More common in children, but occasionally seen in adults
Accounts for 10% of brain tumors in young people
Usually presents with visual symptoms
Etiology:
Environmental factors do not seem to play a role, genetics and hereditary syndromes are more likely linked to brain tumors.1
Signs & Symptoms:
- Compression of the pituitary gland can interrupt the manufacture of part or all of the pituitary hormones. This may cause one or more of the following symptoms:2
Loss of growth in children
Irregular periods
Delayed puberty
Reduction or loss of sexual drive
Fatigue
Low blood pressure
Dry skin
Increased sensitivity to cold and heat
Constipation
Unexplained weight gain
An increase in Prolactin levels, which can cause a milky discharge from the nipples (in both men and women).
- Pressure on the nerves that control vision can cause loss of peripheral vision, which may be noticed especially when driving - Involvement of the hypothalamus, an area at the base of the brain, may result in weight gain, drowsiness, problems with temperature regulation, mood changes and depression or passing large quantities of urine leading to problems with water balance (a condition called Diabetes Insipidus). - Other symptoms can develop for a variety of reasons and may include personality changes, headache, confusion, nausea and vomiting.
Diagnostic Procedures:
Blood tests can detect whether the pituitary gland is manufacturing enough hormones. If any hormones are lacking, it is best to start treatment with hormone tablets as soon as possible.2
Complete Pituitary Function tests to assess all the major pituitary hormones.
Water deprivation test may be required if you are passing much urine and are thirsty.
An eye test (Visual field test) is used to determine whether there is any pressure on the nerves controlling vision. This can usually be done on the same day as the Endocrine Clinic appointment.
MRI scans are used to obtain a picture of the tumor and can tell the extent of the tumor and whether it is invading or pressing on the surrounding brain. Most craniopharyngiomas contain fluid and many also contain some calcium (like bones). This means that they have a unique appearance on a scan that helps to distinguish them from other pituitary tumours. There is often a waiting list for an MRI scan.
Histology:
Craniopharyngiomas are histologically and cytologically benign epithelial tumors of the central nervous system. They typically arise in the sellar/suprasellar region. They can occur anywhere along the infundibulum from the floor of the third ventricle to the pituitary gland.3
Adamantinomatous craniopharyngiomas have a lobulated contour as a result of usually multiple cystic lesions. Solid components are present, but usually form a relatively minor component of the mass, and enhance vividly on both CT and MRI. Overall, calcification is very common, but this is only true of the adamantinomatous subtype (90% are calcified).
Papillary craniopharyngiomas tend to be more spherical in outline and usually lack the prominent cystic component; most are either solid or contain a few smaller cysts. Calcification is uncommon or even rare in the papillary subtype, a fact often forgotten. These tumours tend to displace adjacent structures.
Lymph node drainage:
The central nervous system (CNS) does not contain lymphatic vasculature, so tumors of the CNS do not metastasize through lymphatics.1
Metastatic spread:
Craniopharyngiomas are a slow growing benign tumor that are not very likely to spread.4
Grading:
Adult brain tumor grading:4 Grade I: The tumor grows slowly and contains cells that resemble normal cells. It rarely spreads into nearby tissues and it is possible for surgery to remove the entire tissue.
Grade II: The tumor still grows slowly, but it may spread into adjacent tissue and become a higher grade.
Grade III: The tumor grows quickly and it is likely to spread into adjacent tissue. The cells within the tumor look very different from normal cells.
Grade IV: The tumor grows very aggressively and has cells that look very different from normal cells. It makes it very difficult to treat successfully.
Staging:
There is no standard staging system for adult brain tumors.5 The extent of metastatic spread is usually described as stages. The reason there is no standard staging is because these tumors that begin in the brain rarely spread to other parts of the body.5 Treatment of these tumors is based on the cell type, grade, location, and disease left after surgery.
