Craniopharyngiomas

Craniopharyngiomas are one of the most challenging brain tumors and given their rarity and complexity should only be treated by highly experienced surgeons at comprehensive skull base centers. They arise from the pituitary stalk at the center of the base of the skull and may involve critical structures such as the brain’s hypothalamus, visual pathways, and deep brain vessels.

Long considered pathologically benign, craniopharyngioma is now classified as a low-grade malignancy that shortens life expectancy. An experienced multidisciplinary team (neurosurgery, ENT, endocrinology, ophthalmology, radiation therapy) is essential for the optimal treatment of both pediatric and adult patients. In fact, short- and long-term outcomes are directly related to the expertise of the surgical team.

The Endoscopic Endonasal Approach (EEA) has emerged over the last decade as the ideal surgical route for most craniopharyngiomas. Our team at Stanford, led by Dr. Fernandez-Miranda, has deep expertise treating craniopharyngiomas using the EEA. We have developed surgical techniques and described key surgical anatomy that are paramount to achieve complete tumor resection while minimizing side effects and surgical risks. Our intense dedication and progressive experience have allowed us to successfully treat Craniopharyngiomas considered “inoperable” or “unresectable” by many surgeons.

Symptoms

Craniopharyngiomas cause neurologic signs and symptoms by interfering with communication between the brain and the pituitary gland or by compressing nearby structures.

Disruption of signaling along the pituitary stalk by the tumor can reduce pituitary production of hormones that control water balance, growth, sex function, and energy production. Resulting symptoms and signs include excessive urination and dehydration (diabetes insipidus), short stature in children, infertility (loss of menstruation in women and impotence in men), and fatigue. Compression of adjacent brain structures such as the optic nerves and chiasm, hypothalamus and brain fluid pathways at the third ventricle can cause visual loss, excessive appetite and obesity, and obstructive hydrocephalus leading to headache, vomiting, personality changes and decreased consciousness.

Diagnosis

Such symptoms should prompt detailed examination of visual fields, laboratory studies looking for abnormalities in blood electrolytes (high sodium) and pituitary hormones (low cortisol, thyroid hormone, and growth hormone, and testosterone) and brain imaging.

Brain CT scan will usually show a mass above the pituitary gland that has one or more solid, partially calcified regions and multiple cysts of varying sizes. With large tumors, the lateral and third ventricles may be enlarged. Brain CT-angiogram will help studying the relationship of the tumor with surrounding brain vessels. Brain MRI focused on the pituitary region will show with greater anatomic detail nearby structures and more complete characterization of the tumor’s components. At Stanford, we can integrate all these imaging studies into a Virtual Reality (VR) simulation platform to better understand the three-dimensional anatomical relationships of the tumor with key brain structures.

Treatment

The primary treatment of craniopharyngiomas is surgical resection with the goal of complete tumor removal to maximize the chances of surgical care.

This is particularly desirable in children in whom radiation should be avoided if at all possible. In selected cases, a more limited surgical resection might be preferred to preserve the function of the pituitary gland, but in many cases the function of the gland has already been compromised by tumor growth or will deteriorate as tumor progresses. Similarly, there are cases where the tumor is very adherent to the visual pathways, the hypothalamus, or deep brain vessels; in these situations, the expertise of the surgeon is key to preserve these structures while leaving the minimal possible tumor residual.

The main risk of surgery is loss of pituitary hormonal production. This risk is higher in large tumors that invade the tubero-infundibular region and lower in small tumors arising at the pituitary stalk near the pituitary gland. Fortunately, current hormonal replacement therapies, under the supervision of expert pituitary neuro-endocrinologists such as ours at Stanford, are highly effective and allow patients to have a healthy life.

