Pediatric Craniopharyngioma 

  • Author: Joseph L Lasky III, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Aug 11, 2010
 

Background

Craniopharyngiomas (see the image below) are histologically benign neuroepithelial tumors of the CNS that are predominately observed in children aged 5-10 years.

This MRI sequence was obtained following the intraThis MRI sequence was obtained following the intravenous administration of gadolinium contrast. Observe the relatively homogeneous and cystic mass arising from the sella turcica and extending superiorly and posteriorly with compression of normal regional structures. Note that the lesion is sharply demarcated and smoothly contoured. This fluid-filled mass is consistent with a typical craniopharyngioma.

These tumors arise from squamous cell embryologic rests found along the path of the primitive adenohypophysis and craniopharyngeal duct. Although histologically benign, these tumors frequently recur after treatment. In addition, because they originate near critical intracranial structures (eg, visual pathways, pituitary gland, hypothalamus), both the tumor and complications of curative therapy can cause significant morbidity. These characteristics have led to various treatment approaches, and disagreement continues regarding optimal treatment in children with this disease.

Evidence suggests that adult craniopharyngiomas are histologically and biologically different from pediatric craniopharyngiomas; however, only childhood craniopharyngiomas are discussed in this article.

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Pathophysiology

Pediatric craniopharyngiomas are believed to arise from cellular remnants of the Rathke pouch, which is an embryologic structure that forms both the infundibulum and anterior lobe of the pituitary gland. Its path of development extends from the pharynx to the floor of the sella turcica; not surprisingly, these tumors have been identified extensively in suprasellar, parasellar, and ectopic locations.[1] Typically, the tumors arise within the sella or adjacent suprasellar space. Symptoms are caused by mass effects on adjacent normal intracranial structures.

Little is known regarding the genetic basis for craniopharyngiomas. Transformation of normal cells into neoplastic ones likely involves multiple genomic changes, including loss of tumor-suppressor genes, activation of oncogenes, and alterations in DNA repair and methylation mechanisms. Although these events have started to be elucidated for neuroepithelial neoplasms such as gliomas, little progress has been made in understanding these events in craniopharyngiomas. Some chromosomal abnormalities, including deletions, translocations, and increased copy numbers, have been recognized but are largely nonspecific.[2] The significance of these findings remains to be determined.

However, studies have identified the beta-catenin pathway as playing a potential role in the pathogenesis of these tumors.[3] Beta-catenin is a downstream component of the Wnt signal transduction pathway that plays critical roles in the regulation of cellular proliferation, morphology, and development. One study showed that the accumulation of nuclear beta-catenin as measured immunohistochemically was able to help differentiate craniopharyngiomas from Rathke cleft cysts.[4] Other molecular factors that have been identified in aggressive or recurrent craniopharyngiomas are low expression levels of macrophage-inhibiting factor (MIF) and galectins, retinoic-acid receptor levels, low cathepsin levels, and high vascular-endothelial growth factor (VEGF) levels.[5]

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Epidemiology

Frequency

United States

Craniopharyngiomas are relatively rare, representing 6-10% of intracranial malignancies in children and adolescents (approximately 2-3 cases per 1,000,000 children). A bimodal distribution peak has been reported, with one peak at age 5-14 years and the other at age 65-74 years. More than 300 cases are reported in the United States annually, and roughly one third of these involve children aged 0-14 years.[3, 6, 7] Craniopharyngiomas are the most common childhood tumor that occur in the sella-chiasmatic region.[2]

International

The Childhood Cancer Registry of Piedmont, Italy estimates an incidence of 1.4 cases per million children per year in keeping with reports from other Western countries. Higher incidence rates have been observed in Asia and Africa with 5.25 cases per million children reported in Japan in one series.[8]

Mortality/Morbidity

Previous studies have shown relatively good outcomes, with 10-year overall survival rates of 86-100% among patients who underwent gross total resection. Subtotal resection or recurrence treated with surgery and radiation therapy carry 10-year overall survival rates of 57-86%. The perioperative mortality rate after primary surgical intervention has been estimated to be 1.7-5.4%. However, the mortality rate after re-resection for recurrent disease can be as high as 25%.[2]

Presenting morbidities include the following:

  • Visual loss: Anterior extension to the optic chiasm can result in a classic bitemporal hemianopsia, unilateral temporal hemianopsia, papilledema, or unilateral/bilateral decrease in visual acuity. Children are frequently inattentive to visual loss, and formal testing may be required.
  • Endocrinologic derangement: This results from direct compression or destruction of the hypothalamus and pituitary stalk, leading to growth hormone deficiency, thyroid-stimulating hormone deficiency, adrenocorticotropic hormone deficiency, antidiuretic hormone deficiency, and luteinizing hormone or follicle-stimulating hormone deficiency (See Clinical and Complications). This can present as clinically significant short stature, hypothyroidism, diabetes insipidus, and other signs of panhypopituitarism.[6]

Race

No clear racial predilection has been reported.

