Craniopharyngioma surgery: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Craniopharyngioma}} | {{Craniopharyngioma}} | ||
{{CMG}} | * {{CMG}}{{AE}}{{Marjan}} | ||
==Overview== | ==Overview== | ||
[[Surgery]] is the mainstay of treatment for craniopharyngioma. [[Radical surgery]] is done either by [[Sphenoidal|transsphenoidal approach]] or by [[craniotomy]]. Recent studies propose subtotal resection with [[Post operative complications|post operative]] [[Radiation therapy|radiotherapy]] as the management of choice for craniopharyngiomas, especially in the [[Pediatrics|pediatric population]]. More advanced [[Radiation therapy|radiotherapy]] [[Modality|modalities]] currently under [[Investigational Device Exemption|investigation]] include [[Gamma Knife]] and [[Cyberknife|cyber knife]] [[Radiosurgery|radiosurgery.]] | |||
==Surgery== | ==Surgery== | ||
The | * Surgery is the [[first-line treatment]] option for patients with craniopharyngioma, with or without [[Radiation therapy|radiation]]. | ||
* For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.<ref>Rx of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc</ref> | |||
===Newly diagnosed craniopharyngioma=== | |||
* There is no [[Consensus (medical)|consensus]] on the [[Optimal classification|optimal treatment]] for newly diagnosed craniopharyngioma. | |||
* Treatment is individualized on the basis of factors that include the following: <ref name="pmid18091236">{{cite journal |vauthors=de Divitiis E, Cappabianca P, Cavallo LM, Esposito F, de Divitiis O, Messina A |title=Extended endoscopic transsphenoidal approach for extrasellar craniopharyngiomas |journal=Neurosurgery |volume=61 |issue=5 Suppl 2 |pages=219–27; discussion 228 |date=November 2007 |pmid=18091236 |doi=10.1227/01.neu.0000303220.55393.73 |url=}}</ref> | |||
#[[Tumor|Tumor size]] | |||
#[[Tumor|Tumor location]] | |||
#[[Tumor|Extension of the tumor]] | |||
#[[Toxicity|Potential short-term and long-term toxicity]] | |||
# | |||
*'''Radical surgery with or without radiation therapy''' | |||
**It is possible to remove all [[Tumor|visible tumor]] and achieve [[Control|long-term disease control]] because these [[Tumor|tumors]] are [[Histology|histologically]] [[benign]]. | |||
**A [[Survival analysis|5-year progression-free survival (PFS) rate]] of about 65% has been reported. | |||
**[[Surgery|Radical surgical approaches]] include the following: | |||
***[[Sphenoid|'''Transsphenoidal approach''':]] | |||
***A [[Sphenoidal|transsphenoidal approach]] may be possible for some [[Tumors|small tumors]] located entirely within the [[sella]]. | |||
***The development of [[Expanded Criteria Donor|expanded]] [[Nasal|endonasal techniques]] with [[Endoscopy|endoscopic visualization]] have allowed this approach to be increasingly used. | |||
***'''[[Craniotomy]]''': | |||
***When an [[Nasal|endonasal approach]] is not possible, a [[craniotomy]] is required. | |||
***The surgeon often has a limited view of the [[hypothalamus|hypothalamic]] and [[Sella turcica|sellar regions]], and portions of the mass may remain after [[surgery]]. | |||
***Almost all craniopharyngiomas have an [[Attachment theory|attachment to the]] [[pituitary stalk]], and virtually all will require life-long [[pituitary hormone]] replacement.<ref name="pmid16580494">{{cite journal |vauthors=Fitzek MM, Linggood RM, Adams J, Munzenrider JE |title=Combined proton and photon irradiation for craniopharyngioma: long-term results of the early cohort of patients treated at Harvard Cyclotron Laboratory and Massachusetts General Hospital |journal=Int. J. Radiat. Oncol. Biol. Phys. |volume=64 |issue=5 |pages=1348–54 |date=April 2006 |pmid=16580494 |doi=10.1016/j.ijrobp.2005.09.034 |url=}}</ref> | |||
*'''Subtotal resection with radiation therapy''' | |||
