Craniopharyngioma surgery: Difference between revisions

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__NOTOC__
__NOTOC__
{{Craniopharyngioma}}
{{Craniopharyngioma}}
{{CMG}}{{AE}}{{Marjan}}
* {{CMG}}{{AE}}{{Marjan}}
 
==Overview==
==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]] 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 postoperative [[Radiation therapy|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|radiosurgery.]]


==Surgery==
==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.<ref>Rx of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc</ref> <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> <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 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> <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> <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>
 
===Newly diagnosed craniopharyngioma===
===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: <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> <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>
* There is no consensus on the optimal treatment for newly diagnosed craniopharyngioma.
#''Tumor size''
#''Tumor location''
#''Extension of the tumor''
#''Potential short-term and long-term toxicity''


Treatment options for newly diagnosed craniopharyngioma 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> <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> <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>
* 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> <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> <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>
#[[Tumor|Tumor size]]
#Tumor location
#Extension of the tumor
#Potential short-term and long-term toxicity
#
*'''Radical surgery with or without radiation therapy'''
*'''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]].
**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.
**A 5-year progression-free survival (PFS) rate of about 65% has been reported.
**Radical surgical approaches include the following:
**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.
***'''Transsphenoidal approach''':
***'''''Craniotomy''''': When an endonasal approach is not possible, a [[craniotomy]] is required. The surgeon often has a limited view of the [[hypothalamus|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. <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> <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> <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> <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>
***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 [[hypothalamus|hypothalamic]] and [[Sella turcica|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. <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> <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> <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> <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>


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'''
*'''Subtotal resection with radiation therapy'''
**The goal of ''limited surgery'' is to establish a diagnosis, drain any cysts, and decompress the [[optic nerve]]s. No attempt is made to remove tumor from the pituitary stalk or hypothalamus in an effort to minimize the ''complications associated with radical surgery''. <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 goal of limited surgery is to establish a diagnosis, drain any cysts, and 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 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%. <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> <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> <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 surgical procedure is often followed by radiation therapy, with 10-year overall survival rates higher than 90%. <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> <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> <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>
**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> <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> <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>
**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> <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> <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>
**Surgical complications with subtotal resection are less likely than with radical 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>
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. <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>
*'''Primary cyst drainage with or without radiation therapy'''
*'''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. <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>
**For larger cystic craniopharyngiomas, particularly in children younger than 3 years. <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>
**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.  
**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. <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> <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>
**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. <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> <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===
===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: <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> <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> <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>
* The incidence of tumor progression after subtotal surgical resection and radiotherapy ranges from 12-25%.
#''Surgery''
* Treatment options for recurrent childhood craniopharyngioma 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> <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> <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> <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>
#''Radiation therapy, including radiosurgery''
#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.<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> <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>
* 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.<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> <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>


==References==
==References==

Revision as of 17:06, 14 February 2019

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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] [3] [4]

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] [3] [4]
  1. Tumor size
  2. Tumor location
  3. Extension of the tumor
  4. Potential short-term and long-term toxicity
  • 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. [2] [3] [4] [5] [6]
  • Subtotal resection with radiation therapy
    • The goal of limited surgery is to establish a diagnosis, drain any cysts, and decompress the optic nerves. [6]
    • The surgical procedure is often followed by radiation therapy, with 10-year overall survival rates higher than 90%. [2] [4] [5] [6]
    • Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention. [2] [3] [4] [5]
    • 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. [2]
    • 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. [2] [4] [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: [2] [3] [4] [5]
  1. Surgery
  2. 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] [4] [5]

References

  1. ↑ Rx of Craniopharyngioma. Cancer gov. http://www.cancer.gov/types/brain/hp/child-cranio-treatment-pdq#link/_40_toc
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 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. ↑ 3.0 3.1 3.2 3.3 3.4 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.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 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.
  5. ↑ 5.0 5.1 5.2 5.3 5.4 5.5 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. ↑ 6.0 6.1 6.2 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.


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