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Craniopharyngioma

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Radiation Oncology for Pediatric CNS Tumors

Abstract

Craniopharyngiomas are rare, histologically benign tumors that arise in the suprasellar space. As noted by Harvey Cushing as early as 1932, these lesions while benign in nature are frequently adherent to adjacent critical structures, which makes complete surgical resection challenging. Because a microscopic gross total resection is rarely obtained, adjuvant radiotherapy plays an important role in the management of these lesions. However, given the proximity of critical normal tissues adjacent to or even within target volumes, radiation therapy may be associated with adverse effects. Both surgical- and radiation-induced adverse effects are compounded by the frequent presence of morbidities arising from the tumor itself. While morbidities are frequent, patients with craniopharyngioma have excellent survival outcomes. Thus, practitioners of all specialties must take great care in treatment planning and survivorship in order to optimize post-treatment outcomes.

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References

  • Aggarwal A, Fersht N, Brada M (2013) Radiotherapy for craniopharyngioma. Pituitary 16:26–33

    Article  PubMed  Google Scholar 

  • Amendola BE, Wolf A, Coy SR et al (2003) Role of radiosurgery in craniopharyngiomas: a preliminary report. Med Pediatr Oncol 41:123–127

    Article  PubMed  Google Scholar 

  • Becker G, Kortmann RD, Skalej M et al (1999) The role of radiotherapy in the treatment of craniopharyngioma–indications, results, side effects. Front Radiat Ther Oncol 33:100–113

    Article  CAS  PubMed  Google Scholar 

  • Bishop AJ, Greenfield B, Mahajan A et al (2014) Proton beam therapy versus conformal photon radiation therapy for childhood craniopharyngioma: multi-institutional analysis of outcomes, cyst dynamics, and toxicity. Int J Radiat Oncol Biol Phys 90:354–361

    Article  PubMed  PubMed Central  Google Scholar 

  • Blackburn TP, Doughty D, Plowman PN (1999) Stereotactic intracavitary therapy of recurrent cystic craniopharyngioma by instillation of 90yttrium. Br J Neurosurg 13:359–365

    Article  CAS  PubMed  Google Scholar 

  • Boehling NS, Grosshans DR, Bluett JB et al (2012) Dosimetric comparison of three-dimensional conformal proton radiotherapy, intensity-modulated proton therapy, and intensity-modulated radiotherapy for treatment of pediatric craniopharyngiomas. Int J Radiat Oncol Biol Phys 82:643–652. Epub 2011 Jan 27

    Article  PubMed  Google Scholar 

  • Brastianos PK, Taylor-Weiner A, Manley PE et al (2014) Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat Genet 46:161–165

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Bunin GR, Surawicz TS, Witman PA et al (1998) The descriptive epidemiology of craniopharyngioma. J Neurosurg 89:547–551

    Article  CAS  PubMed  Google Scholar 

  • Buslei R, Nolde M, Hofmann B et al (2005) Common mutations of beta-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region. Acta Neuropathol 109:589–597

    Article  CAS  PubMed  Google Scholar 

  • Caldarelli M, Massimi L, Tamburrini G et al (2005) Long-term results of the surgical treatment of craniopharyngioma: the experience at the Policlinico Gemelli, Catholic University, Rome. Childs Nerv Syst 21:747–757

    Article  CAS  PubMed  Google Scholar 

  • Cavalheiro S, Di Rocco C, Valenzuela S et al (2010) Craniopharyngiomas: intratumoral chemotherapy with interferon-alpha: a multicenter preliminary study with 60 cases. Neurosurg Focus 28:E12

    Article  PubMed  Google Scholar 

  • Choux M, Lena G (1979) Bases of surgical management of craniopharyngioma in children [proceedings]. Acta Neurochir Suppl (Wien) 28:348

    CAS  Google Scholar 

  • Chung WY, Pan DH, Shiau CY et al (2000) Gamma knife radiosurgery for craniopharyngiomas. J Neurosurg 93(suppl 3):47–56

    PubMed  Google Scholar 

  • Clark AJ, Cage TA, Aranda D et al (2012) Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr 10:293–301

    Article  PubMed  Google Scholar 

  • Clark AJ, Cage TA, Aranda D et al (2013) A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst 29:231–238

    Article  PubMed  Google Scholar 

  • De Vile CJ, Grant DB, Kendall BE et al (1996) Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg 85:73–81

    Article  PubMed  Google Scholar 

  • Duff J, Meyer FB, Ilstrup DM et al (2000) Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery 46:291–302; discussion 302–305

