Craniopharyngioma


Article Author:
Michael Ortiz Torres


Article Editor:
Fassil Mesfin


Editors In Chief:
Neha Amin
Manuj Agarwal


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Kyle Blair
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
1/30/2019 10:25:51 AM

Introduction

Craniopharyngioma is a rare, virtually benign tumor of the central nervous system (CNS). It is a partly cystic embryonic malformation that can occur in the sellar/parasellar region and can produce a wide array of symptomatology such as headaches, nausea and vomiting, visual disturbances, and endocrine disturbances. It represents a special challenge for the physicians that treat it; these physicians commonly include neurosurgeons, neuro-ophthalmologists, neurologists, endocrinologists, and pediatricians. The challenge is due to the tumor's ability to adhere to the surfaces that surround it. For this reason, it is extremely difficult to control, and it is also notorious for its high rates of recurrence.[1][2][3][4]

Etiology

There are two major theories of the development of craniopharyngioma: the embryonic theory and the metaplastic theory. These two theories correlate with the two subtypes of craniopharyngioma which are the adamantinomatous craniopharyngiomas and the papillary craniopharyngiomas.

The embryonic theory is related to the development of adamantinomatous craniopharyngiomas, which are believed to be the most common subtype of craniopharyngioma that occurs in the pediatric population. During embryogenesis, there is an outpouching of the ectodermal roof of the stomodeum. This outpouching, known as Rathke's pouch, extends cranially towards the floor of the diencephalon to later form the adenohypophysis or anterior pituitary gland. While migrating cranially, its extension forms the craniopharyngeal duct which later involutes. In some occasions, involution is not total, and remnants of ectodermal cells can be present. These embryonic cells can proliferate around the extension of the craniopharyngeal duct and develop into a craniopharyngioma.

The metaplastic theory is related to the development of papillary craniopharyngiomas, which are believed to be the most common subtype of craniopharyngioma in adult patients. It states that adenohypophyseal cells of the pars tuberalis can undergo metaplasia and result in the formation of squamous cell nests. These nests can then proliferate and lead to a papillary craniopharyngioma.

Epidemiology

Craniopharyngioma has an incidence of 0.5 to 2 cases per million persons per year. Almost half of these cases occur during the first two decades of life. It represents 1.2% to 4% of all childhood intracranial tumors. It has a classical bimodal distribution of incidence with increased incidence rates in patients aged five to 14 years and 50 to 74 years. No statistically significant differences have been described regarding demographic characteristics such as age, gender, race, and geographical location. Interesting instances of craniopharyngioma have been reported, including craniopharyngiomas of the cerebellopontine angle, malignant transformation of craniopharyngiomas and familial cases of craniopharyngiomas.

Craniopharyngioma has a very high recurrence rate, with reported rates as high as 50%. It also has high survival rates (83% to 96% five-year survival and 65% to 100% 10-year survival) but also carries similar rates of morbidity, with almost all patients developing some sequelae.

Pathophysiology

The most common location of craniopharyngioma is the sellar/suprasellar region, with 95% of craniopharyngiomas having a suprasellar component. Its location defines its pathophysiology. Craniopharyngiomas can compress normal pituitary tissue and result in pituitary deficiencies, particularly of the anterior pituitary hormones. It can also compress the optic chiasm and/or optic nerves and cause different degrees and types of visual disturbances, from blurry vision to blindness. It can also present with hydrocephalus secondary to third ventricle compression. In cases of significant suprasellar extension, non-specific symptoms of intracranial hypertension such as a headache, nausea, and vomiting can also occur. Cases of isolated oculomotor nerve and abducens nerve palsies have been described.

Histopathology

As mentioned before, there are two subtypes of craniopharyngioma: adamantinomatous and papillary.

Adamantinomatous craniopharyngioma is characterized by dense nodules and trabeculae of squamous epithelium bordered by a palisade of columnar epithelium sometimes referred to as a "picket fence." These nests of squamous epithelium are surrounded by loose aggregates of squamous epithelium known as stellate reticulum. Spread between the mix, one can find cystic cavities which hold an oily proteinaceous fluid along with cholesterol, piloid gliosis, granulomatous inflammation, calcification, and nodules of "wet keratin."

On the other hand, papillary craniopharyngioma is characterized as well-differentiated squamous epithelium lacking surface maturation, with occasional goblet cells and ciliated epithelium. It is not nearly as organized as the adamantinomatous subtype, and calcifications are rare.

