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Craniopharyngioma
Karen L. Salzman, MD; John H. Rees, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Craniopharyngioma (CP)
          • Synonyms

            • Craniopharyngeal duct tumor, Rathke pouch tumor, adamantinoma
          • Definitions

            • Benign, often partially cystic sellar region tumor derived from Rathke pouch epithelium
              • 2 histologies: Adamantinomatous and papillary

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Greenfield BJ et al: Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma. Radiother Oncol. 114(2):224-9, 2015
                                        2. Kim JH et al: BRAF V600E mutation is a useful marker for differentiating Rathke's cleft cyst with squamous metaplasia from papillary craniopharyngioma. J Neurooncol. ePub, 2015
                                        3. Lee HJ et al: Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients. AJNR Am J Neuroradiol. 36(3):508-17, 2015
                                        4. Sterkenburg AS et al: Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol. ePub, 2015
                                        5. Lee CC et al: Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. J Neurosurg. 121 Suppl:167-78, 2014
                                        6. Clark AJ et al: A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst. 29(2):231-8, 2013
                                        7. Müller HL: Childhood craniopharyngioma. Pituitary. 16(1):56-67, 2013
                                        8. Chentli F et al: Congenital craniopharyngioma: a case report and literature review. J Pediatr Endocrinol Metab. 25(11-12):1181-3, 2012
                                        9. Clark AJ et al: Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr. 10(4):293-301, 2012
                                        10. İnci MF et al: A rare presentation of craniopharyngioma: delayed puberty. BMJ Case Rep. 2012, 2012
                                        11. Shi Z et al: Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation. J Neurooncol. 109(2):349-55, 2012
                                        12. Boongird A et al: Malignant craniopharyngioma; case report and review of the literature. Neuropathology. 29(5):591-6, 2009
                                        13. Frangou EM et al: Metastatic craniopharyngioma: case report and literature review. Childs Nerv Syst. 25(9):1143-7, 2009
                                        14. Keil MF et al: Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics. Expert Rev Neurother. 8(4):563-74, 2008
                                        15. Garrè ML et al: Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 19(4):471-9, 2007
                                        16. Powers CJ et al: Cerebellopontine angle craniopharyngioma: case report and literature review. Pediatr Neurosurg. 43(2):158-63, 2007
                                        17. Rodriguez FJ et al: The spectrum of malignancy in craniopharyngioma. Am J Surg Pathol. 31(7):1020-8, 2007
                                        18. Shuman AG et al: Extracranial nasopharyngeal craniopharyngioma: case report. Neurosurgery. 60(4):E780-1; discussion E781, 2007
                                        19. Aquilina K et al: Primary cerebellopontine angle craniopharyngioma in a patient with gardner syndrome. Case report and review of the literature. J Neurosurg. 105(2):330-3, 2006
                                        20. Haupt R et al: Epidemiological aspects of craniopharyngioma. J Pediatr Endocrinol Metab. 19 Suppl 1:289-93, 2006
                                        21. Prabhu VC et al: The pathogenesis of craniopharyngiomas. Childs Nerv Syst. 21(8-9):622-7, 2005
                                        22. Wang KC et al: Origin of craniopharyngiomas: implication on the growth pattern. Childs Nerv Syst. 21(8-9):628-34, 2005
                                        23. Srinivasan S et al: Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab. 89(1):81-6, 2004
                                        24. Behari S et al: Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature. Surg Neurol. 60(3):245-52; discussion 252-3, 2003
                                        25. Saeki N et al: MR imaging study of edema-like change along the optic tract in patients with pituitary region tumors. AJNR Am J Neuroradiol. 24(3):336-42, 2003
                                        26. Barajas MA et al: Multimodal management of craniopharyngiomas: neuroendoscopy, microsurgery, and radiosurgery. J Neurosurg. 97(5 Suppl):607-9, 2002
                                        27. Fujimoto Y et al: Craniopharyngioma involving the infrasellar region: a case report and review of the literature. Pediatr Neurosurg. 37(4):210-6, 2002
                                        28. Green AL et al: Craniopharyngioma in a mother and daughter. Acta Neurochir (Wien). 144(4):403-4, 2002
                                        29. Sekine S et al: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. 161(6):1997-2001, 2002
                                        30. Van Effenterre R et al: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 97(1):3-11, 2002
                                        31. Chen CJ: Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology. 43(9):760-2, 2001
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Craniopharyngioma (CP)
                                                • Synonyms

                                                  • Craniopharyngeal duct tumor, Rathke pouch tumor, adamantinoma
                                                • Definitions

