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Pediatric craniopharyngioma: a 20-year study on epidemiological features, clinical presentation, and survival outcomes in a tertiary care center from LMIC

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Abstract

Introduction

Craniopharyngioma constitutes approximately 10% of primary brain tumors in children. It can cause considerable morbidity and mortality due to the local aggressiveness of the tumor itself or its management affecting the hypothalamus-pituitary axis and optic pathway involvement. There is very scarce data available from LMIC which makes the management controversial where multidisciplinary teams are already not available in most of the centers. This is a single-center cross-sectional retrospective review of 20-year record of 49 patients with craniopharyngioma treated between 2001 and 2020 at Aga Khan University Hospital, a tertiary care center in Karachi, Pakistan.

Methods

We have assessed the epidemiological data of children presenting with the diagnosis of craniopharyngioma, treatment modalities used, and neurological, endocrine, and hypothalamic complications in these patients. The assessment involved a retrospective review of medical records and medical follow-up.

Results

Out of a total of 49 patients, 26 (53%) were male, and 23 (46.9%) were female. The mean age was 9.5 years (SD ± 4.5 years). Most common symptoms at initial presentation were headache 41 (83.6%), visual deficit 40 (81.6%), nausea and vomiting 26 (53%), and endocrine abnormalities 16 (32%). Treatment modalities used at our center include gross total resection 11 (22%) and subtotal resection 38 (77%) out of total, while 6 (12.2%) patients received intracystic interferon. Histopathologic findings of the majority of patients (40 (81%)) revealed an adamantinomatous type of tumor. Only 23 (46.9%) children followed in clinic post-op. Median follow-up after craniopharyngioma presentation was 5 years (± 2.1 SD, range: 2–10 years). Pituitary hormone deficiencies (98%) and visual disturbances (75%) were the most common long-term health conditions observed.

Conclusions

Since pituitary hormone deficiencies and visual disturbance were the most common long-term health conditions observed in our study, these patients require a multidisciplinary team follow-up to improve their quality of life.

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Abbreviations

STR:

Subtotal resection

GTR:

Gross total resection

LMIC:

Low- or middle-income country

BMI:

Body mass index

References

  1. Hoffman HJ (1994) Surgical management of craniopharyngioma. Pediatr Neurosurg 21(Suppl. 1):44–49

    Article  PubMed  Google Scholar 

  2. Muller HL (2008) Childhood craniopharyngioma. Recent advances in diagnosis, treatment, and follow-up. Hormone Res 69(4):193–202

  3. Müller HL (2010) Childhood craniopharyngioma—current concepts in diagnosis, therapy, and follow-up. Nat Rev Endocrinol 6(11):609–618

    Article  PubMed  Google Scholar 

  4. Poretti A, Grotzer MA, Ribi K, Schönle E, Boltshauser E (2004) Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol 46(4):220–229

    Article  PubMed  Google Scholar 

  5. Fischer EG, Welch K, Shillito J, Winston KR, Tarbell NJ (1990) Craniopharyngiomas in children: long-term effects of conservative surgical procedures combined with radiation therapy. J Neurosurg 73(4):534–540

    Article  CAS  PubMed  Google Scholar 

  6. Grewal MR, Spielman DB, Safi C, Overdevest JB, Otten M, Bruce J et al (2020) Gross total versus subtotal surgical resection in the management of craniopharyngiomas. Allergy Rhinol 11:2152656720964158

    Article  Google Scholar 

  7. Hoffman HJ, Hendrick EB, Humphreys RP, Buncic JR, Armstrong DL, Jenkin RD (1977) Management of craniopharyngioma in children. J Neurosurg 47(2):218–227

    Article  CAS  PubMed  Google Scholar 

  8. Haroon S, Afzal A, Zia S, Ali SJ, Zia F, Shamail F et al (2021) Clinicopathological features of craniopharyngioma: a 15-year study from a tertiary care center in Pakistan. Cureus 13(3):e14153. https://doi.org/10.7759/cureus.14153

  9. Khalid MU, Shah MM, Bajwa MH, Mirza FA, Laghari AA (2022) Craniopharyngioma: a lower-middle-income-country epidemiology perspective. J Pak Med Assoc 72(Suppl 4)(11):S61–S67

  10. Mazher S, Ashraf J (2015) Craniopharyngioma total or near total surgical resection: an outcome-based learning experience. Pak J Med Dent 4(01):10

    Google Scholar 

  11. Drapeau A, Walz PC, Eide JG, Rugino AJ, Shaikhouni A, Mohyeldin A et al (2019) Pediatric craniopharyngioma. Child’s Nervous. System 35:2133–2145