    Article  CAS  PubMed  Google Scholar 

  • Elliott RE, Jane JA Jr, Wisoff JH (2011) Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches. Neurosurgery 69:630–643; discussion 643

    Article  PubMed  Google Scholar 

  • Fahlbusch R, Honegger J, Paulus W et al (1999) Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90:237–250

    Article  CAS  PubMed  Google Scholar 

  • Fernandez-Miranda JC, Gardner PA, Snyderman CH et al (2012) Craniopharyngioma: a pathologic, clinical, and surgical review. Head Neck 34:1036–1044

    Article  PubMed  Google Scholar 

  • Gardner PA, Kassam AB, Snyderman CH et al (2008) Outcomes following endoscopic, expanded endonasal resection of suprasellar craniopharyngiomas: a case series. J Neurosurg 109:6–16

    Article  PubMed  Google Scholar 

  • Greenfield BJ, Okcu MF, Baxter PA et al (2015) Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma. Radiother Oncol 114:224–229. doi:10.1016/j.radonc.2014.11.035 . Epub 2014 Dec 23

    Article  PubMed  Google Scholar 

  • Habrand JL, Ganry O, Couanet D et al (1999) 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 44:255–263

    Article  CAS  PubMed  Google Scholar 

  • Habrand JL, Saran F, Alapetite C et al (2006) Radiation therapy in the management of craniopharyngioma: current concepts and future developments. J Pediatr Endocrinol Metab 19(suppl 1):389–394

    PubMed  Google Scholar 

  • Hoffman HJ, De Silva M, Humphreys RP et al (1992) Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76:47–52

    Article  CAS  PubMed  Google Scholar 

  • Izuora GI, Ikerionwu S, Saddeqi N et al (1989) Childhood intracranial neoplasms Enugu, Nigeria. West Afr J Med 8:171–174

    CAS  PubMed  Google Scholar 

  • Jane JA Jr, Laws ER (2006) Craniopharyngioma. Pituitary 9:323–326

    Article  PubMed  Google Scholar 

  • Kalapurakal JA, Goldman S, Hsieh YC et al (2000) Clinical outcome in children with recurrent craniopharyngioma after primary surgery. Cancer J 6:388–393

    CAS  PubMed  Google Scholar 

  • Karavitaki N, Brufani C, Warner JT et al (2005) Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol 62:397–409

    Article  CAS  Google Scholar 

  • Karavitaki N, Cudlip S, Adams CB et al (2006) Craniopharyngiomas. Endocr Rev 27:371–397

    Article  PubMed  Google Scholar 

  • Khan RB, Merchant TE, Boop FA et al (2013) Headaches in children with craniopharyngioma. J Child Neurol 28:1622–1625

    Article  PubMed  Google Scholar 

  • Kiehna EN, Merchant TE (2010) Radiation therapy for pediatric craniopharyngioma. Neurosurg Focus 28:E10

    Article  PubMed  Google Scholar 

  • Kobayashi T (2009) Long-term results of gamma knife radiosurgery for 100 consecutive cases of craniopharyngioma and a treatment strategy. Prog Neurol Surg 22:63–76

    Article  PubMed  Google Scholar 

  • Lee CC, Yang HC, Chen CJ et al (2014) Gamma knife surgery for craniopharyngioma: report on a 20-year experience. J Neurosurg 121:167–178. doi:10.3171/2014.8.GKS141411

    PubMed  Google Scholar 

  • Leksell L (1952) LK: a therapeutic trial with radioactive isotope in cystic brain tumor: radioisotope technieques I. Med Physiol Appl 1:1–4

    Google Scholar 

  • Liu AK, Bagrosky B, Fenton LZ et al (2009) Vascular abnormalities in pediatric craniopharyngioma patients treated with radiation therapy. Pediatr Blood Cancer 52:227–230

    Article  PubMed  Google Scholar 

  • Lo AC, Howard AF, Nichol A et al (2014) Long-term outcomes and complications in patients with craniopharyngioma: the British Columbia Cancer Agency experience. Int J Radiat Oncol Biol Phys 88:1011–1018

    Article  PubMed  Google Scholar 

  • Lo AC, Howard AF, Nichol A et al (2016) A cross-sectional cohort study of cerebrovascular disease and late effects after radiation therapy for Craniopharyngioma. Pediatr Blood Cancer 63:786–793

