MCID: PPL009
MIFTS: 28

Papillary Craniopharyngioma

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Papillary Craniopharyngioma

MalaCards integrated aliases for Papillary Craniopharyngioma:

Name: Papillary Craniopharyngioma 12 15 17 70
Papillary Rathke's Pouch Tumor 12
Craniopharyngioma, Papillary 12
Craniopharyngioma Papillary 54

Classifications:



External Ids:

Disease Ontology 12 DOID:3847
MeSH 44 D003397
NCIt 50 C4725
SNOMED-CT 67 134215002
UMLS 70 C0431128

Summaries for Papillary Craniopharyngioma

MalaCards based summary : Papillary Craniopharyngioma, also known as papillary rathke's pouch tumor, is related to craniopharyngioma and gallbladder adenoma, and has symptoms including visual disturbance An important gene associated with Papillary Craniopharyngioma is CTNNB1 (Catenin Beta 1), and among its related pathways/superpathways is Oncogenic MAPK signaling. The drugs Vemurafenib and Protein Kinase Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include pituitary and pineal.

Related Diseases for Papillary Craniopharyngioma

Diseases related to Papillary Craniopharyngioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 79)
# Related Disease Score Top Affiliating Genes
1 craniopharyngioma 30.8 MMP20 KRT7 ENAM CTNNB1 BRAF
2 gallbladder adenoma 10.3 KRT7 CTNNB1
3 vaginal adenoma 10.3 KRT7 CTNNB1
4 vaginal benign neoplasm 10.3 KRT7 CTNNB1
5 endosalpingiosis 10.3 KRT7 BRAF
6 adenomyoma 10.3 KRT7 CTNNB1
7 intrahepatic bile duct adenoma 10.3 KRT7 CTNNB1
8 metanephric adenoma 10.3 KRT7 BRAF
9 sclerosing hemangioma 10.3 KRT7 CTNNB1
10 adenofibroma 10.2 KRT7 BRAF
11 pulmonary sclerosing hemangioma 10.2 KRT7 CTNNB1
12 female reproductive endometrioid cancer 10.2 KRT7 CTNNB1
13 mature teratoma 10.2 KRT7 BRAF
14 ovary adenocarcinoma 10.2 KRT7 CTNNB1 BRAF
15 endocervical carcinoma 10.2 KRT7 CTNNB1
16 colorectal adenocarcinoma 10.2 KRT7 CTNNB1 BRAF
17 optic nerve neoplasm 10.2 KIAA1549 FAM131B
18 birt-hogg-dube syndrome 10.2 KRT7 CTNNB1
19 jalili syndrome 10.2 MMP20 ENAM
20 thyroid gland follicular carcinoma 10.2 KRT7 CTNNB1 BRAF
21 adamantinous craniopharyngioma 10.1 MMP20 ENAM CTNNB1
22 pseudomyxoma peritonei 10.1 KRT7 CTNNB1
23 renal cell carcinoma, papillary, 1 10.1 KRT7 CTNNB1 BRAF
24 hypopituitarism 10.1
25 cerebrovascular benign neoplasm 10.1 PCDHGB4 KRT7
26 villous adenoma 10.1 KRT7 BRAF
27 dentine erosion 10.1 ENAM AMELX
28 pulp degeneration 10.1 ENAM AMELX
29 dental pulp calcification 10.1 ENAM AMELX
30 dysembryoplastic neuroepithelial tumor 10.1 KIAA1549 FAM131B
31 mixed cell type cancer 10.1 KRT7 CTNNB1
32 tooth erosion 10.1 ENAM AMELX
33 amelogenesis imperfecta, type ib 10.1 ENAM AMELX
34 childhood pilocytic astrocytoma 10.1 KIAA1549 FAM131B BRAF
35 juvenile astrocytoma 10.1 KIAA1549 FAM131B BRAF
36 enamel erosion 10.1 MMP20 AMELX
37 pleomorphic xanthoastrocytoma 10.1 KIAA1549 FAM131B BRAF
38 cholangiocarcinoma 10.1 KRT7 CTNNB1 BRAF
39 diffuse astrocytoma 10.1 KIAA1549 FAM131B BRAF
40 central nervous system benign neoplasm 10.0 KIAA1549 FAM131B CTNNB1
41 hydrocephalus 10.0
42 obstructive hydrocephalus 10.0
43 pituitary adenoma 10.0
44 adenoma 10.0
45 diabetes insipidus 10.0
46 pituitary tumors 10.0
47 amelogenesis imperfecta hypomaturation type 10.0 MMP20 AMELX
48 respiratory system benign neoplasm 10.0 KRT7 CTNNB1
49 malignant astrocytoma 10.0 KIAA1549 CTNNB1 BRAF
50 amelogenesis imperfecta, type ic 9.9 MMP20 ENAM AMELX