History and Physical

Craniopharyngioma most commonly manifests with signs of increased intracranial pressure (ICP) including a headache and nausea and vomiting along with visual and endocrine disturbances (62% to 84% and 52% to 87%, respectively). The most common visual disturbance encountered is temporal hemianopsia due to optic chiasm compression. The onset of blindness is considered a neurosurgical emergency. At the time of presentation, around 40% to 87% of patients present with at least one hormonal deficit. The hormonal deficiency is secondary to normal pituitary compression, particularly of the anterior pituitary. In some cases, posterior pituitary hormonal deficiencies can be seen, particularly diabetes insipidus. In children, failure to thrive and decreased growth rate can be the initial presentation as growth hormone is the most commonly affected hormone.

Evaluation

Any presentation with a combination of a headache, visual disturbance, and endocrine disturbance should have a pituitary region lesion in the differential diagnosis.

Visual exam, including acuity and visual fields, can suggest a visual disturbance and visual fields can be used to confirm it. In terms of hormonal deficiencies, laboratory tests for hormone level measurement or stimulation of hormone production can be used to confirm a suspected hormonal deficiency.

Treatment / Management

Multiple modalities can be implemented in the management of craniopharyngioma, including neurological surgery, radiotherapy, and instillation of sclerosing substances. There is no consensus on the best treatment regimen. The types of modalities chosen depend on neurosurgeon judgment and experience.[5][6][7][8]

Neurosurgery

The most common surgical approaches include pterional, subfrontal, and transsphenoidal. Transcallosal approaches for craniopharyngiomas with third ventricle extension, retrosigmoid approaches for posterior fossa craniopharyngiomas and transorbital approaches have also been described. Extension of resection is a matter of debate. Gross total resection has been associated with increased incidence of post-surgical deficits, with no clear benefits in regards to recurrence rates. Unless the tumor is clearly visualized and with no extension into neural structures including the hypothalamus, optic nerves, optic chiasm, and/or carotids, most neurosurgeons will favor a partial resection. These partial resections can be accompanied by adjuvant methods including radiotherapy and instillation of sclerosing substances. For the later, in cases of cystic tumors, the surgeon can introduce a catheter into the tumor and connect it to a reservoir. The reservoir then permits instillation of substances into the tumor as well as aspiration of cystic fluid, which can be performed as an outpatient procedure.

Radiotherapy

Radiation therapy includes various modalities: conventional external radiotherapy, proton beam therapy, stereotactic radiotherapy, radiosurgery, and brachytherapy. The goal of radiotherapy is to decrease tumor burden while protecting essential neural structures. Specific Gy doses have been designated for every radiation modality. Multiple reports have suggested decreased mortality with slightly reduced morbidity following radiation therapy. Despite this, radiation therapy has not been proven to reduce recurrence rate. Therefore, it continues to be an adjuvant modality to neurosurgical intervention.

Instillation of sclerosing substances

This method consists of instillation of different toxic substances with the endpoint of producing tumor fibrosis and sclerosis, for example, radioactive isotopes, bleomycin, interferon alpha. This method has been reported to produce significant cyst shrinkage, but prospective data are still missing, making it a promising option. A disadvantage of this option is that severe neurotoxicity can occur in some cases due to cystic leakage of the sclerosing substance.

Pearls and Other Issues

The differential diagnosis for craniopharyngioma include:

  1. Other tumors: pituitary adenomas, primitive neuroectodermal tumors, hypothalamic hamartoma, germ cell tumor, epidermoid or dermoid tumor, meningioma, medulloblastoma, brainstem glioma and lymphoma
  2. Other congenital conditions: Rathke's cleft cyst and arachnoid cysts
  3. Inflammatory conditions: pituitary abscess, lymphocytic hypophysitis, infundibulitis, histiocytosis, sarcoidosis, tuberculosis, and syphilis
  4. Vascular malformations: giant suprasellar carotid aneurysm, cavernous sinus hemangioma, and carotid-cavernous fistula.

