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Papillary craniopharyngioma

MedGen UID:
98125
•Concept ID:
C0431128
•
Neoplastic Process
Synonyms: Craniopharyngioma, Papillary; Craniopharyngiomas, Papillary; Papillary Craniopharyngioma; Papillary Craniopharyngiomas
SNOMED CT: Craniopharyngioma, papillary (128782006); Papillary craniopharyngioma (128782006)
 
Monarch Initiative: MONDO:0002788

Definition

A craniopharyngioma composed of sheets of squamous epithelium which separate to form pseudopapillae. This variant typically lacks nuclear palisading, wet keratin, calcification, and cholesterol deposits. Clinically, endocrine deficiencies are more often associated with papillary craniopharyngioma than with the adamantinomatous type. (Adapted from WHO) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPapillary craniopharyngioma

Professional guidelines

PubMed

Contemporary Biological Insights and Clinical Management of Craniopharyngioma.
Apps JR, Muller HL, Hankinson TC, Yock TI, Martinez-Barbera JP
Endocr Rev 2023 May 8;44(3):518-538. doi: 10.1210/endrev/bnac035. PMID: 36574377
Diagnosis and Management of Pediatric Papillary Craniopharyngiomas.
Zhao C, Zhou Z, Zhang Y, Qi X, Wang X, Lin D, Liu H, Wang Y, Weng C, Chen J, Hu W, Liao L, Lin Z
World Neurosurg 2022 Sep;165:e148-e158. Epub 2022 May 31 doi: 10.1016/j.wneu.2022.05.115. PMID: 35660482
Diagnosis and management of craniopharyngiomas in the era of genomics and targeted therapy.
Martinez-Gutierrez JC, D'Andrea MR, Cahill DP, Santagata S, Barker FG 2nd, Brastianos PK
Neurosurg Focus 2016 Dec;41(6):E2. doi: 10.3171/2016.9.FOCUS16325. PMID: 27903124

Recent clinical studies

Etiology

Endoscopic Supraorbital Translaminar Approach.
Khaleghi M, Wu KC, Prevedello DM
Adv Tech Stand Neurosurg 2024;52:171-182. doi: 10.1007/978-3-031-61925-0_13. PMID: 39017794
Craniopharyngioma-An update on metabolic and cognitive complications and new therapy.
Erfurth EM
J Intern Med 2023 Sep;294(3):269-280. Epub 2023 Jul 9 doi: 10.1111/joim.13684. PMID: 37424210
Contemporary Biological Insights and Clinical Management of Craniopharyngioma.
Apps JR, Muller HL, Hankinson TC, Yock TI, Martinez-Barbera JP
Endocr Rev 2023 May 8;44(3):518-538. doi: 10.1210/endrev/bnac035. PMID: 36574377
Diagnosis and Management of Pediatric Papillary Craniopharyngiomas.
Zhao C, Zhou Z, Zhang Y, Qi X, Wang X, Lin D, Liu H, Wang Y, Weng C, Chen J, Hu W, Liao L, Lin Z
World Neurosurg 2022 Sep;165:e148-e158. Epub 2022 May 31 doi: 10.1016/j.wneu.2022.05.115. PMID: 35660482
Craniopharyngioma: a roadmap for scientific translation.
Gupta S, Bi WL, Giantini Larsen A, Al-Abdulmohsen S, Abedalthagafi M, Dunn IF
Neurosurg Focus 2018 Jun;44(6):E12. doi: 10.3171/2018.3.FOCUS1861. PMID: 29852761