Graphical network of the top 20 diseases related to Papillary Craniopharyngioma:



Diseases related to Papillary Craniopharyngioma

Symptoms & Phenotypes for Papillary Craniopharyngioma

UMLS symptoms related to Papillary Craniopharyngioma:


visual disturbance

Drugs & Therapeutics for Papillary Craniopharyngioma

Drugs for Papillary Craniopharyngioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vemurafenib Approved Phase 2 918504-65-1 42611257 23252090
2 Protein Kinase Inhibitors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Trial of BRAF/MEK Inhibitors in Papillary Craniopharyngiomas Recruiting NCT03224767 Phase 2 Vemurafenib;Cobimetinib

Search NIH Clinical Center for Papillary Craniopharyngioma

Genetic Tests for Papillary Craniopharyngioma

Anatomical Context for Papillary Craniopharyngioma

MalaCards organs/tissues related to Papillary Craniopharyngioma:

40
Pituitary, Pineal

Publications for Papillary Craniopharyngioma

Articles related to Papillary Craniopharyngioma:

(show top 50) (show all 68)
# Title Authors PMID Year
1
Establishing a papillary craniopharyngioma cell line by SV40LT-mediated immortalization. 61
33044631 2021
2
Intrinsic Third Ventricular Papillary Craniopharyngioma: A Report of Five Cases and Literature Review. 61
33623444 2021
3
Successful Use of BRAF/MEK Inhibitors as a Neoadjuvant Approach in the Definitive Treatment of Papillary Craniopharyngioma. 61
33285519 2020
4
Pituitary Adenoma and Papillary Craniopharyngioma: A Rare Case of Collision Tumor and Review of the Literature. 61
32298831 2020
5
Adamantinomatous Craniopharyngioma in an Adult: A Case Report with NGS Analysis. 61
32368160 2020
6
Clinical Situation, Therapy, and Follow-Up of Adult Craniopharyngioma. 61
31589293 2020
7
Craniopharyngioma. 61
31699993 2019
8
Dual BRAF/MEK therapy in BRAF V600E-mutated primary brain tumors: a case series showing dramatic clinical and radiographic responses and a reduction in cutaneous toxicity. 61
31675726 2019
9
Newly diagnosed papillary craniopharyngioma with BRAF V600E mutation treated with single-agent selective BRAF inhibitor dabrafenib: a case report. 61
31666933 2019
10
Papillary Craniopharyngioma in a Young Child: The Importance of BRAF Mutational Testing. 61
31796130 2019
11
Awake Surgical Management of Third Ventricular Tumors: A Preliminary Safety, Feasibility, and Clinical Applications Study. 61
30753637 2019
12
Intratumoral Rathke's Cleft Cyst Remnants Within Craniopharyngioma, Pituitary Adenoma, Suprasellar Dermoid, and Epidermoid Cysts: A Ubiquitous Signature of Ectodermal Lineage or a Transitional Entity? 61
30010935 2019
13
Clinical features and operative technique of transinfundibular craniopharyngioma. 61
31200374 2019
14
Craniopharyngiomas and odontogenic tumors mimic normal odontogenesis and share genetic mutations, histopathologic features, and molecular pathways activation. 61
30598409 2019
15
Papillary craniopharyngioma in a 4-year-old girl with BRAF V600E mutation: a case report and review of the literature. 61
30069716 2019
16
Genetic and immune profiling for potential therapeutic targets in adult human craniopharyngioma. 61
31712784 2019
17
Noninvasive molecular diagnosis of craniopharyngioma with MRI-based radiomics approach. 61
30616515 2019
18
Clinical and biological significance of adamantinomatous craniopharyngioma with CTNNB1 mutation. 61
30074466 2018
19
Multiplexed immunofluorescence reveals potential PD-1/PD-L1 pathway vulnerabilities in craniopharyngioma. 61
29509940 2018
20
The diagnostic value of preoperative inflammatory markers in craniopharyngioma: a multicenter cohort study. 61
29388032 2018
21
Recurrent papillary craniopharyngioma with BRAF V600E mutation treated with dabrafenib: case report. 61
29701552 2018
22
WHO 2016 classification: changes and advancements in the diagnosis of miscellaneous primary CNS tumours. 61
28295484 2018
23
Extended Endoscopic Approach for Resection of Craniopharyngiomas. 61
29404251 2018
24
High-resolution melting and immunohistochemical analysis efficiently detects mutually exclusive genetic alterations of adamantinomatous and papillary craniopharyngiomas. 61
28840946 2018
25
Rathke's Cleft Cyst as Origin of a Pediatric Papillary Craniopharyngioma. 61
29520296 2018
26
Recurrent papillary craniopharyngioma with BRAFV600E mutation treated with neoadjuvant-targeted therapy. 61
28918496 2017
27
MAPK pathway control of stem cell proliferation and differentiation in the embryonic pituitary provides insights into the pathogenesis of papillary craniopharyngioma. 61
28506993 2017
28
Papillary craniopharyngioma: A clinicopathologic study of a rare entity from a major tertiary care center in Pakistan. 61
28488624 2017
29
BRAF-V600E mutant papillary craniopharyngioma dramatically responds to combination BRAF and MEK inhibitors. 61
28425764 2017
30
Genomic Alterations of Adamantinomatous and Papillary Craniopharyngioma. 61
28069929 2017
31
Anorexia-cachexia syndrome-like hypothalamic neuroendocrine dysfunction in a patient with a papillary craniopharyngioma. 61
28469924 2017
32
[A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma]. 61
29076472 2017
33
Diagnosis and management of craniopharyngiomas in the era of genomics and targeted therapy. 61
27903124 2016
34
Potential evolution of neurosurgical treatment paradigms for craniopharyngioma based on genomic and transcriptomic characteristics. 61
27903126 2016
35
Distinct patterns of primary and motile cilia in Rathke's cleft cysts and craniopharyngioma subtypes. 61
27562488 2016
36
Analysis and Long-Term Follow-Up of the Surgical Treatment of Children With Craniopharyngioma. 61
28005815 2016
37
Pronounced response of papillary craniopharyngioma to treatment with vemurafenib, a BRAF inhibitor. 61
26115708 2016
38
TREM-1 expression in craniopharyngioma and Rathke's cleft cyst: its possible implication for controversial pathology. 61
27409178 2016
39
ENDOCRINE TUMORS: BRAF V600E mutations in papillary craniopharyngioma. 61
26563980 2016
40
Dramatic Response of BRAF V600E Mutant Papillary Craniopharyngioma to Targeted Therapy. 61
26498373 2016
41
Lack of BRAF-V600E Mutation in Papillary Tumor of the Pineal Region. 61
26125673 2015
42
BRAF V600E mutation is a useful marker for differentiating Rathke's cleft cyst with squamous metaplasia from papillary craniopharyngioma. 61
25820214 2015
43
Cross-reactivity of the BRAF VE1 antibody with epitopes in axonemal dyneins leads to staining of cilia. 61
25412847 2015
44
Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients. 61
25339645 2015
45
Superficial siderosis of the central nervous system caused by hemorrhagic intraventricular craniopharyngioma: case report and literature review. 61
24670310 2015
46
Does the calcification of adamantinomatous craniopharyngioma resemble the calcium deposition of osteogenesis/odontogenesis? 61
24387671 2014
47
BRAF V600E mutations are characteristic for papillary craniopharyngioma and may coexist with CTNNB1-mutated adamantinomatous craniopharyngioma. 61
24715106 2014
48
Association of craniopharyngioma and pituitary adenoma. 61
23377700 2013
49
Primary isolated cerebellopontine angle papillary craniopharyngioma. 61
22954990 2012
50
Expression of aberrant beta-catenin and impaired p63 in craniopharyngiomas. 54
20128632 2010