The second most common risk of surgery is cerebro-spinal fluid (CSF) leak, which in our experience occurs in less than 5% of patients and requires a second operation for repair of the leakage. Advanced reconstruction techniques done in conjunction with our ENT-Rhinology team using local nasal mucosal flaps are highly effective to prevent and/or treat CSF leaks. The expertise of the ENT-Rhinology team is also critical to minimize damage to sino-nasal structures and expedite their recovery.

The risks of surgery to surrounding brain structures, causing visual loss, hypothalamic injury, or stroke, is directly related to the skills and experience of the surgical team. At Stanford, our current complete tumor resection rates are above 90% with less than 5% risk of visual loss, less than 1% risk of stroke, and no incidence of hypothalamic injury. We have successfully removed craniopharyngiomas using the EEA in patients as young as 2-year old, and in many that had undergone multiple previous operations (via EEA and/or transcranial), radiation treatments, or had giant or very calcified tumors. Several of these cases had been considered “inoperable” at other major academic centers. Our results are the consequence of a life-long dedication to excellence and innovation in endoscopic endonasal surgery.

Even with the best possible surgical treatment, some tumors cannot be completely removed or regrow years later. Radiation is indicated in selected cases of craniopharyngiomas that are incompletely removed or recur. New radiation techniques such as CyberKnife stereotactic radiosurgery permit highly accurate delivery of radiation focused on the tumor so as to limit damage to surrounding brain structures. Transcranial approaches are rarely used for craniopharyngiomas at our center, given the superiority of the EEA, but still applied in certain cases that extend beyond the anatomical limits of the EEA. Tumor cysts that recur after surgery may require drainage through an implanted (Ommaya) reservoir or instillation of radioactive isotopes.

If you or your loved one have been diagnosed with a craniopharyngioma, we will be privileged to evaluate your case and offer our recommendations for treatment.

Patient Story: David Silva
Treated for a Craniopharyngioma

David Silva was diagnosed with a craniopharyngioma brain tumor when he was just 22 years old. Over the course of a decade Silva endured five brain surgeries at multiple hospitals, but the procedures were all unsuccessful and the tumor persisted. Silva was ready to give up hope, but then he heard about a neurosurgeon with unique expertise at Stanford.

Read David Silva's Story

Virtual Reality Simulation:

Presurgical Planning on a Complex Craniopharyngioma

Endoscopic Endonasal Approach for Giant Craniopharyngioma

Removal of Giant Pediatric Craniopharyngioma

Craniopharyngioma Removal at Stanford

Webinar: Endoscopic Endonasal Surgery for "Inoperable Craniopharyngiomas"

Webinar: Endoscopic Resection of Craniopharyngiomas

Endoscopic Endonasal Approach for Recurrent Sellar-Cavernous Sinus Craniopharyngioma

References

Fernandez-Miranda JC, Prevedello DM, Madhok R, Barges-Coll J, Morera VA, Gardner PA, Snyderman CH, Carrau R, Kassam AB. Sphenoid Septations and the Internal Carotid Artery. Laryngoscope 119:1893-6, 2009

Fernandez-Miranda JC, Barges-Coll J, Prevedello DM, Engh J, Snyderman C, Carrau R, Gardner PA, Kassam AB. Animal model for endoscopic neurosurgical training: technical note. Minim Invasive Neurosurg. 2010 Oct;53(5):286-9

Snyderman CH, Fernandez-Miranda JC, Gardner PA. Training in neurorhinology: the impact of case volume on the learning curve. Otolaryngol Clin North Am 2011 Oct; 44(5):12223-8.

Fernandez-Miranda JC, Gardner PA, Snyderman CH, Devaney KO, Strojan P, Suarez C, Genden EM, Rinaldo A, Ferlito A. Craniopharyngioma: a pathologic, clinical and surgical review. Head Neck. 2012 Jul;34(7):1036-44.

Paluzzi A, Gardner P, Fernandez-Miranda JC, Snyderman C. The expanding role of endoscopic skull base surgery. Br J Neurosurg. 2012 Oct;26(5):649-61.

