenthal fibers: eosinophilic fibers with corkscrew-like configuration
  • GFAP positive
Medulloblastoma
Ependymoma [43]
  • Perivascular pseudorosettes: tumor cells that are arranged in a papillary structure around a central blood vessel
Pinealoma

In children, most primary brain tumors arise infratentorial, craniopharyngiomas being an important exception.

Adult primary brain tumors [41]

Overview of adult primary brain tumors [27][42]
Tumor Precursor Typical locations [25] Typical histology [29]
Glioblastoma multiforme (WHO grade IV astrocytoma)
Meningioma
  • Arachnoid cap cells
Hemangioblastoma
  • Vascular system origin
Schwannoma
  • Spindle cells in palisades (Antoni A tissue) alternating with myxoid areas (Antoni B tissue)
  • S-100 positive
Oligodendroglioma
Pituitary adenoma [44]
  • Monomorphic, acidophilic or basophilic, polygonal cells arranged in sheets or cords

In adults, most primary brain tumors arise supratentorially, hemangioblastomas and schwannomas being important exceptions.

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