                                                  • Benign, often partially cystic sellar region tumor derived from Rathke pouch epithelium
                                                    • 2 histologies: Adamantinomatous and papillary

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Staging, Grading, & Classification

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Greenfield BJ et al: Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma. Radiother Oncol. 114(2):224-9, 2015
                                                                              2. Kim JH et al: BRAF V600E mutation is a useful marker for differentiating Rathke's cleft cyst with squamous metaplasia from papillary craniopharyngioma. J Neurooncol. ePub, 2015
                                                                              3. Lee HJ et al: Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients. AJNR Am J Neuroradiol. 36(3):508-17, 2015
                                                                              4. Sterkenburg AS et al: Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. Neuro Oncol. ePub, 2015
                                                                              5. Lee CC et al: Gamma Knife surgery for craniopharyngioma: report on a 20-year experience. J Neurosurg. 121 Suppl:167-78, 2014
                                                                              6. Clark AJ et al: A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst. 29(2):231-8, 2013
                                                                              7. Müller HL: Childhood craniopharyngioma. Pituitary. 16(1):56-67, 2013
                                                                              8. Chentli F et al: Congenital craniopharyngioma: a case report and literature review. J Pediatr Endocrinol Metab. 25(11-12):1181-3, 2012
                                                                              9. Clark AJ et al: Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr. 10(4):293-301, 2012
                                                                              10. İnci MF et al: A rare presentation of craniopharyngioma: delayed puberty. BMJ Case Rep. 2012, 2012
                                                                              11. Shi Z et al: Transient enlargement of craniopharyngioma after radiation therapy: pattern of magnetic resonance imaging response following radiation. J Neurooncol. 109(2):349-55, 2012
                                                                              12. Boongird A et al: Malignant craniopharyngioma; case report and review of the literature. Neuropathology. 29(5):591-6, 2009
                                                                              13. Frangou EM et al: Metastatic craniopharyngioma: case report and literature review. Childs Nerv Syst. 25(9):1143-7, 2009
                                                                              14. Keil MF et al: Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics. Expert Rev Neurother. 8(4):563-74, 2008
                                                                              15. Garrè ML et al: Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 19(4):471-9, 2007
                                                                              16. Powers CJ et al: Cerebellopontine angle craniopharyngioma: case report and literature review. Pediatr Neurosurg. 43(2):158-63, 2007
                                                                              17. Rodriguez FJ et al: The spectrum of malignancy in craniopharyngioma. Am J Surg Pathol. 31(7):1020-8, 2007
                                                                              18. Shuman AG et al: Extracranial nasopharyngeal craniopharyngioma: case report. Neurosurgery. 60(4):E780-1; discussion E781, 2007
                                                                              19. Aquilina K et al: Primary cerebellopontine angle craniopharyngioma in a patient with gardner syndrome. Case report and review of the literature. J Neurosurg. 105(2):330-3, 2006
                                                                              20. Haupt R et al: Epidemiological aspects of craniopharyngioma. J Pediatr Endocrinol Metab. 19 Suppl 1:289-93, 2006
                                                                              21. Prabhu VC et al: The pathogenesis of craniopharyngiomas. Childs Nerv Syst. 21(8-9):622-7, 2005
                                                                              22. Wang KC et al: Origin of craniopharyngiomas: implication on the growth pattern. Childs Nerv Syst. 21(8-9):628-34, 2005
                                                                              23. Srinivasan S et al: Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab. 89(1):81-6, 2004
                                                                              24. Behari S et al: Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature. Surg Neurol. 60(3):245-52; discussion 252-3, 2003
                                                                              25. Saeki N et al: MR imaging study of edema-like change along the optic tract in patients with pituitary region tumors. AJNR Am J Neuroradiol. 24(3):336-42, 2003
                                                                              26. Barajas MA et al: Multimodal management of craniopharyngiomas: neuroendoscopy, microsurgery, and radiosurgery. J Neurosurg. 97(5 Suppl):607-9, 2002
                                                                              27. Fujimoto Y et al: Craniopharyngioma involving the infrasellar region: a case report and review of the literature. Pediatr Neurosurg. 37(4):210-6, 2002
                                                                              28. Green AL et al: Craniopharyngioma in a mother and daughter. Acta Neurochir (Wien). 144(4):403-4, 2002
                                                                              29. Sekine S et al: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. 161(6):1997-2001, 2002
                                                                              30. Van Effenterre R et al: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 97(1):3-11, 2002
                                                                              31. Chen CJ: Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology. 43(9):760-2, 2001