Diagnosis

Craniopharyngioma in Pediatrics and Adults.
Piloni M, Gagliardi F, Bailo M, Losa M, Boari N, Spina A, Mortini P
Adv Exp Med Biol 2023;1405:299-329. doi: 10.1007/978-3-031-23705-8_11. PMID: 37452943
Contemporary Biological Insights and Clinical Management of Craniopharyngioma.
Apps JR, Muller HL, Hankinson TC, Yock TI, Martinez-Barbera JP
Endocr Rev 2023 May 8;44(3):518-538. doi: 10.1210/endrev/bnac035. PMID: 36574377
Diagnosis and Management of Pediatric Papillary Craniopharyngiomas.
Zhao C, Zhou Z, Zhang Y, Qi X, Wang X, Lin D, Liu H, Wang Y, Weng C, Chen J, Hu W, Liao L, Lin Z
World Neurosurg 2022 Sep;165:e148-e158. Epub 2022 May 31 doi: 10.1016/j.wneu.2022.05.115. PMID: 35660482
Craniopharyngioma.
Müller HL, Merchant TE, Warmuth-Metz M, Martinez-Barbera JP, Puget S
Nat Rev Dis Primers 2019 Nov 7;5(1):75. doi: 10.1038/s41572-019-0125-9. PMID: 31699993
Craniopharyngioma: a roadmap for scientific translation.
Gupta S, Bi WL, Giantini Larsen A, Al-Abdulmohsen S, Abedalthagafi M, Dunn IF
Neurosurg Focus 2018 Jun;44(6):E12. doi: 10.3171/2018.3.FOCUS1861. PMID: 29852761

Therapy

Systematic characterization of antibody-drug conjugate targets in central nervous system tumors.
Coy S, Lee JS, Chan SJ, Woo T, Jones J, Alexandrescu S, Wen PY, Sorger PK, Ligon KL, Santagata S
Neuro Oncol 2024 Mar 4;26(3):458-472. doi: 10.1093/neuonc/noad205. PMID: 37870091Free PMC Article
BRAF plus MEK inhibition effective in papillary craniopharyngioma.
Sidaway P
Nat Rev Clin Oncol 2023 Oct;20(10):661. doi: 10.1038/s41571-023-00807-5. PMID: 37491596
Clinical Situation, Therapy, and Follow-Up of Adult Craniopharyngioma.
Mende KC, Kellner T, Petersenn S, Honegger J, Evangelista-Zamora R, Droste M, Stalla G, Deutschbein T, Wang Y, Moskopp D, Knappe U, Schilbach K, Flitsch J
J Clin Endocrinol Metab 2020 Jan 1;105(1) doi: 10.1210/clinem/dgz043. PMID: 31589293
Diagnosis and management of craniopharyngiomas in the era of genomics and targeted therapy.
Martinez-Gutierrez JC, D'Andrea MR, Cahill DP, Santagata S, Barker FG 2nd, Brastianos PK
Neurosurg Focus 2016 Dec;41(6):E2. doi: 10.3171/2016.9.FOCUS16325. PMID: 27903124
ENDOCRINE TUMORS: BRAF V600E mutations in papillary craniopharyngioma.
Brastianos PK, Santagata S
Eur J Endocrinol 2016 Apr;174(4):R139-44. Epub 2015 Nov 12 doi: 10.1530/EJE-15-0957. PMID: 26563980Free PMC Article