Sex

The most recent large series demonstrate equal sex distribution, although a slight male preponderance has been historically reported.

Age

Peak incidence of childhood craniopharyngiomas occurs in individuals aged 5-14 years. Neonatal craniopharyngiomas are rare. Of the more than 300 cases per year in the United States, approximately one third involve children aged 0-14 years. The incidence of adult craniopharyngiomas has a second peak in individuals aged 50-74 years.

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Contributor Information and Disclosures
Author

Joseph L Lasky III, MD  Clinical Assistant Professor of Pediatrics and Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine; Physician Specialist, Division of Pediatric Hematology/Oncology, Harbor-UCLA Medical Center

Joseph L Lasky III, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology Institute, UCLA

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Jerry L Barker, Jr, MD  Staff Physician, Clinical Associate Professor of Radiation Oncology, Department of Radiation Oncology, University of Texas Southwestern Moncrief Cancer Center

Jerry L Barker, Jr, MD is a member of the following medical societies: American Society for Therapeutic Radiology and Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

  1. Prabhu VC, Brown HG. The pathogenesis of craniopharyngiomas. Childs Nerv Syst. Aug 2005;21(8-9):622-7. [Medline].

  2. Karavitaki N, Wass JA. Craniopharyngiomas. Endocrinol Metab Clin North Am. Mar 2008;37(1):173-93, ix-x. [Medline].

  3. Garre ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. Aug 2007;19(4):471-9. [Medline].

  4. Hofmann BM, Kreutzer J, Saeger W, et al. Nuclear beta-catenin accumulation as reliable marker for the differentiation between cystic craniopharyngiomas and rathke cleft cysts: a clinico-pathologic approach. Am J Surg Pathol. Dec 2006;30(12):1595-603. [Medline].

  5. Pettorini BL, Frassanito P, Caldarelli M, Tamburrini G, Massimi L, Di Rocco C. Molecular pathogenesis of craniopharyngioma: switching from a surgical approach to a biological one. Neurosurg Focus. Apr 2010;28(4):E1. [Medline].

  6. Halac I, Zimmerman D. Endocrine manifestations of craniopharyngioma. Childs Nerv Syst. Aug 2005;21(8-9):640-8. [Medline].

  7. Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg. Oct 1998;89(4):547-51. [Medline].

  8. Kuratsu J, Ushio Y. Epidemiological study of primary intracranial tumors in childhood. A population-based survey in Kumamoto Prefecture, Japan. Pediatr Neurosurg. Nov 1996;25(5):240-6; discussion 247. [Medline].

  9. Jakacki RI, Cohen BH, Jamison C, et al. Phase II evaluation of interferon-alpha-2a for progressive or recurrent craniopharyngiomas. J Neurosurg. Feb 2000;92(2):255-60. [Medline].

  10. Takahashi H, Yamaguchi F, Teramoto A. Long-term outcome and reconsideration of intracystic chemotherapy with bleomycin for craniopharyngioma in children. Childs Nerv Syst. Aug 2005;21(8-9):701-4. [Medline].

  11. Liu AK, Bagrosky B, Fenton LZ, Gaspar LE, Handler MH, McNatt SA. Vascular abnormalities in pediatric craniopharyngioma patients treated with radiation therapy. Pediatr Blood Cancer. Oct 20 2008;52(2):227-230. [Medline].

  12. Laws ER Jr, Morris AM, Maartens N. Gliadel for pituitary adenomas and craniopharyngiomas. Neurosurgery. Aug 2003;53(2):255-69; discussion 259-60. [Medline].

  13. Wen BC, Hussey DH, Staples J, et al. A comparison of the roles of surgery and radiation therapy in the management of craniopharyngiomas. Int J Radiat Oncol Biol Phys. Jan 1989;16(1):17-24. [Medline].

  14. Kiehna EN, Merchant TE. Radiation therapy for pediatric craniopharyngioma. Neurosurg Focus. Apr 2010;28(4):E10. [Medline].

  15. Rodriguez FJ, Scheithauer BW, Tsunoda S, Kovacs K, Vidal S, Piepgras DG. The spectrum of malignancy in craniopharyngioma. Am J Surg Pathol. Jul 2007;31(7):1020-8. [Medline].

  16. Allen ED, Byrd SE, Darling CF, et al. The clinical and radiological evaluation of primary brain tumors in children, Part I: Clinical evaluation. J Natl Med Assoc. Jun 1993;85(6):445-51. [Medline].