    Google Scholar 

  12. Lithgow K, Hamblin R, Pohl U, Karavitaki N (2022) Craniopharyngiomas. Endotext. http://www.endotext.org/

  13. Hoffmann A, Boekhoff S, Gebhardt U, Sterkenburg AS, Daubenbüchel AM, Eveslage M et al (2015) History before diagnosis in childhood craniopharyngioma: associations with initial presentation and long-term prognosis. Eur J Endocrinol 173(6):853–862

    Article  CAS  PubMed  Google Scholar 

  14. Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML, Blaser SI (1992) Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76(1):47–52

    Article  CAS  PubMed  Google Scholar 

  15. Müller H, Gebhardt U, Maroske J, Hanisch E (2011) Long-term follow-up of morbidly obese patients with childhood craniopharyngioma after laparoscopic adjustable gastric banding (LAGB). Klin Padiatr 223(06):372–373

    Article  PubMed  Google Scholar 

  16. Yaşargil MG, Curcic M, Kis M, Siegenthaler G, Teddy PJ, Roth P (1990) Total removal of craniopharyngiomas: approaches and long-term results in 144 patients. J Neurosurg 73(1):3–11

    Article  PubMed  Google Scholar 

  17. Abrams LS, Repka MX (1997) Visual outcome of craniopharyngioma in children. J Pediatr Ophthalmol Strabismus 34(4):223–228

  18. Mortini P, Losa M, Pozzobon G, Barzaghi R, Riva M, Acerno S et al (2011) Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series. J Neurosurg 114(5):1350–1359

    Article  PubMed  Google Scholar 

  19. Kumar S, Stecher G, Li M, Knyaz C, Tamura K (2018) MEGA X: molecular evolutionary genetics analysis across computing platforms. Mol Biol Evol 35(6):1547

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. DeVile CJ, Grant DB, Hayward RD, Stanhope R (1996) Growth and endocrine sequelae of craniopharyngioma. Arch Dis Child 75(2):108–114

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Daneman D, Hoffman HJ, Ehrlich RM (1994) The endocrine outcome after surgical removal of craniopharyngiomas. Pediatr Neurosurg 21(1):24–27

    Article  PubMed  Google Scholar 

  22. Gonc EN, Yordam N, Ozon A, Alikasifoglu A, Kandemir N (2004) Endocrinological outcome of different treatment options in children with craniopharyngioma: a retrospective analysis of 66 cases. Pediatr Neurosurg 40(3):112–119

    Article  PubMed  Google Scholar 

  23. Karavitaki N, Brufani C, Warner J, Adams C, Richards P, Ansorge O et al (2005) Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol 62(4):397–409

    Article  CAS  Google Scholar 

  24. Stripp DC, Maity A, Janss AJ, Belasco JB, Tochner ZA, Goldwein JW 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(3):714–20

  25. Webb KL, Pruter WW, Hinkle ML, Walsh MT (2023) Comparing surgical approaches for craniopharyngioma resection among adults and children: a meta-analysis and systematic review. World Neurosurg 175:e876–e896

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Acknowledgements

No individuals other than the listed co-authors contributed to this publication.

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Authors and Affiliations

  1. Department of Pediatrics, Aga Khan University, Karachi, Pakistan

    Fozia Memon, Khadija Nuzhat Humayun & Muzna Arif

  2. Department of Pediatric Oncology, Indus Hospital, Karachi, Pakistan

    Quratulain Riaz

  3. Aga Khan Medical College, Karachi, Pakistan

    Noor ul Huda

  4. Section of Neurosurgery, Department of Surgery, Aga Khan University, Karachi, Pakistan

    Altaf Ali Laghari

  5. Department of Radiology, Aga Khan University, Karachi, Pakistan

    Kiran Hilal

  6. Department of Oncology, Aga Khan University, Karachi, Pakistan

    Naureen Mushtaq

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  1. Fozia Memon
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  2. Khadija Nuzhat Humayun
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  6. Altaf Ali Laghari
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Contributions

Dr Naureen Mushtaq and Dr Fozia Memon wrote the main manuscript text. Dr Khadija Nuzhat Humayun conceptualized the idea and did proofreading. Dr Kiran Hilal added the radiology section and reviewed the article. Dr Altaf Laghari and Dr Muzna Arif reviewed the article and did the final correction. All authors read and approved the final manuscript with corrections.

Corresponding author

Correspondence to Naureen Mushtaq.

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The retrospective data collection of this study has been approved by the institutional ethics committee (2022-7168-20979).

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Memon, F., Humayun, K.N., Riaz, Q. et al. Pediatric craniopharyngioma: a 20-year study on epidemiological features, clinical presentation, and survival outcomes in a tertiary care center from LMIC. Childs Nerv Syst 40, 427–434 (2024). https://doi.org/10.1007/s00381-023-06177-8

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  • DOI: https://doi.org/10.1007/s00381-023-06177-8

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