**The goal of [[Surgery|limited surgery]] is to establish a [[diagnosis]], [[Cyst|drain any cysts]], and [[Decompression|decompress]] the [[optic nerve]]s. <ref name="pmid16580494">{{cite journal |vauthors=Fitzek MM, Linggood RM, Adams J, Munzenrider JE |title=Combined proton and photon irradiation for craniopharyngioma: long-term results of the early cohort of patients treated at Harvard Cyclotron Laboratory and Massachusetts General Hospital |journal=Int. J. Radiat. Oncol. Biol. Phys. |volume=64 |issue=5 |pages=1348–54 |date=April 2006 |pmid=16580494 |doi=10.1016/j.ijrobp.2005.09.034 |url=}}</ref> | |||
**The [[Surgery|surgical procedure]] is often followed by [[radiation therapy]] with [[Survival analysis|10-year overall survival rates]] higher than 90%. | |||
**[[Cyst|Transient cyst enlargement]] may be noted soon after [[radiation therapy]] but generally resolves without further intervention. <ref name="pmid18091236">{{cite journal |vauthors=de Divitiis E, Cappabianca P, Cavallo LM, Esposito F, de Divitiis O, Messina A |title=Extended endoscopic transsphenoidal approach for extrasellar craniopharyngiomas |journal=Neurosurgery |volume=61 |issue=5 Suppl 2 |pages=219–27; discussion 228 |date=November 2007 |pmid=18091236 |doi=10.1227/01.neu.0000303220.55393.73 |url=}}</ref> <ref name="pmid23486689">{{cite journal |vauthors=Cohen M, Bartels U, Branson H, Kulkarni AV, Hamilton J |title=Trends in treatment and outcomes of pediatric craniopharyngioma, 1975-2011 |journal=Neuro-oncology |volume=15 |issue=6 |pages=767–74 |date=June 2013 |pmid=23486689 |pmc=3661103 |doi=10.1093/neuonc/not026 |url=}}</ref> | |||
**[[Complications|Surgical complications]] with [[Resection|subtotal resection]] are less likely than with [[radical surgery]]. | |||
*'''Primary cyst drainage with or without radiation therapy''' | |||
**For [[Cystic|larger cystic]] craniopharyngiomas, particularly in children younger than 3 years. | |||
**Benefits include [[Pressure|temporary relief of fluid pressure]] by serial drainage, and in some cases, for [[Mammary adenosis|intracystic instillation of sclerosing agents]]. | |||
**This procedure allows to use a two-staged approach: [[Cyst|first draining the cyst]] via the [[Catheter|implanted catheter]], to relieve pressure and complicating symptoms and then later [[Resection|resecting the tumor]] or employing [[radiation therapy]]. <ref name="pmid28570730">{{cite journal |vauthors=Jalali R, Gupta T, Goda JS, Goswami S, Shah N, Dutta D, Krishna U, Deodhar J, Menon P, Kannan S, Sarin R |title=Efficacy of Stereotactic Conformal Radiotherapy vs Conventional Radiotherapy on Benign and Low-Grade Brain Tumors: A Randomized Clinical Trial |journal=JAMA Oncol |volume=3 |issue=10 |pages=1368–1376 |date=October 2017 |pmid=28570730 |pmc=5710529 |doi=10.1001/jamaoncol.2017.0997 |url=}}</ref> | |||
===Recurrent craniopharyngioma=== | |||
* The incidence of [[Tumor|tumor progression]] after [[Surgical resection|subtotal surgical resection]] and [[Radiation therapy|radiotherapy]] ranges from 12-25%. | |||
* Treatment options for recurrent childhood craniopharyngioma include the following: <ref name="pmid23633208">{{cite journal |vauthors=Elowe-Gruau E, Beltrand J, Brauner R, Pinto G, Samara-Boustani D, Thalassinos C, Busiah K, Laborde K, Boddaert N, Zerah M, Alapetite C, Grill J, Touraine P, Sainte-Rose C, Polak M, Puget S |title=Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity |journal=J. Clin. Endocrinol. Metab. |volume=98 |issue=6 |pages=2376–82 |date=June 2013 |pmid=23633208 |doi=10.1210/jc.2012-3928 |url=}}</ref> | |||
#[[Surgery]] | |||
#[[Radiation therapy]] including [[radiosurgery]] | |||
* The management of [[Craniopharyngioma|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. | |||