Variations for Papillary Craniopharyngioma

Expression for Papillary Craniopharyngioma

Search GEO for disease gene expression data for Papillary Craniopharyngioma.

Pathways for Papillary Craniopharyngioma

Pathways related to Papillary Craniopharyngioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.93 KIAA1549 FAM131B BRAF

GO Terms for Papillary Craniopharyngioma

Cellular components related to Papillary Craniopharyngioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cilium GO:0005929 9.46 KIAA1549 DNAH7 DNAH2 DNAH12
2 axonemal dynein complex GO:0005858 8.96 DNAH7 DNAH2
3 dynein complex GO:0030286 8.8 DNAH7 DNAH2 DNAH12

Biological processes related to Papillary Craniopharyngioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 biomineral tissue development GO:0031214 9.26 ENAM AMELX
2 cilium-dependent cell motility GO:0060285 9.16 DNAH7 DNAH2
3 amelogenesis GO:0097186 8.96 MMP20 ENAM
4 microtubule-based movement GO:0007018 8.8 DNAH7 DNAH2 DNAH12

Molecular functions related to Papillary Craniopharyngioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 motor activity GO:0003774 9.43 DNAH7 DNAH2 DNAH12
2 dynein intermediate chain binding GO:0045505 9.37 DNAH7 DNAH2
3 microtubule motor activity GO:0003777 9.33 DNAH7 DNAH2 DNAH12
4 dynein light intermediate chain binding GO:0051959 9.32 DNAH7 DNAH2
5 structural constituent of tooth enamel GO:0030345 8.96 ENAM AMELX
6 ATP-dependent microtubule motor activity, minus-end-directed GO:0008569 8.8 DNAH7 DNAH2 DNAH12

Sources for Papillary Craniopharyngioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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