Enhancing Healthcare Team Outcomes

Craniopharyngiomas are rare benign tumors of the central nervous system (CNS). These patients usually present to the primary care provider or nurse practitioners with a wide array of symptomatology such as headaches, nausea and vomiting, visual disturbances, and endocrine disturbances. It represents a special challenge for the physicians that treat it; these physicians commonly include neurosurgeons, neuro-ophthalmologists, neurologists, endocrinologists, and pediatricians. The challenge is due to the tumor's ability to adhere to the surfaces that surround it. For this reason, it is extremely difficult to control, and it is also notorious for its high rates of recurrence. However, the outcomes in most people are good. [9][10](Level V)


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Craniopharyngioma - Questions

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Which tumor is often associated with Rathke pouch?



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What is the radiological hallmark of a craniopharyngioma on a plain radiograph?



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Which of the following is not on the histological spectrum of a craniopharyngioma?



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What is the MOST sensitive imaging modality to make a diagnosis of a craniopharyngioma?



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What type of tumor often arises from Rathke pouch?



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Which of the following statements about craniopharyngioma is TRUE?



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Which of the following does not present in children with a craniopharyngioma?



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Which of the following findings will NOT be present in children with craniopharyngioma?



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A previously healthy young girl presents with a 2-month history of headaches. She has gained 15 pounds over this time period. She had an episode of emesis today and double vision. She is drowsy and a funduscopic exam shows papilledema, but she has no neck stiffness. Which of the following is the most likely cause?



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A false statement concerning craniopharyngiomas would be:



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A 7-year-old female is brought in with polyuria. The child has had intermittent headaches with associated emesis. The exam show weight is below the 5th percentile and mild papilledema. The glucose is normal, but the first morning urine after fluid restriction shows a specific gravity of 1.005 g/mL. What other finding is likely?



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What is the hallmark appearance of the mass on CT scan in an 8-year-old child suspected of having a craniopharyngioma?



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Craniopharyngioma - References

References

Qi W,Gu F,Wu C, Growth hormone replacement therapy improves hypopituitarism-associated hypoxemia in a patient after craniopharyngioma surgery: A case report. Medicine. 2019 Jan;     [PubMed]
Feng Y,Ni M,Wang YG,Zhong LY, Comparison of neuroendocrine dysfunction in patients with adamantinomatous and papillary craniopharyngiomas. Experimental and therapeutic medicine. 2019 Jan;     [PubMed]
Chen W,Gardner PA,Branstetter BF,Liu SD,Chang YF,Snyderman CH,Goldstein JA,Tyler-Kabara EC,Schuster LA, Long-term impact of pediatric endoscopic endonasal skull base surgery on midface growth. Journal of neurosurgery. Pediatrics. 2019 Jan 11;     [PubMed]
Kiliç M,Can SM,Özdemir B,Tanik C, Management of Craniopharyngioma. The Journal of craniofacial surgery. 2019 Jan 10;     [PubMed]
Mou J,Wang X,Huo G,Ruan L,Jin K,Tan S,Wang F,Hua H,Yang G, Endoscopic endonasal surgery for craniopharyngiomas: a series of 60 patients. World neurosurgery. 2019 Jan 2;     [PubMed]
Fujio S,Juratli TA,Arita K,Hirano H,Nagano Y,Takajo T,Yoshimoto K,Bihun IV,Kaplan AB,Nayyar N,Fink AL,Bertalan MS,Tummala SS,Curry WT Jr,Jones PS,Martinez-Lage M,Cahill DP,Barker FG 2nd,Brastianos PK, A Clinical Rule for Preoperative Prediction of BRAF Mutation Status in Craniopharyngiomas. Neurosurgery. 2018 Nov 27;     [PubMed]
Thompson CJ,Costello RW,Crowley RK, Management of Hypothalamic Disease in Patients with Craniopharyngioma. Clinical endocrinology. 2019 Jan 4;     [PubMed]
Nguyen B,Blasco M,Svider PF,Lin H,Liu JK,Eloy JA,Folbe AJ, Recurrence of Ventral Skull Base Lesions Attributed to Tumor Seeding: A Systematic Review. World neurosurgery. 2018 Dec 31;     [PubMed]
O'steen L,Indelicato DJ, Advances in the management of craniopharyngioma. F1000Research. 2018;     [PubMed]
Li X,Wu W,Miao Q,He M,Zhang S,Zhang Z,Lu B,Yang Y,Shou X,Li Y,Wang Y,Ye H, Endocrine and Metabolic Outcomes After Transcranial and Endoscopic Endonasal Approaches for Primary Resection of Craniopharyngiomas. World neurosurgery. 2019 Jan;     [PubMed]

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