Prognosis

Distinction of papillary and adamantinomatous craniopharyngioma: Clinical features, surgical nuances and hypothalamic outcomes.
Yang L, Liu Y, Wang C, Feng Z, Yu L, Pan J, Peng J, Nie J, Zhou M, Ou Y, Liu T, Qi S, Fan J
Neoplasia 2024 Nov;57:101060. Epub 2024 Oct 1 doi: 10.1016/j.neo.2024.101060. PMID: 39357265Free PMC Article
Craniopharyngioma in Pediatrics and Adults.
Piloni M, Gagliardi F, Bailo M, Losa M, Boari N, Spina A, Mortini P
Adv Exp Med Biol 2023;1405:299-329. doi: 10.1007/978-3-031-23705-8_11. PMID: 37452943
Characteristics and factors influencing hypothalamic pituitary dysfunction in patients with craniopharyngioma.
Guo Y, Pei L, Li Y, Li C, Gui S, Ni M, Liu P, Zhang Y, Zhong L
Front Endocrinol (Lausanne) 2023;14:1180591. Epub 2023 Jun 2 doi: 10.3389/fendo.2023.1180591. PMID: 37324266Free PMC Article
Immune infiltration in aggressive papillary craniopharyngioma: High infiltration but low action.
Jia Y, Ma L, Cai K, Zhang B, Wu W, Xiao Y, Qiao N, Ru S, Cao L, Gao H, Gui S
Front Immunol 2022;13:995655. Epub 2022 Nov 1 doi: 10.3389/fimmu.2022.995655. PMID: 36389809Free PMC Article
Clinical Situation, Therapy, and Follow-Up of Adult Craniopharyngioma.
Mende KC, Kellner T, Petersenn S, Honegger J, Evangelista-Zamora R, Droste M, Stalla G, Deutschbein T, Wang Y, Moskopp D, Knappe U, Schilbach K, Flitsch J
J Clin Endocrinol Metab 2020 Jan 1;105(1) doi: 10.1210/clinem/dgz043. PMID: 31589293

Clinical prediction guides

Distinction of papillary and adamantinomatous craniopharyngioma: Clinical features, surgical nuances and hypothalamic outcomes.
Yang L, Liu Y, Wang C, Feng Z, Yu L, Pan J, Peng J, Nie J, Zhou M, Ou Y, Liu T, Qi S, Fan J
Neoplasia 2024 Nov;57:101060. Epub 2024 Oct 1 doi: 10.1016/j.neo.2024.101060. PMID: 39357265Free PMC Article
Immune infiltration in aggressive papillary craniopharyngioma: High infiltration but low action.
Jia Y, Ma L, Cai K, Zhang B, Wu W, Xiao Y, Qiao N, Ru S, Cao L, Gao H, Gui S
Front Immunol 2022;13:995655. Epub 2022 Nov 1 doi: 10.3389/fimmu.2022.995655. PMID: 36389809Free PMC Article
Papillary Craniopharyngioma: A Type of Tumor Primarily Impairing the Hypothalamus - A Comprehensive Anatomo-Clinical Characterization of 350 Well-Described Cases.
Prieto R, Barrios L, Pascual JM
Neuroendocrinology 2022;112(10):941-965. Epub 2021 Dec 28 doi: 10.1159/000521652. PMID: 35108706
Biological Behaviour of Craniopharyngiomas.
Martinez-Barbera JP, Andoniadou CL
Neuroendocrinology 2020;110(9-10):797-804. Epub 2020 Mar 4 doi: 10.1159/000506904. PMID: 32126562
Clinical Situation, Therapy, and Follow-Up of Adult Craniopharyngioma.
Mende KC, Kellner T, Petersenn S, Honegger J, Evangelista-Zamora R, Droste M, Stalla G, Deutschbein T, Wang Y, Moskopp D, Knappe U, Schilbach K, Flitsch J
J Clin Endocrinol Metab 2020 Jan 1;105(1) doi: 10.1210/clinem/dgz043. PMID: 31589293

Recent systematic reviews

Papillary Craniopharyngioma: A Type of Tumor Primarily Impairing the Hypothalamus - A Comprehensive Anatomo-Clinical Characterization of 350 Well-Described Cases.
Prieto R, Barrios L, Pascual JM
Neuroendocrinology 2022;112(10):941-965. Epub 2021 Dec 28 doi: 10.1159/000521652. PMID: 35108706
Association of histological subtype with risk of recurrence in craniopharyngioma patients: a systematic review and meta-analysis.
Wu J, Wu X, Yang YQ, Ding H, Yang L, Bao YY, Zhou L, Yang CX, Hong T
Neurosurg Rev 2022 Feb;45(1):139-150. Epub 2021 Jun 22 doi: 10.1007/s10143-021-01563-9. PMID: 34159471

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