    Article  CAS  PubMed  Google Scholar 

  • Luu QT, Loredo LN, Archambeau JO et al (2006) Fractionated proton radiation treatment for pediatric craniopharyngioma: preliminary report. Cancer J 12:155–159

    PubMed  Google Scholar 

  • Marucci G, de Biase D, Zoli M et al (2015) Targeted BRAF and CTNNB1 next-generation sequencing allows proper classification of nonadenomatous lesions of the sellar region in samples with limiting amounts of lesional cells. Pituitary 18:905–911. doi:10.1007/s11102-015-0669-y

    Article  CAS  PubMed  Google Scholar 

  • Merchant TE, Kiehna EN, Sanford RA et al (2002) Craniopharyngioma: the St. Jude Children’s Research Hospital experience 1984-2001. Int J Radiat Oncol Biol Phys 53:533–542

    Article  PubMed  Google Scholar 

  • Merchant TE, Kiehna EN, Kun LE et al (2006) Phase II trial of conformal radiation therapy for pediatric patients with craniopharyngioma and correlation of surgical factors and radiation dosimetry with change in cognitive function. J Neurosurg 104:94–102

    PubMed  Google Scholar 

  • Merchant TE, Kun LE, Hua CH et al (2013) Disease control after reduced volume conformal and intensity modulated radiation therapy for childhood craniopharyngioma. Int J Radiat Oncol Biol Phys 85:e187–e192

    Article  PubMed  Google Scholar 

  • Minniti G, Esposito V, Amichetti M et al (2009) The role of fractionated radiotherapy and radiosurgery in the management of patients with craniopharyngioma. Neurosurg Rev 32:125–132; discussion 132

    Article  CAS  PubMed  Google Scholar 

  • Mokry M (1999) Craniopharyngiomas: a six year experience with gamma knife radiosurgery. Stereotact Funct Neurosurg 72(suppl 1):140–149

    Article  PubMed  Google Scholar 

  • Moore KCW (2000) Craniopharyngioma. In: Bernstein M, Berger M (eds) Neuro-oncology: the essentials. Thieme, New York

    Google Scholar 

  • Mueller S, Fullerton HJ, Stratton K et al (2013) Radiation, atherosclerotic risk factors, and stroke risk in survivors of pediatric cancer: a report from the Childhood Cancer Survivor Study. Int J Radiat Oncol Biol Phys 86:649–655

    Article  PubMed  PubMed Central  Google Scholar 

  • Muller HL (2008) Childhood craniopharyngioma. Recent advances in diagnosis, treatment and follow-up. Horm Res 69:193–202

    PubMed  Google Scholar 

  • Muller HL (2010) Childhood craniopharyngioma: current controversies on management in diagnostics, treatment and follow-up. Expert Rev Neurother 10:515–524

    Article  PubMed  Google Scholar 

  • Muller HL (2013) Childhood craniopharyngioma. Pituitary 16:56–67

    Article  PubMed  Google Scholar 

  • Muller HL (2014) Craniopharyngioma. In: Eliers EKM, Romijn JA (eds) Handbook of clinical neurology. Elsevier, Cambridge

    Google Scholar 

  • Park YS, Chang JH, Park YG et al (2011) Recurrence rates after neuroendoscopic fenestration and Gamma Knife surgery in comparison with subtotal resection and Gamma Knife surgery for the treatment of cystic craniopharyngiomas. J Neurosurg 114:1360–1368. doi:10.3171/2009.9.JNS09301 . Epub 2009 Oct 30

    Article  PubMed  Google Scholar 

  • Pereira AM, Schmid EM, Schutte PJ et al (2005) High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma. Clin Endocrinol 62:197–204

    Article  Google Scholar 

  • Petito CK, DeGirolami U, Earle KM (1976) Craniopharyngiomas: a clinical and pathological review. Cancer 37:1944–1952

    Article  CAS  PubMed  Google Scholar 

  • Pollock BE, Lunsford LD, Kondziolka D et al (1995) Phosphorus-32 intracavitary irradiation of cystic craniopharyngiomas: current technique and long-term results. Int J Radiat Oncol Biol Phys 33:437–446

    Article  CAS  PubMed  Google Scholar 

  • Poretti A, Grotzer MA, Ribi K et al (2004) Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol 46:220–229

    Article  PubMed  Google Scholar 

  • Rath SR, Lee S, Kotecha RS et al (2013) Childhood craniopharyngioma: 20-year institutional experience in Western Australia. J Paediatr Child Health 49:403–408

