MCID: CRN036
MIFTS: 59

Craniopharyngioma malady

Categories: Rare diseases, Neuronal diseases, Endocrine diseases, Cancer diseases

Aliases & Classifications for Craniopharyngioma

Aliases & Descriptions for Craniopharyngioma:

Name: Craniopharyngioma 12 50 56 52 42 14 69
Dysodontogenic Epithelial Tumor 50
Craniopharyngeal Duct Tumor 50
Neoplasm of Rathke's Pouch 12
Adamantinomatous Tumor 50
Rathke's Pouch Tumor 50

Characteristics:

Orphanet epidemiological data:

56
craniopharyngioma
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe),1-9/1000000 (United States),1-9/1000000 (Sweden); Age of onset: All ages;

Classifications:



External Ids:

Disease Ontology 12 DOID:3840
MeSH 42 D003397
NCIt 47 C2964
SNOMED-CT 64 189179009 40009002
Orphanet 56 ORPHA54595
ICD10 via Orphanet 34 D44.4
MESH via Orphanet 43 D003397
UMLS via Orphanet 70 C0010276
UMLS 69 C0010276

Summaries for Craniopharyngioma

NIH Rare Diseases : 50 craniopharyngioma is a slow-growing benign (not cancerous) brain tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain which produces several important hormones) and the hypothalamus (an endocrine organ which controls the release of hormones by the pituitary gland). this tumor most commonly affects children between 5 and 10 years of age; however, adults can sometimes be affected. although craniopharyngiomas are not cancerous, they may grow and press on nearby parts of the brain, causing symptoms including hormonal changes, vision changes, slow growth, headaches, nausea and vomiting, loss of balance, and hearing loss. the cause of these tumors is not well understood; however, researchers suspect that they begin to form during the early stages of embryo development in pregnancy (embryogenesis) and may result from metaplasia (abnormal transformation of cells). treatment for craniopharyngioma varies and may include: surgery to remove the tumor, radiation therapy, chemotherapy, and biologic therapy.  last updated: 3/31/2017

MalaCards based summary : Craniopharyngioma, also known as dysodontogenic epithelial tumor, is related to adamantinous craniopharyngioma and papillary craniopharyngioma, and has symptoms including headache, seizures and vertigo. An important gene associated with Craniopharyngioma is CTNNB1 (Catenin Beta 1), and among its related pathways/superpathways are Glioma and PI3K-Akt signaling pathway. The drugs Hydrocortisone and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include pituitary, brain and hypothalamus, and related phenotypes are Increased Nanog expression and growth/size/body region

Wikipedia : 71 Craniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue, that occurs... more...

Related Diseases for Craniopharyngioma

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

(show top 50) (show all 167)
id Related Disease Score Top Affiliating Genes
1 adamantinous craniopharyngioma 33.0 IGF1 POU1F1 PRL TRH
2 papillary craniopharyngioma 30.3 BRAF CTNNB1 GHRL GNRH1 IGF1 INS
3 ocular melanoma 29.2 CTNNB1 KRT14 MKI67 VEGFA
4 bardet-biedl syndrome 29.1 CTNNB1 GNRH1 IGF1 VEGFA
5 cystadenoma 10.8
6 obesity 10.3
7 spondylarthropathy 10.2 BRAF MKI67
8 cerebellar vermis medulloblastoma 10.2 BRAF CTNNB1
9 seow najjar syndrome 10.2 IGF1 INS
10 childhood-onset cerebral x-linked adrenoleukodystrophy 10.2 CTNNB1 KRT8 MKI67
11 breast malignant phyllodes tumor 10.2 CTNNB1 KRT8 MKI67
12 gershoni-baruch syndrome 10.2 PRL TRH
13 gynandroblastoma 10.2 GHRL IGF1 POU1F1
14 bone benign neoplasm 10.2 CTNNB1 IGF1 PRL
15 diabetic encephalopathy 10.1 GHRL IGF1 INS
16 oral squamous cell carcinoma 10.1 BRAF CTNNB1 VEGFA
17 hepatopulmonary syndrome 10.1 INS VEGFA
18 caudal regression syndrome 10.1 IGF1 POU1F1 PRL
19 african histoplasmosis 10.1 GNRH1 PRL
20 albinism, oculocutaneous, type ib 10.1 CTNNB1 IGF1 VEGFA
21 adrenal adenoma 10.1 GHRL IGF1 INS
22 dacryoadenitis 10.1 IGF1 INS PRL
23 mucopolysaccharidosis type vi 10.1 IGF1 POU1F1 PRL
24 congenital tricuspid stenosis 10.1 PRL TRH
25 exostosis 10.1 GHRL IGF1 INS
26 prostatic adenoma 10.1 CTNNB1 MKI67 VEGFA
27 adenoma 10.1
28 hemolytic anemia, cd59-mediated, with or without immune-mediated polyneuropathy 10.1 GHRL IGF1 PRL
29 immune system organ benign neoplasm 10.1 IGF1 PRL TRH
30 cold-induced sweating syndrome 2 10.1 IGF1 PRL TRH
31 brucella canis brucellosis 10.1 IGF1 INS VEGFA
32 endometriosis 10.1 GNRH1 PRL
33 peroneal neuropathy 10.1 GHRL IGF1 INS
34 vernal conjunctivitis 10.1 IGF1 INS VEGFA
35 adult i blood group with or without congenital cataract 10.1 POU1F1 PRL TRH
36 pituitary adenoma 10.0
37 citrullinemia, type ii, neonatal-onset 10.0 IGF1 INS VEGFA
38 submucous uterine fibroid 10.0 INS VEGFA
39 haverhill fever 10.0 GNRH1 IGF1 PRL
40 blind loop syndrome 10.0 GHRL IGF1 INS
41 bladder colonic type adenocarcinoma 10.0 CTNNB1 IGF1 MKI67 PRL
42 placenta praevia 10.0 CTNNB1 KRT8 MKI67 VEGFA
43 hypoglycemic coma 10.0 GNRH1 INS TRH
44 edict syndrome 10.0 IGF1 INS PRL
45 vertebral artery occlusion 10.0 GNRH1 INS PRL
46 breast fibroadenoma 10.0 INS VEGFA
47 endocrine organ benign neoplasm 10.0 CTNNB1 IGF1 PRL TRH
48 sphingolipidosis 10.0 GNRH1 INS PRL
49 bone chondrosarcoma 10.0 KRT14 KRT8 MMP20
50 body dysmorphic disorder 10.0 GHRL IGF1 INS PRL

Graphical network of the top 20 diseases related to Craniopharyngioma:



Diseases related to Craniopharyngioma

Symptoms & Phenotypes for Craniopharyngioma

Human phenotypes related to Craniopharyngioma:

56 32 (show all 48)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 headache 56 32 Frequent (79-30%) HP:0002315
2 seizures 56 32 Very rare (<4-1%) HP:0001250
3 vertigo 56 32 Very rare (<4-1%) HP:0002321
4 nausea and vomiting 56 32 Frequent (79-30%) HP:0002017
5 obesity 56 32 Frequent (79-30%) HP:0001513
6 hydrocephalus 56 32 Occasional (29-5%) HP:0000238
7 intellectual disability 56 32 Very rare (<4-1%) HP:0001249
8 cerebral calcification 56 32 Very frequent (99-80%) HP:0002514
9 sleep apnea 56 32 Occasional (29-5%) HP:0010535
10 hearing impairment 56 32 Occasional (29-5%) HP:0000365
11 global developmental delay 56 32 Very rare (<4-1%) HP:0001263
12 increased intracranial pressure 56 32 Occasional (29-5%) HP:0002516
13 type ii diabetes mellitus 56 32 Occasional (29-5%) HP:0005978
14 optic atrophy 56 32 Occasional (29-5%) HP:0000648
15 delayed puberty 56 32 Occasional (29-5%) HP:0000823
16 myocardial infarction 56 32 Very rare (<4-1%) HP:0001658
17 postnatal growth retardation 56 32 Very rare (<4-1%) HP:0008897
18 cerebral ischemia 56 32 Occasional (29-5%) HP:0002637
19 coma 56 32 Very rare (<4-1%) HP:0001259
20 recurrent infections 56 32 Very rare (<4-1%) HP:0002719
21 papilledema 56 32 Frequent (79-30%) HP:0001085
22 slow decrease in visual acuity 56 32 Frequent (79-30%) HP:0007924
23 polyphagia 56 32 Occasional (29-5%) HP:0002591
24 pituitary hypothyroidism 56 32 Frequent (79-30%) HP:0008245
25 bitemporal hemianopia 56 32 Frequent (79-30%) HP:0030521
26 prolactin excess 56 32 Frequent (79-30%) HP:0000870
27 increased susceptibility to fractures 56 32 Occasional (29-5%) HP:0002659
28 proportionate short stature 56 32 Occasional (29-5%) HP:0003508
29 central diabetes insipidus 56 32 Frequent (79-30%) HP:0000863
30 progressive visual field defects 56 32 Frequent (79-30%) HP:0007987
31 sudden loss of visual acuity 56 32 Very rare (<4-1%) HP:0001117
32 central adrenal insufficiency 56 32 Frequent (79-30%) HP:0011734
33 abnormal hypothalamus morphology 56 32 Obligate (100%) HP:0012286
34 enlarged pituitary gland 56 32 Very frequent (99-80%) HP:0012505
35 intracranial cystic lesion 56 32 Very frequent (99-80%) HP:0010576
36 neoplasm of the anterior pituitary 56 32 Very frequent (99-80%) HP:0011750
37 abnormal visual field test 56 32 Frequent (79-30%) HP:0030588
38 abnormality of the nasal bone 56 32 Very rare (<4-1%) HP:0010939
39 abnormality of the frontal bone 56 32 Very rare (<4-1%) HP:0430000
40 excessive daytime somnolence 32 HP:0001262
41 sleep disturbance 56 Frequent (79-30%)
42 behavioral abnormality 56 Very rare (<4-1%)
43 growth delay 56 Occasional (29-5%)
44 hypogonadotrophic hypogonadism 32 HP:0000044
45 hypogonadism 56 Frequent (79-30%)
46 somnolence 56 Frequent (79-30%)
47 low gonadotropins (secondary hypogonadism) 56 Frequent (79-30%)
48 hypopituitarism 56 Very frequent (99-80%)