Radiation side effects:
The main side effect that must be watched when doing radiation therapy to this type of disease is optic neuropathy and brain necrosis.1 This risk is associated with doses higher than 60 gray (Gy). There are other side effects possible that are not specifically related to the radiation, but to the tumor and its relationship to the pituitary gland. These might consist of abnormal growth hormone levels, thyroid levels, testosterone levels, and immune system problems.
Prognosis:
The prognosis for patients with craniopharyngioma is excellent.6 Patients have a 80-90% chance of permanent cure if the tumor can be completely removed with surgery or treated with radiation. Most recurrences occur within the first 2 years after surgery.
Treatments:
Craniopharyngiomas can be treated with complete surgical resection.1 When partial surgical resection is performed radiation is needed. Typical radiation dose are 50.4Gy to 50Gy delivered to the tumor volume with a 1cm margin. Three field opposing lateral and frontal portals, 3D conformal, or IMRT techniques can be used for radiation delivery.
TD 5/5:
The critical structures and their TD 5/5's for the whole organ are:7 Brain: 6000 cGy Lens: 500 cGy Retina: 5500 cGy Cornea: 5000 cGy Ear: 5000 cGy Pituitary Gland: 4500 cGy Brain Stem: 5000 cGy Optic Nerve: 5000 cGy Optic Chiasm: 5000 cGy
Additional images
Reprinted from Saint John's Health Center, 2013.8
Reprinted from Saint John's Health Center, 2013.8
Reprinted from the International Journal of Radiation Oncology Biology Physics, 2013.9
Digitally reconstructed radiographs (DRRs) of 5 noncoplanar treatment fields for craniopharyngioma.
References:
Chao K, Perez C, Brady L. Radiation Oncology Management Decisions. 3rd ed. Philadelphia, PA: Lippincott, Williams and Wilkins; 2011.
Tome WA, Meeks SL, Buatti JM, Bova FJ, Friedman WA, Li D. A high-precision system for conformal intracranial radiotherapy. Int J Radiat Oncol Biol Phys. 2000;47(4):1137-1143.
- Loss of growth in children
- Irregular periods
- Delayed puberty
- Reduction or loss of sexual drive
- Fatigue
- Low blood pressure
- Dry skin
- Increased sensitivity to cold and heat
- Constipation
- Unexplained weight gain
- An increase in Prolactin levels, which can cause a milky discharge from the nipples (in both men and women).
- Pressure on the nerves that control vision can cause loss of peripheral vision, which may be noticed especially when driving- Involvement of the hypothalamus, an area at the base of the brain, may result in weight gain, drowsiness, problems with temperature regulation, mood changes and depression or passing large quantities of urine leading to problems with water balance (a condition called Diabetes Insipidus).
- Other symptoms can develop for a variety of reasons and may include personality changes, headache, confusion, nausea and vomiting.
Grade I: The tumor grows slowly and contains cells that resemble normal cells. It rarely spreads into nearby tissues and it is possible for surgery to remove the entire tissue.
Grade II: The tumor still grows slowly, but it may spread into adjacent tissue and become a higher grade.
Grade III: The tumor grows quickly and it is likely to spread into adjacent tissue. The cells within the tumor look very different from normal cells.
Grade IV: The tumor grows very aggressively and has cells that look very different from normal cells. It makes it very difficult to treat successfully.
Brain: 6000 cGy
Lens: 500 cGy
Retina: 5500 cGy
Cornea: 5000 cGy
Ear: 5000 cGy
Pituitary Gland: 4500 cGy
Brain Stem: 5000 cGy
Optic Nerve: 5000 cGy
Optic Chiasm: 5000 cGy
Reprinted from Saint John's Health Center, 2013.8
Reprinted from Saint John's Health Center, 2013.8
Reprinted from the International Journal of Radiation Oncology Biology Physics, 2013.9
Digitally reconstructed radiographs (DRRs) of 5 noncoplanar treatment fields for craniopharyngioma.
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