Craniopharyngioma surgery
|
Craniopharyngioma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Craniopharyngioma surgery On the Web |
|
American Roentgen Ray Society Images of Craniopharyngioma surgery |
|
Risk calculators and risk factors for Craniopharyngioma surgery |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]
Overview
Surgery is the mainstay of treatment for craniopharyngioma. Radical surgery is done either by transsphenoidal approach or by craniotomy. Recent studies propose subtotal resection with postoperative radiotherapy as the management paradigm of choice for craniopharyngiomas, especially in the pediatric population. More advanced radiotherapy modalities currently under investigation include Gamma Knife and CyberKnife radiosurgery.
Surgery
Surgery is the first-line treatment option for patients with craniopharyngioma, with or without radiation. For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.[1] [2]
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: [2]
- Tumor size
- Tumor location
- Extension of the tumor
- Potential short-term and long-term toxicity
Treatment options for newly diagnosed craniopharyngioma include the following: [2]
- 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. [2]
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 complications associated with radical surgery.
- The surgical procedure is often followed by radiation therapy, with a 5-year progression-free survival (PFS) rate of about 70% to 90%; and 10-year overall survival rates higher than 90%. [2]
- Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention. [2]
Research shows 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.
Surgical complications with subtotal resection are less likely than with radical surgery. [2]
- Primary cyst drainage with or without radiation therapy
- For larger cystic craniopharyngiomas, particularly in children younger than 3 years, stereotactic or open implantation of an intracystic catheter with a subcutaneous reservoir may be a valuable alternative treatment option. [2]
- Benefits include temporary relief of fluid pressure by serial drainage, and in some cases, for intracystic instillation of sclerosing agents as a means to postpone or obviate radiation treatment.
- This procedure allows to use a two-staged approach: first draining the cyst via the implanted catheter, to relieve pressure and complicating symptoms; and then later resecting the tumor or employing radiation therapy. [2]
Recurrent craniopharyngioma
The incidence of tumor progression after subtotal surgical resection and radiotherapy ranges from 12-25% and is similar to rates associated with failed gross total resection and radiotherapy (4-25%).. Treatment options for recurrent childhood craniopharyngioma include the following: [2]
- Surgery
- Radiation therapy, including radiosurgery
The management of recurrent craniopharyngioma is determined largely by previous therapy. Repeat attempts at gross-total resections are difficult, and long-term disease control is less often achieved. Complications are more frequent than with initial surgery. If not previously employed, external-beam radiation therapy is an option, to include consideration of radiosurgery in selected circumstances.[2]
References
- ↑ Rx of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 de Divitiis E, Cappabianca P, Cavallo LM, Esposito F, de Divitiis O, Messina A (November 2007). "Extended endoscopic transsphenoidal approach for extrasellar craniopharyngiomas". Neurosurgery. 61 (5 Suppl 2): 219–27, discussion 228. doi:10.1227/01.neu.0000303220.55393.73. PMID 18091236.