* [[Complication (medicine)|Complications]] are more frequent than with initial [[surgery]].<ref name="pmid18091236">{{cite journal |vauthors=de Divitiis E, Cappabianca P, Cavallo LM, Esposito F, de Divitiis O, Messina A |title=Extended endoscopic transsphenoidal approach for extrasellar craniopharyngiomas |journal=Neurosurgery |volume=61 |issue=5 Suppl 2 |pages=219–27; discussion 228 |date=November 2007 |pmid=18091236 |doi=10.1227/01.neu.0000303220.55393.73 |url=}}</ref> <ref name="pmid23633208">{{cite journal |vauthors=Elowe-Gruau E, Beltrand J, Brauner R, Pinto G, Samara-Boustani D, Thalassinos C, Busiah K, Laborde K, Boddaert N, Zerah M, Alapetite C, Grill J, Touraine P, Sainte-Rose C, Polak M, Puget S |title=Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity |journal=J. Clin. Endocrinol. Metab. |volume=98 |issue=6 |pages=2376–82 |date=June 2013 |pmid=23633208 |doi=10.1210/jc.2012-3928 |url=}}</ref> | |||
==References== | ==References== | ||
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- 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 post operative radiotherapy as the management of choice for craniopharyngiomas, especially in the pediatric population. More advanced radiotherapy modalities currently under investigation include Gamma Knife and cyber knife 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]
Newly diagnosed craniopharyngioma
- There is no consensus on the optimal treatment for newly diagnosed craniopharyngioma.
- Treatment is individualized on the basis of factors that 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.
- 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.
- Almost all craniopharyngiomas have an attachment to the pituitary stalk, and virtually all will require life-long pituitary hormone replacement.[3]
- Subtotal resection with radiation therapy
- The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. [3]
- The surgical procedure is often followed by radiation therapy with 10-year overall survival rates higher than 90%.
- Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention. [2] [4]
- Surgical complications with subtotal resection are less likely than with radical surgery.
- Primary cyst drainage with or without radiation therapy
- For larger cystic craniopharyngiomas, particularly in children younger than 3 years.
- Benefits include temporary relief of fluid pressure by serial drainage, and in some cases, for intracystic instillation of sclerosing agents.
- 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. [5]
Recurrent craniopharyngioma
- The incidence of tumor progression after subtotal surgical resection and radiotherapy ranges from 12-25%.
- Treatment options for recurrent childhood craniopharyngioma include the following: [6]
- 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.[2] [6]
References
- ↑ Rx of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc
- ↑ 2.0 2.1 2.2 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.
- ↑ 3.0 3.1 Fitzek MM, Linggood RM, Adams J, Munzenrider JE (April 2006). "Combined proton and photon irradiation for craniopharyngioma: long-term results of the early cohort of patients treated at Harvard Cyclotron Laboratory and Massachusetts General Hospital". Int. J. Radiat. Oncol. Biol. Phys. 64 (5): 1348–54. doi:10.1016/j.ijrobp.2005.09.034. PMID 16580494.
- ↑ Cohen M, Bartels U, Branson H, Kulkarni AV, Hamilton J (June 2013). "Trends in treatment and outcomes of pediatric craniopharyngioma, 1975-2011". Neuro-oncology. 15 (6): 767–74. doi:10.1093/neuonc/not026. PMC 3661103. PMID 23486689.
- ↑ Jalali R, Gupta T, Goda JS, Goswami S, Shah N, Dutta D, Krishna U, Deodhar J, Menon P, Kannan S, Sarin R (October 2017). "Efficacy of Stereotactic Conformal Radiotherapy vs Conventional Radiotherapy on Benign and Low-Grade Brain Tumors: A Randomized Clinical Trial". JAMA Oncol. 3 (10): 1368–1376. doi:10.1001/jamaoncol.2017.0997. PMC 5710529. PMID 28570730.
- ↑ 6.0 6.1 Elowe-Gruau E, Beltrand J, Brauner R, Pinto G, Samara-Boustani D, Thalassinos C, Busiah K, Laborde K, Boddaert N, Zerah M, Alapetite C, Grill J, Touraine P, Sainte-Rose C, Polak M, Puget S (June 2013). "Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity". J. Clin. Endocrinol. Metab. 98 (6): 2376–82. doi:10.1210/jc.2012-3928. PMID 23633208.