Craniopharyngioma surgery: Difference between revisions

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{{Craniopharyngioma}}
{{Craniopharyngioma}}
{{CMG}}
* {{CMG}}{{AE}}{{Marjan}}
 
==Overview==
==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]] 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==
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>
* 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===
===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:
* There is no [[Consensus (medical)|consensus]] on the [[Optimal classification|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:
* 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'''
*'''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 [[Tumor|visible tumor]] and achieve [[Control|long-term disease control]] because these [[Tumor|tumors]] are [[Histology|histologically]] [[benign]].
**A 5-year progression-free survival (PFS) rate of about 65% has been reported
**A [[Survival analysis|5-year progression-free survival (PFS) rate]] of about 65% has been reported.
**Radical surgical approaches include the following:
**[[Surgery|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.
***[[Sphenoid|'''Transsphenoidal approach''':]]
***'''''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.
***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>


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 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''.
**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 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%.  
**The [[Surgery|surgical procedure]] is often followed by [[radiation therapy]] with [[Survival analysis|10-year overall survival rates]] higher than 90%.  
**Transient cyst enlargement may be noted soon after radiation therapy but generally resolves without further intervention.
**[[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]].  


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.
*'''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.  
**For [[Cystic|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 as a means to postpone or obviate radiation treatment.  
**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: first draining the cyst via the implanted catheter, to relieve pressure and complicating symptoms; and then later resecting the tumor or employing radiation therapy.
**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===
===Recurrent craniopharyngioma===
*Surgery
* The incidence of [[Tumor|tumor progression]] after [[Surgical resection|subtotal surgical resection]] and [[Radiation therapy|radiotherapy]] ranges from 12-25%.
*Radiation therapy, including radiosurgery
* 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>
*Intracavitary instillation of radioactive P-32, bleomycin, or interferon-alpha, for those with cystic recurrences
#[[Surgery]]
*Systemic interferon
#[[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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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

  • Treatment is individualized on the basis of factors that include the following: [2]
  1. Tumor size
  2. Tumor location
  3. Extension of the tumor
  4. Potential short-term and long-term toxicity

Recurrent craniopharyngioma

  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] [6]

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 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 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.
  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.
  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 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.


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