Purpose of This PDQ Summary
General Information
Background Information About Childhood Craniopharyngioma

Incidence and Presentation Prognosis

Histopathologic Classification of Childhood Craniopharyngioma
Diagnostic Evaluation of Childhood Craniopharyngioma
Stage Information
Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

Radical Surgery Limited Surgery and Radiation Therapy Intracavitary Radiation Therapy and/or Chemotherapy

Treatment Options for Recurrent Childhood Craniopharyngioma
Late Effects in Patients Treated for Childhood Craniopharyngioma
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Changes to the Summary (03/05/2009)
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Purpose of This PDQ Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood craniopharyngioma. This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board ¹.

Information about the following is included in this summary:

• Clinical presentation.
• Prognosis.
• Diagnostic evaluation.
• Histopathologic classification.
• Treatment options.
• Late effects.

This summary is intended as a resource to inform and assist clinicians and other health professionals who care for pediatric cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric and Adult Treatment Editorial Boards use a formal evidence ranking system ² in developing their level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for reimbursement determinations.

This summary is also available in a patient version ³, which is written in less technical language, and in Spanish ⁴.

General Information

The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. The PDQ childhood brain tumor treatment summaries are organized primarily according to the 2000 World Health Organization classification of nervous system tumors.[1]

In recent decades, dramatic improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer ⁵ for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)

Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification. (Refer to the PDQ summary on Childhood Brain and Spinal Cord Tumors Overview ⁶ for information about the general classification of childhood brain and spinal cord tumors.)

References

1. Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000. 
   
   

Background Information About Childhood Craniopharyngioma

Incidence and Presentation

Craniopharyngiomas are relatively rare pediatric tumors, accounting for about 6% of all intracranial tumors in children.[1] They are believed to be congenital in origin, and may arise from embryonic remnants. No predisposing factors have been identified.

Because craniopharyngiomas occur in the region of the pituitary gland, endocrine function and growth may be affected. Additionally, the close proximity of the tumor to the optic nerves and chiasm may result in vision problems. Some patients present with obstructive hydrocephalus due to tumor obstruction of the third ventricle.

Long-term survival for children with craniopharyngioma is generally good. Regardless of the treatment modality, long-term survival is approximately 79%.[2]

References

1. Bunin GR, Surawicz TS, Witman PA, et al.: The descriptive epidemiology of craniopharyngioma. J Neurosurg 89 (4): 547-51, 1998.  [PUBMED Abstract]
   
   
2. Sanford RA, Muhlbauer MS: Craniopharyngioma in children. Neurol Clin 9 (2): 453-65, 1991.  [PUBMED Abstract]
   
   

Histopathologic Classification of Childhood Craniopharyngioma

Craniopharyngiomas are histologically benign and do not metastasize to remote brain locations or to areas outside the sellar region except by direct extension. They may be invasive, however, and may recur locally. They may be classified as adamantinomous or squamous papillary, with the former being the predominant form in children.[1] They are typically composed of both a solid portion with an abundance of calcification, and a cystic component which is filled with a dark, oily fluid.

References

1. Miller DC: Pathology of craniopharyngiomas: clinical import of pathological findings. Pediatr Neurosurg 21 (Suppl 1): 11-7, 1994.  [PUBMED Abstract]
   
   

Diagnostic Evaluation of Childhood Craniopharyngioma

The results of imaging studies (computerized tomography scans and magnetic resonance imaging [MRI] scans) are often diagnostic for craniopharyngiomas, with most demonstrating intratumoral calcifications. Craniopharyngiomas without calcification may be confused with other tumor types, such as germinoma or hypothalamic/chiasmatic astrocytoma, and biopsy may be required.[1] Magnetic resonance spectroscopy may be diagnostically helpful in some cases.[2] MRI of the spinal axis is not routinely performed.

Apart from imaging, patients often undergo formal visual examination including visual field evaluation, and endocrine testing.

References

1. Harwood-Nash DC: Neuroimaging of childhood craniopharyngioma. Pediatr Neurosurg 21 (Suppl 1): 2-10, 1994.  [PUBMED Abstract]
   
   
2. Sutton LN, Wang ZJ, Wehrli SL, et al.: Proton spectroscopy of suprasellar tumors in pediatric patients. Neurosurgery 41 (2): 388-94; discussion 394-5, 1997.  [PUBMED Abstract]
   
   

Stage Information

There is no generally applied staging system for childhood craniopharyngiomas. Patients are classified as having newly diagnosed or recurrent disease.

Treatment Options for Newly Diagnosed Childhood Craniopharyngioma

There is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma. Little data exists to compare the different modalities in terms of recurrence rate or quality of life.[1] For this reason, treatment is individualized.