  17. Baskin DS, Wilson CB. Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg. Jul 1986;65(1):22-7. [Medline].

  18. Blethen SL. Growth in children with a craniopharyngioma. Pediatrician. 1987;14(4):242-5. [Medline].

  19. Cavalheiro S, Dastoli PA, Silva NS, et al. Use of interferon alpha in intratumoral chemotherapy for cystic craniopharyngioma. Childs Nerv Syst. Aug 2005;21(8-9):719-24. [Medline].

  20. Duffner PK, Cohen ME, Freeman AI. Pediatric brain tumors: an overview. CA Cancer J Clin. Sep-Oct 1985;35(5):287-301. [Medline].

  21. Dunbar SF, Tarbell NJ, Kooy HM, et al. Stereotactic radiotherapy for pediatric and adult brain tumors: preliminary report. Int J Radiat Oncol Biol Phys. Oct 15 1994;30(3):531-9. [Medline].

  22. Fisher PG, Jenab J, Gopldthwaite PT, et al. Outcomes and failure patterns in childhood craniopharyngiomas. Childs Nerv Syst. Oct 1998;14(10):558-63. [Medline].

  23. Garrè ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. Aug 2007;19(4):471-9. [Medline].

  24. Gonzales-Portillo G, Tomita T. The syndrome of inappropriate secretion of antidiuretic hormone: an unusual presentation for childhood craniopharyngioma: report of three cases. Neurosurgery. Apr 1998;42(4):917-21; discussion 921-2. [Medline].

  25. Habrand JL, Ganry O, Couanet D, et al. The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys. May 1 1999;44(2):255-63. [Medline].

  26. Kollias SS, Barkovich AJ, Edwards MS. Magnetic resonance analysis of suprasellar tumors of childhood. Pediatr Neurosurg. 1991-92;17(6):284-303. [Medline].

  27. Miller DC. Pathology of craniopharyngiomas: clinical import of pathological findings. Pediatr Neurosurg. 1994;21 Suppl 1:11-7. [Medline].

  28. Mori K, Handa H, Murata T, et al. Results of treatment for craniopharyngioma. Childs Brain. 1980;6(6):303-12. [Medline].

  29. Rajan B, Ashley S, Thomas DG, et al. Craniopharyngioma: improving outcome by early recognition and treatment of acute complications. Int J Radiat Oncol Biol Phys. Feb 1 1997;37(3):517-21. [Medline].

  30. Regine WF, Kramer S. Pediatric craniopharyngiomas: long term results of combined treatment with surgery and radiation. Int J Radiat Oncol Biol Phys. 1992;24(4):611-7. [Medline].

  31. Regine WF, Mohiuddin M, Kramer S. Long-term results of pediatric and adult craniopharyngiomas treated with combined surgery and radiation. Radiother Oncol. Apr 1993;27(1):13-21. [Medline].

  32. Sanford RA. Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg. 1994;21 Suppl 1:39-43. [Medline].

  33. Sanford RA, Muhlbauer MS. Craniopharyngioma in children. Neurol Clin. May 1991;9(2):453-65. [Medline].

  34. Shiminski-Maher T, Rosenberg M. Late effects associated with treatment of craniopharyngiomas in childhood. J Neurosci Nurs. Aug 1990;22(4):220-6. [Medline].

  35. Tomita T. Management of craniopharyngiomas in children. Pediatr Neurosci. 1988;14(4):204-11. [Medline].

  36. Tomita T, McLone DG. Radical resections of childhood craniopharyngiomas. Pediatr Neurosurg. 1993;19(1):6-14. [Medline].

  37. Tsao MN, Wara WM, Larson DA. Radiation therapy for benign central nervous system disease. Semin Radiat Oncol. Apr 1999;9(2):120-33. [Medline].

  38. Wilson CB. Diagnosis and surgical treatment of childhood brain tumors. Cancer. Mar 1975;35(3 suppl):950-6. [Medline].

  39. Yasargil MG, Curcic M, Kis M, et al. Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg. Jul 1990;73(1):3-11. [Medline].

 
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This MRI sequence was obtained following the intravenous administration of gadolinium contrast. Observe the relatively homogeneous and cystic mass arising from the sella turcica and extending superiorly and posteriorly with compression of normal regional structures. Note that the lesion is sharply demarcated and smoothly contoured. This fluid-filled mass is consistent with a typical craniopharyngioma.
This axial CT scan image demonstrates a cystic lesion in the typical location of a craniopharyngioma. Although most of the lesion is fluid filled, a rim of enhancing soft tissue is observed following the administration of intravenous contrast.
 
 
 
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