Craniopharyngioma surgery
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Craniopharyngioma Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Craniopharyngioma surgery On the Web |
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American Roentgen Ray Society Images of Craniopharyngioma surgery |
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Risk calculators and risk factors for Craniopharyngioma surgery |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Patients with craniopharyngioma have many treatment options. The selection depends on the size, location of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities. Because these treatments often damage healthy cells and tissues, side effects are common. Side effect may not be the same for each person, and they may change from one treatment session to the next.
Surgery
For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.[1]
Newly diagnosed craniopharyngioma
There is no consensus on the optimal treatment for newly diagnosed craniopharyngioma, in part because of the lack of prospective randomized trials that compare the different treatment options. Treatment is individualized on the basis of factors that include the following:
- Tumor size
- Tumor location
- Extension of the tumor
- Potential short-term and long-term toxicity
Treatment options for newly diagnosed craniopharyngioma include the following:
- Radical surgery with or without radiation therapy
- It is possible to remove all visible tumor and achieve long-term disease control because these tumors are histologically benign
- A 5-year progression-free survival (PFS) rate of about 65% has been reported
- Radical surgical approaches include the following:
- Transsphenoidal approach: A transsphenoidal approach may be possible for some small tumors located entirely within the sella. The development of expanded endonasal techniques with endoscopic visualization have allowed this approach to be increasingly used, even for sizeable tumors.
- Craniotomy: When an endonasal approach is not possible, a craniotomy is required. The surgeon often has a limited view of the hypothalamic and sellar regions, and portions of the mass may remain after surgery, accounting for some recurrences. Almost all craniopharyngiomas have an attachment to the pituitary stalk, and of the patients who undergo radical surgery, virtually all will require life-long pituitary hormone replacement with multiple medications.
If the surgeon indicates that the tumor was not completely removed or if post-operative imaging reveals residual craniopharyngioma, radiation therapy may be recommended to prevent early progression. Periodic surveillance using MRI is performed for several years after radical surgery because of the possibility of tumor recurrence.
- Subtotal resection with radiation therapy
The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. No attempt is made to remove tumor from the pituitary stalk or hypothalamus in an effort to minimize the late effects associated with radical surgery.[9] The surgical procedure is often followed by radiation therapy, with a 5-year PFS rate of about 70% to 90%[4,12]; [13][Level of evidence: 3iDiii] and 10-year overall survival rates higher than 90%.[14][Level of evidence: 3iiA]; [15][Level of evidence: 3iiiDiii] Conventional radiation therapy is fractionated external-beam radiation, with a recommended dose of 54 Gy to 55 Gy in 1.8-Gy fractions.[16] Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention.[17][Level of evidence: 3iDiv]
A systematic review of 109 reports that described extent of resection found that subtotal resection plus radiation therapy was associated with rates of tumor control similar to those for gross-total resection. It was also reported that both approaches were associated with higher PFS rates than was subtotal resection alone.[15][Level of evidence: 3iiiDiii]
Surgical complications with a subtotal resection are less likely than with radical surgery. Complications of radiation therapy include the following:
Loss of pituitary hormonal function. Cognitive dysfunction. Development of late strokes and vascular malformations. Delayed blindness. Development of second tumors. Malignant transformation of the primary tumor within the radiation field (rare).[18,19]
Newer radiation technologies such as intensity-modulated proton therapy may reduce scatter during whole-brain and whole-body irradiation and result in the sparing of normal tissues. When these highly conformal radiation treatments are employed, interim imaging is commonly performed to detect changes in cyst volume, with treatment plans modified as appropriate.[20-22] It is unknown whether such technologies result in reduced late effects from radiation.[13,21-23]
Tumor progression remains a concern, and it is usually not possible to repeat the radiation dose. In selected cases, stereotactic radiation therapy can be delivered as a single large dose of radiation to a small field.[24][Level of evidence: 3iC] Proximity of the craniopharyngioma to vital structures, particularly the optic nerves, limits this to small tumors within the sella.[25][Level of evidence: 3iiiDiii]
- Primary cyst drainage with or without radiation therapy
Recurrent craniopharyngioma
- Surgery
- Radiation therapy, including radiosurgery
- Intracavitary instillation of radioactive P-32, bleomycin, or interferon-alpha, for those with cystic recurrences
- Systemic interferon
References
- ↑ Rx of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc