CoChat
Sign Up
Cited by 4Pituitary Gland Disorders and TreatmentsNeuroendocrine Tumor Research AdvancesGlioma Diagnosis and Treatment

Retrospective dataset and survey analyses identify gaps in data collection for craniopharyngioma and priorities of patients and families affected by the disease

Open access
Marshall, Emily, Joshi, Nikhil, Crowley, Julia, McCormack, Shana, Cheng, Sylvia, Faig, Walter, Storm, Phillip B., Resnick, Adam +3 more·2023·Neoplasia
DOI 10.1016/j.neo.2022.100873PMC PMC9852952
Open this paper in CoChat — free account

Abstract

INTRODUCTION: Craniopharyngioma is a rare, low-grade tumor located in the suprasellar region of the brain, near critical structures like the pituitary gland. Here, we concurrently investigate the status of clinical and genomic data in a retrospective craniopharyngioma cohort and survey-based data to better understand patient-relevant outcomes associated with existing therapies and provide a foundation to inform new treatment strategies. METHODS: Clinical, genomic, and outcome data for a retrospective cohort of patients with craniopharyngioma were collected and reviewed through the Children's Brain Tumor Network (CBTN) database. An anonymous survey was distributed to patients and families with a diagnosis of craniopharyngioma to understand their experiences throughout diagnosis and treatment. RESULTS: The CBTN repository revealed a large proportion of patients (40 - 70%) with specimens that are available for sequencing but lacked relevant quality of life (QoL) and functional outcomes. Frequencies of reported patient comorbidities ranged from 20-35%, which is significantly lower than historically reported. Survey results from 159 patients/families identified differences in treatment considerations at time of diagnosis versus time of recurrence. In retrospective review, patients and families identified preference for therapy that would improve QoL, rather than decrease risk of recurrence (mean 3.9 vs. 4.4 of 5) and identified endocrine issues as having the greatest impact on patients' lives. CONCLUSIONS: This work highlights the importance of prospective collection of QoL and functional metrics alongside robust clinical and molecular correlates in individuals with craniopharyngioma. Such comprehensive measures will facilitate biologically relevant therapeutic strategies that also prioritize patient needs.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Integrity signals

Registry

Crossref · 10.1016/j.neo.2022.100873

Cites

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

A few free checks a day · papers already verified open instantly · results are public

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Hypothalamic syndrome represents a substantial burden for patients and families.

    “Hypothalamic syndrome represents a substantial burden for patients and families (5-8), and correspondingly, poses a considerable challenge to clinicians.”

    From Approach to the patient with acquired hypothalamic syndrome (2026) · cites this paper as [6]

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Cites

No references could be resolved yet.

Research works printed but not linked: 40

  1. [1]Buslei R. Nolde M. Hofmann B. Common mutations of beta-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region Acta Neuropathol. 109 6 2005 589 597 10.1007/s00401-005-1004-x 15891929 · DOI
  2. [2]Müller H.L. Craniopharyngioma Handb. Clin. Neurol. 124 2014 235 253 10.1016/B978-0-444-59602-4.00016-2 25248591 · DOI
  3. [3]Wang G. Zhang X. Feng M. Guo F. Comparing survival outcomes of gross total resection and subtotal resection with radiotherapy for craniopharyngioma: a meta-analysis J. Surg. Res. 226 2018 131 139 10.1016/j.jss.2018.01.029 29661278 · DOI
  4. [4]Akinduro O.O. Izzo A. Lu V.M. Endocrine and visual outcomes following gross total resection and subtotal resection of adult craniopharyngioma: systematic review and meta-analysis World Neurosurg. 127 2019 e656 e668 10.1016/j.wneu.2019.03.239 30947004 · DOI
  5. [5]Wan M.J. Zapotocky M. Bouffet E. Bartels U. Kulkarni A.V. Drake JM. Long-term visual outcomes of craniopharyngioma in children J. Neurooncol. 137 3 2018 645 651 10.1007/s11060-018-2762-3 29344823 · DOI
  6. [6]Lucas J.T. Jr Faught A.M. Hsu C.Y. Pre- and posttherapy risk factors for vasculopathy in pediatric patients with craniopharyngioma treated with surgery and proton radiation therapy Int. J. Radiat. Oncol. Biol. Phys. 113 1 2022 152 160 10.1016/j.ijrobp.2021.12.172 34990778 PMC9018579 · DOI
  7. [7]Beer-Furlan A. Abi-Hachem R. Goksel B. Otero J.J. Carrau R.L. Prevedello D.M. Letter: radiation-induced malignant transformation of craniopharyngiomas Neurosurgery 79 2 2016 E313 E315 10.1227/NEU.0000000000001292 27434162 · DOI
  8. [8]Hamblin R. Vardon A. Akpalu J. Risk of second brain tumour after radiotherapy for pituitary adenoma or craniopharyngioma: a retrospective, multicentre, cohort study of 3679 patients with long-term imaging surveillance Lancet Diabetes Endocrinol. 10 8 2022 581 588 10.1016/S2213-8587(22)00160-7 35780804 · DOI
  9. [9]Koutourousiou M. Gardner P.A. Fernandez-Miranda J.C. Tyler-Kabara E.C. Wang E.W. Snyderman C.H. Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients J. Neurosurg. 119 5 2013 1194 1207 10.3171/2013.6.JNS122259 23909243 · DOI
  10. [10]Elliott R.E. Jane J.A. Jr Wisoff J.H. Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches Neurosurgery 69 3 2011 630 643 10.1227/NEU.0b013e31821a872d 21499159 · DOI
  11. [11]Lohkamp L.N. Kulkarni A.V. Drake J.M. Preservation of endocrine function after Ommaya reservoir insertion in children with cystic craniopharyngioma J. Neurooncol. 159 3 2022 597 607 10.1007/s11060-022-04099-0 35925530 · DOI
  12. [12]Kilday J.P. Caldarelli M. Massimi L. Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN Neuro. Oncol. 19 10 2017 1398 1407 10.1093/neuonc/nox056 28499018 PMC5596165 · DOI
  13. [13]Zhou Z. Zhang S. Hu F. Endocrine disorder in patients with craniopharyngioma Front. Neurol. 12 2021 737743 10.3389/fneur.2021.737743 Published 2021 Dec 2 PMC8675636 34925209 · DOI
  14. [14]Lustig RH. Hypothalamic obesity after craniopharyngioma: mechanisms, diagnosis, and treatment Front. Endocrinol. (Lausanne) 2 2011 60 10.3389/fendo.2011.00060 Published 2011 Nov 3 22654817 PMC3356006 · DOI
  15. [15]Müller H.L. Merchant T.E. Warmuth-Metz M. Martinez-Barbera J.P. Puget S. Craniopharyngioma Nat. Rev. Dis. Prim. 5 1 2019 75 10.1038/s41572-019-0125-9 Published 2019 Nov 7 31699993 · DOI
  16. [16]Yano S. Kudo M. Hide T. Quality of life and clinical features of long-term survivors surgically treated for pediatric craniopharyngioma World Neurosurg. 85 2016 153 162 10.1016/j.wneu.2015.08.059 26341425 · DOI
  17. [17]Clark A.J. Cage T.A. Aranda D. Parsa A.T. Auguste K.I. Gupta N. Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review J Neurosurg. Pediatr. 10 4 2012 293 301 22920295
  18. [18]Daubenbüchel A.M. Müller HL. Neuroendocrine disorders in pediatric craniopharyngioma patients J. Clin. Med. 4 3 2015 389 413 10.3390/jcm4030389 Published 2015 Mar 9 26239246 PMC4470135 · DOI
  19. [19]Sands S.A. Milner J.S. Goldberg J. Quality of life and behavioral follow-up study of pediatric survivors of craniopharyngioma J. Neurosurg. 103 4 Suppl 2005 302 311 10.3171/ped.2005.103.4.0302 16270681 · DOI
  20. [20]Manley P.E. McKendrick K. McGillicudy M. Sleep dysfunction in long term survivors of craniopharyngioma J. Neurooncol. 108 3 2012 543 549 10.1007/s11060-012-0859-7 22528788 · DOI
  21. [23]Petralia F. Tignor N. Reva B. Integrated proteogenomic characterization across major histological types of pediatric brain cancer Cell 183 7 2020 1962 1985.e31 10.1016/j.cell.2020.10.044 33242424 PMC8143193 · DOI · Paper page
  22. [24]Zacharia B.E. Bruce S.S. Goldstein H. Malone H.R. Neugut A.I. Bruce JN. Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program Neuro. Oncol. 14 8 2012 1070 1078 10.1093/neuonc/nos142 22735773 PMC3408265 · DOI
  23. [25]Craven M. Crowley J.H. Chiang L. A survey of patient-relevant outcomes in pediatric craniopharyngioma: focus on hypothalamic obesity Front. Endocrinol. (Lausanne) 13 2022 876770 10.3389/fendo.2022.876770 Published 2022 May 9 PMC9124861 35615720 · DOI · Paper page
  24. [26]Clusmann H. Höllig A. Craniopharyngioma: the benefits of a conservative approach Dtsch. Arztebl. Int. 116 18 2019 319 320 10.3238/arztebl.2019.0319 31219032 PMC6620758 · DOI
  25. [27]Schoenfeld A. Pekmezci M. Barnes M.J. The superiority of conservative resection and adjuvant radiation for craniopharyngiomas J. Neurooncol. 108 1 2012 133 139 10.1007/s11060-012-0806-7 22350375 PMC3879154 · DOI
  26. [28]Cohen M. Bartels U. Branson H. Kulkarni A.V. Hamilton J. Trends in treatment and outcomes of pediatric craniopharyngioma, 1975–2011 Neuro. Oncol. 15 6 2013 767 774 10.1093/neuonc/not026 23486689 PMC3661103 · DOI
  27. [29]Merchant T.E. Edmonston D.Y. Wu S. Li Y. Boop F.A. Lustig R.H. Endocrine outcomes after limited surgery and conformal photon radiation therapy for pediatric craniopharyngioma: long-term results from the RT1 Protocol [published online ahead of print, 2022 Apr 27] Neuro. Oncol. 2022 noac115 10.1093/neuonc/noac115 PMC9713512 35481829 · DOI
  28. [30]Rao Y.J. Hassanzadeh C. Fischer-Valuck B. Patterns of care and treatment outcomes of patients with Craniopharyngioma in the national cancer database J. Neurooncol. 132 1 2017 109 117 10.1007/s11060-016-2342-3 28012064 · DOI
  29. [31]Romigi A. Feola T. Cappellano S. Sleep disorders in patients with craniopharyngioma: a physiopathological and practical update Front. Neurol. 12 2022 817257 10.3389/fneur.2021.817257 Published 2022 Feb 9 PMC8863754 35222233 · DOI · Paper page
  30. [32]Cordani R. Veneruso M. Napoli F. Sleep disturbances in craniopharyngioma: a challenging diagnosis [published correction appears in J Neurol. 2021 Sep 24;:] J. Neurol. 268 11 2021 4362 4369 10.1007/s00415-021-10794-1 34522989 PMC8439529 · DOI
  31. [33]Müller HL. Childhood craniopharyngioma – current concepts in diagnosis, therapy and follow-up Nat. Rev. Endocrinol. 6 11 2010 609 618 10.1038/nrendo.2010.168 20877295 · DOI
  32. [34]Visser J. Hukin J. Sargent M. Steinbok P. Goddard K. Fryer C. Late mortality in pediatric patients with craniopharyngioma J. Neurooncol. 100 1 2010 105 111 10.1007/s11060-010-0145-5 20204458 · DOI
  33. [35]Steinbok P. Craniopharyngioma in children: long-term outcomes Neurol. Med. Chir. 55 9 2015 722 726 10.2176/nmc.ra.2015-0099 PMC4605079 26345668 · DOI
  34. [36]Wijnen M. Olsson D.S. van den Heuvel-Eibrink M.M. Excess morbidity and mortality in patients with craniopharyngioma: a hospital-based retrospective cohort study Eur. J. Endocrinol. 178 1 2018 93 102 10.1530/EJE-17-0707 29046325 · DOI
  35. [37]Tan T.S.E. Patel L. Gopal-Kothandapani J.S. The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres Eur. J. Endocrinol. 176 3 2017 359 369 10.1530/EJE-16-0812 28073908 · DOI
  36. [38]Gautier A. Godbout A. Grosheny C. Markers of recurrence and long-term morbidity in craniopharyngioma: a systematic analysis of 171 patients J. Clin. Endocrinol. Metab. 97 4 2012 1258 1267 10.1210/jc.2011-2817 22319039 · DOI
  37. [39]Lo A.C. Howard A.F. Nichol A. Long-term outcomes and complications in patients with Craniopharyngioma: the British Columbia cancer agency experience Int. J. Radiat. Oncol. Biol. Phys. 88 5 2014 1011 1018 10.1016/j.ijrobp.2014.01.019 24661653 · DOI
  38. [40]Poretti A. Grotzer M.A. Ribi K. Schönle E. Boltshauser E. Outcome of craniopharyngioma in children: long-term complications and quality of life Dev. Med. Child Neurol. 46 4 2004 220 229 10.1017/s0012162204000374 15077699 · DOI
  39. [41]Pereira A.M. Schmid E.M. Schutte P.J. High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma Clin. Endocrinol. (Oxf) 62 2 2005 197 204 10.1111/j.1365-2265.2004.02196.x 15670196 · DOI
  40. [42]Zada G. Kintz N. Pulido M. Amezcua L. Prevalence of neurobehavioral, social, and emotional dysfunction in patients treated for childhood craniopharyngioma: a systematic literature review PLoS One 8 11 2013 e76562 10.2176/nmc.ra.2015-0099 Published 2013 Nov 5. 24223703 PMC3818366 · DOI

Cited by

Sorted by citations

Authors
Marshall, E., Joshi, N., Crowley, J., McCormack, S., Cheng, S., Faig, W., Storm, P. B., Resnick, A., Mueller, S., Malbari, F., & Kline, C.
Affiliations
  • Thomas Jefferson University (US)
  • University of Pennsylvania (US)
  • Children's Hospital of Philadelphia (US)
  • BC Children's Hospital (CA)
  • University of California, San Francisco (US)
  • Baylor College of Medicine (US)
Corresponding author
Kline, Cassie
ORCID
Venue
Neoplasia
Publisher
Elsevier BV
Published
2023-03
Volume
37
Pages
100873
ISSN
1476-5586 (ISSN-L)
DOI
10.1016/j.neo.2022.100873
PMC
PMC9852952
OpenAlex
W4317360156
Cited by
4
References
0
Citations by year
2026: 3 · 2025: 1
FWCI
0.82
Type
journal article
Language
en
Open access
Licence
Locations
  • Neoplasia (publishedVersion, cc-by-nc-nd)
  • PubMed Central (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • The Medicine Forum (submittedVersion, cc-by-nc-nd)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
Kortney Rose Foundation
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Genetics › Glioma Diagnosis and Treatment
Keywords
craniopharyngioma, quality of life, functional outcome, patient and family experiences, treatment preferences, tumor recurrence, endocrine dysfunction, comorbidities, pediatric brain tumors
MeSH terms
Child, Craniopharyngioma, Data Collection, Humans, Pituitary Neoplasms, Prospective Studies, Quality of Life, Retrospective Studies

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review