Fernandez-Miranda JC, Tormenti M, Latorre F, Gardner P, Snyderman C. Endoscopic endonasal middle clinoidectomy: anatomical, radiological, and technical note. Neurosurgery. 2012 Dec;71[ONS Suppl 2]:ons233-ons239.

Koutourousiou M, Gardner PA, Kofler JK, Fernandez-Miranda JC, Snyderman CH, Lunsford LD. Rare infundibular lesions: clinical presentation, imaging findings and the role of endoscopic endonasal surgery in their management. J Neurol Surg B. 2013 Feb;74:1-11.

Labib MA, Prevedello DM, Fernandez-Miranda JC, Sivakanthan S, Benet A, Morera V, Carrau R, Kassam A. The medial opticocarotid recess: an anatomic study of an endoscopic "key landmark" for the ventral cranial base. Neurosurgery. 2013 Mar;72(1 Suppl Operative):66-76

Chivukula S, Koutourousiou M, Snyderman CH, Fernandez-Miranda JC, Gardner PA, Tyler-Kabara EC. Endoscopic endonasal skull base surgery in the pediatric population. J Neurosurg Pediatr. 2013 Mar;11(3):227-41.

Koutourousiou M, Gardner PA, Fernandez-Miranda JC, Tyler-Kabara EC, Wang EW, Snyderman CH. Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients. J Neurosurg. 2013 Nov; 119(5): 1194-207.

Kaplan DJ, Vaz-Guimaraes F, Fernandez-Miranda JC, Snyderman CH. Validation of a chicken wing training model for endoscopic microsurgical dissection. Laryngoscope. 2015 Mar;125(3):571-6.

Vaz-Guimaraes F, Su SY, Fernandez-Miranda JC, Wang EW, Snyderman CH, Gardner PA. Hemostasis in endoscopic endonasal skull base surgery. J Neurol Surg B Skull Base. 2015 Aug;76(4):296-302.

Abhinav K, Acosta Y, Wang WH, Bonilla LR, Koutourousiou M, Wang E, Snyderman C, Gardner P, Fernandez-Miranda JC. Endoscopic endonasal approach to the optic canal: Anatomic considerations and surgical relevance. Neurosurgery. 2015 Sep;11 Suppl 3:431-46

Vaz-Guimaraes F, Rastelli Jr MM, Fernandez-Miranda JC, Wang EW, Gardner PA, Snyderman CH. Impact of dynamic endoscopy and bimanual-binarial dissection in endoscopic endonasal surgery training: a laboratory investigation. J Neurol Surg B. 2015 Sep;76(5):365-71.

Zhang X, Wang EW, Wei H, Shi J, Snyderman CH, Gardner PA, Fernandez-Miranda JC. Anatomy of the posterior septal artery with surgical implications on the vascularized pedicled nasoseptal flap. Head Neck. 2015 Oct;37(10):1470-6.

Patel CR, Fernandez-Miranda JC, Wang WH, Wang EW. Skull base anatomy. Otolaryngol Clin North Am. 2016 Feb;49(1):9-20.

Gardner PA, Snyderman CH, Fernandez-Miranda JC, Jankowitz BT. Management of major vascular injuiry during endoscopic endonasal skull base surgery. Otolaryngol Clin North Am. 2016 Jun;49(3):819-28.

Fraser S, Gardner PA, Koutourousiou M, Kubik M, Fernandez-Miranda JC, Snyderman CH, Wang EW. Risk factors associated with postoperative cerebrospinal fluid leak after endoscopic endonasal skull base surgery. J Neurosurg. 2017 Jun 9:1-6

Pacca P, Jhawar SS, Seclen DV, Wang E, Snyderman C, Gardner PA, Aboud E, Fernandez-Miranda JC. “Live cadaver” model for internal carotid artery injury simulation in endoscopic endonasal skull base surgery. Oper Neurosurg (Hagerstown). 2017 Jun 29