    Article  PubMed  Google Scholar 

  • Rose SR, Lustig RH, Pitukcheewanont P et al (1999) Diagnosis of hidden central hypothyroidism in survivors of childhood cancer. J Clin Endocrinol Metab 84:4472–4479

    CAS  PubMed  Google Scholar 

  • Samii M, Tatagiba M (1997) Surgical management of craniopharyngiomas: a review. Neurol Med Chir (Tokyo) 37:141–149

    Article  CAS  Google Scholar 

  • Sanford RA (1994) Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg 21(suppl 1):39–43

    PubMed  Google Scholar 

  • Schoenfeld A, Pekmezci M, Barnes MJ et al (2012) The superiority of conservative resection and adjuvant radiation for craniopharyngiomas. J Neuro-Oncol 108:133–139

    Article  Google Scholar 

  • Schubert T, Trippel M, Tacke U et al (2009) Neurosurgical treatment strategies in childhood craniopharyngiomas: is less more? Childs Nerv Syst 25:1419–1427

    Article  PubMed  Google Scholar 

  • Sekine S, Shibata T, Kokubu A et al (2002) Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol 161:1997–2001

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Shi XE, Wu B, Fan T et al (2008) Craniopharyngioma: surgical experience of 309 cases in China. Clin Neurol Neurosurg 110:151–159

    Article  PubMed  Google Scholar 

  • Stiller CA, Nectoux J (1994) International incidence of childhood brain and spinal tumours. Int J Epidemiol 23:458–464

    Article  CAS  PubMed  Google Scholar 

  • Stripp DC, Maity A, Janss AJ et al (2004) Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults. Int J Radiat Oncol Biol Phys 58:714–720

    Article  PubMed  Google Scholar 

  • Sughrue M, Lustig RH, Fisch B et al (2010) Craniopharyngioma. In: Gupta N, Banerjee A, Haas-Kogan D (eds) Pediatric CNS tumors (2nd edn). Springer, Berlin, pp xxv, 357

    Google Scholar 

  • Uto M, Mizowaki T, Ogura K et al (2016) Non-coplanar volumetric-modulated arc therapy (VMAT) for craniopharyngiomas reduces radiation doses to the bilateral hippocampus: a planning study comparing dynamic conformal arc therapy, coplanar VMAT, and non-coplanar VMAT. Radiat Oncol 11:86. doi:10.1186/s13014–016-0659-x

    Article  PubMed  PubMed Central  Google Scholar 

  • Van Effenterre R, Boch AL (2002) Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3–11

    Article  PubMed  Google Scholar 

  • Villani RM, Tomei G, Bello L et al (1997) Long-term results of treatment for craniopharyngioma in children. Childs Nerv Syst 13:397–405

    Article  CAS  PubMed  Google Scholar 

  • Warmuth-Metz M, Gnekow AK, Muller H et al (2004) Differential diagnosis of suprasellar tumors in children. Klin Padiatr 216:323–330

    Article  CAS  PubMed  Google Scholar 

  • Weiner HL, Wisoff JH, Rosenberg ME et al (1994) Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35:1001–1010; discussion 1010–1011

    Article  CAS  PubMed  Google Scholar 

  • Winkfield KM, Linsenmeier C, Yock TI et al (2009) Surveillance of craniopharyngioma cyst growth in children treated with proton radiotherapy. Int J Radiat Oncol Biol Phys 73:716–721

    Article  PubMed  Google Scholar 

  • Xu Z, Yen CP, Schlesinger D et al (2011) Outcomes of Gamma Knife surgery for craniopharyngiomas. J Neurooncol 104:305–313. doi:10.1007/s11060–010–0494-0. Epub 2010 Dec 14

    Article  CAS  PubMed  Google Scholar 

  • Yasargil MG, Curcic M, Kis M et al (1990) Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg 73:3–11

    Article  CAS  PubMed  Google Scholar 

  • Zacharia BE, Bruce SS, Goldstein H et al (2012) Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro-Oncology 14:1070–1078

    Article  PubMed  PubMed Central  Google Scholar 

  • Zona G, Spaziante R (2006) Management of cystic craniopharyngiomas in childhood by a transsphenoidal approach. J Pediatr Endocrinol Metab 19(suppl 1):381–388

    PubMed  Google Scholar 

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Correspondence to David R. Grosshans M.D., Ph.D. .

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Bishop, A.J., Grosshans, D.R. (2018). Craniopharyngioma. In: Mahajan, A., Paulino, A. (eds) Radiation Oncology for Pediatric CNS Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-55430-3_16

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