Because these tumors are histologically benign, it may be possible to remove all the visible tumor resulting in long-term disease control. Many surgical approaches have been described, and the route should be determined by the size, location, and extension of the tumor. A transsphenoidal approach may be possible in some small tumors located entirely within the sella, but this is not usually possible in children, in which case a craniotomy is usually required.

Gross total resection is technically challenging because the tumor is surrounded by vital structures, including the optic nerves and chiasm, the carotid artery and its branches, the hypothalamus, and the third cranial nerve. The tumor may be adherent to these structures, which may cause complications, and may limit the ability to remove all the tumor. The surgeon often has limited visibility in the region of the hypothalamus and in the sella, and portions of the mass may be left in these areas, 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.[2]

Complications of radical surgery include the need for hormone replacement, obesity, alteration in mood, blindness, seizures, spinal fluid leak, false aneurysms,[3] and difficulty with eye movements. Rare complications include death from intraoperative hemorrhage, hypothalamic damage, or stroke.

If the surgeon feels that tumor remains, or if postoperative imaging reveals residual craniopharyngioma that is not resectable, radiation therapy is generally recommended to prevent early progression. Periodic surveillance magnetic resonance imaging is performed for several years after radical surgery because of the possibility of tumor recurrence.

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. The surgical procedure is followed by radiation therapy. Conventional radiation is fractionated external-beam radiation with a recommended dose of 54 Gy to 55 Gy in 1.8 Gy fractions.[4] Surgical complications are less likely than with radical surgery. Complications of radiation include loss of pituitary hormonal function, development of late strokes and vascular malformations, delayed blindness, and development of second tumors within the radiation field. Tumor progression remains a possibility, and it is usually not possible to repeat the radiation dose. In selected cases, stereotactic radiation therapy can be delivered as a single large dose of radiation to a very small field. Proximity of the craniopharyngioma to vital structures limits this to very small tumors that are in the sella.[5]

Some craniopharyngiomas with a large cystic component may be treated by stereotaxic delivery of 32P, a radioactive substance with a very short penetration. This is usually considered for recurrent tumors.[5]

References

1. Sanford RA: Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg 21 (Suppl 1): 39-43, 1994.  [PUBMED Abstract]
   
   
2. Sands SA, Milner JS, Goldberg J, et al.: Quality of life and behavioral follow-up study of pediatric survivors of craniopharyngioma. J Neurosurg 103 (4 Suppl): 302-11, 2005.  [PUBMED Abstract]
   
   
3. Sutton LN: Vascular complications of surgery for craniopharyngioma and hypothalamic glioma. Pediatr Neurosurg 21 (Suppl 1): 124-8, 1994.  [PUBMED Abstract]
   
   
4. Wara WM, Sneed PK, Larson DA: The role of radiation therapy in the treatment of craniopharyngioma. Pediatr Neurosurg 21 (Suppl 1): 98-100, 1994.  [PUBMED Abstract]
   
   
5. Lunsford LD, Pollock BE, Kondziolka DS, et al.: Stereotactic options in the management of craniopharyngioma. Pediatr Neurosurg 21 (Suppl 1): 90-7, 1994.  [PUBMED Abstract]
   
   

Treatment Options for Recurrent Childhood Craniopharyngioma

Recurrence of craniopharyngioma occurs with all modalities of primary therapy. Management is determined in large part by prior therapy. Repeat attempts at gross total resection are difficult, and complications are more frequent than with initial surgery.[1] External-beam radiation therapy is an option if this has not been previously employed. Cystic recurrences may be treated with intracavitary instillation of radioactive 32P, or bleomycin,[2] and a reservoir may be placed to permit intermittent outpatient aspiration. Chemotherapy is generally not utilized.

References

1. Wisoff JH: Surgical management of recurrent craniopharyngiomas. Pediatr Neurosurg 21 (Suppl 1): 108-13, 1994.  [PUBMED Abstract]
   
   
2. Takahashi H, Nakazawa S, Shimura T: Evaluation of postoperative intratumoral injection of bleomycin for craniopharyngioma in children. J Neurosurg 62 (1): 120-7, 1985.  [PUBMED Abstract]
   
   

Late Effects in Patients Treated for Childhood Craniopharyngioma

Quality-of-life issues are important in this group of patients, and are difficult to assess due to various treatment modalities. Whereas intelligence quotient is usually maintained, behavioral issues and memory deficits attributed to the frontal lobe and hypothalamus are common, and occur in about 13% to 55% of patients in various series.[1] Other common problems include visual loss, obesity, and the almost universal need for life-long endocrine replacement with multiple pituitary hormones.

Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer ⁵ for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.

References

1. Hoffman HJ, De Silva M, Humphreys RP, et al.: Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76 (1): 47-52, 1992.  [PUBMED Abstract]
   
   

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Changes to the Summary (03/05/2009)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

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