Chabot JD, Patel CR, Hughes MA, Wang EW, Snyderman CH, Gardner PA, Fernandez-Miranda JC. Nasoseptal flap necrosis: a rare complication of endoscopic endonasal surgery. J Neurosurg. 2017 Jul 21;1-10

Geltzeiler M, Nakassa ACI, Turner M, Setty P, Zenonos G, Hebert A, Wang E, Fernandez-Miranda J, Snyderman C, Gardner P. Evaluation of Intranasal Flap Perfusion by Intraoperative Indocyanine Green Fluorescence Angiography. Oper Neurosurg (Hagerstown). 2018 Dec 1

Koutourousiou M, Fernandez-Miranda JC, Wang EW, Snyderman CH, Gardner PA. The limits of transsellar/transtuberculum surgery for craniopharyngioma. J Neurosurg Sci. 2018 Jun;62(3):301-309

Truong HQ, Najera E, Zanabria-Ortiz R, Celtikci E, Sun X, Borghei-Razavi H, Gardner PA, Fernandez-Miranda JC. Surgical anatomy of the superior hypophyseal artery and its relevance for endoscopic endonasal surgery. J Neurosurg. 2018 Jul 13:1-9

National and International Lectures (by Dr. Fernandez-Miranda)

Inoperable Craniopharyngiomas: Technical Nuances and Management of Complications. American Association of Neurological Surgeons, San Diego, CA, April, 2019

Endoscopic Endonasal Surgery for “Inoperable” Craniopharyngiomas. International Endonasal Surgery Symposium, Beijing, China, March 2019

Endoscopic Endonasal Skull Base Surgery: Suprasellar Region. 3rd Stanford-Asia Rhinology Forum. Stanford, CA, November 10, 2018

Endoscopic endonasal approaches to the hypothalamus and brainstem. Skull Base Symposium. Samsung Medical Center, Seoul, South Korea, November 2nd, 2018

Craniopharyngioma – what’s new? Brigham and Women's Hospital Cerebrovascular and Skull Base Symposium. Brigham Women’s Hospital, October 6, 2017.

3D Endoscopic Endonasal Surgical Anatomy: Suprasellar Region and Anterior Skull Base. 2017 Advanced Endoscopic Skull Base Surgery Course, Shanghai, China, September 9, 2017.

3D Surgical Anatomy Lecture: Sinonasal, Sellar, and Parasellar Regions, 3D Surgical Anatomy Lecture: Sinonasal, Sellar and Parasellar Regions, National Skull Base Endoscope Conference, Xi’an, China, April 9, 2017.

Endoscopic endonasal surgery for suprasellar meningiomas and craniopharyngiomas. Annual Scientific Meeting of the Royal College of Neurological Surgeons of Thailand, Bangkok, Thailand, February 14, 2017.

Cirugia Endoscopica de los Craneofaringiomas. Neuropinamar, Buenos Aires, Argentina, December 2, 2016

Anatomy of the sella turcica and parasellar region, skull base surgery and endoscopic surgery.Dr.Rhoton’s Memorial Session in the Annual Meeting, Fukuoka, Japan, September 29, 2016.

Endoscopic endonasal approach to the suprasellar region and anterior skull base. 31st Brazilian Congress of Neurosurgery (CBN2016), September 6, 2016.

Endonasal endoscopic surgery of the sellar region, parasellar, suprasellar, and retroinfundibular. Endoscopy Endonasal skull base surgery course. Madrid, Spain, June 28, 2016.

3D Endoscopic endonasal approach to the sellar and suprasellar regions, cavernous sinus and middle fossa. 2015 American Association of Neurological Surgeons Annual Meeting, May 2, 2015

Endoscopic endonasal skull base surgery: from the lab to the OR. Pre-Meeting Course, 25th Annual Meeting of the North American Skull Base Society, Tampa, FL, February 18, 2015.

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Craniopharyngiomas