UMLS symptoms related to Craniopharyngioma:


visual disturbance

GenomeRNAi Phenotypes related to Craniopharyngioma according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased Nanog expression GR00371-A-1 9.23 MCM6 MKI67 SP7 VEGFA
2 Increased Nanog expression GR00371-A-2 9.23 VEGFA
3 Increased Nanog expression GR00371-A-3 9.23 MCM6
4 Increased Nanog expression GR00371-A-4 9.23 VEGFA
5 Increased Nanog expression GR00371-A-5 9.23 VEGFA

MGI Mouse Phenotypes related to Craniopharyngioma:

44 (show all 15)
id Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.29 BRAF CTNNB1 GNRH1 IGF1 INS KRT14
2 homeostasis/metabolism MP:0005376 10.29 BRAF CTNNB1 GHRL GNRH1 IGF1 INS
3 endocrine/exocrine gland MP:0005379 10.26 BRAF CTNNB1 GHRL GNRH1 IGF1 INS
4 hematopoietic system MP:0005397 10.19 BRAF CTNNB1 GNRH1 IGF1 INS KRT14
5 immune system MP:0005387 10.16 BRAF CTNNB1 GNRH1 IGF1 INS KRT14
6 digestive/alimentary MP:0005381 10.14 BRAF CTNNB1 GHRL GNRH1 INS KRT14
7 integument MP:0010771 10.03 BRAF CTNNB1 GNRH1 IGF1 INS KRT14
8 craniofacial MP:0005382 10.02 BRAF CTNNB1 KRT14 MMP20 POU1F1 VEGFA
9 neoplasm MP:0002006 9.97 BRAF CTNNB1 GNRH1 IGF1 KRT14 MCM6
10 nervous system MP:0003631 9.97 BRAF CTNNB1 GNRH1 IGF1 INS POU1F1
11 liver/biliary system MP:0005370 9.95 KRT8 PRL VEGFA BRAF CTNNB1 GNRH1
12 normal MP:0002873 9.91 BRAF CTNNB1 GHRL GNRH1 IGF1 INS
13 reproductive system MP:0005389 9.65 VEGFA BRAF CTNNB1 GNRH1 IGF1 INS
14 renal/urinary system MP:0005367 9.63 GNRH1 IGF1 INS VEGFA BRAF CTNNB1
15 skeleton MP:0005390 9.23 BRAF CTNNB1 GNRH1 IGF1 INS POU1F1

Drugs & Therapeutics for Craniopharyngioma

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

(show top 50) (show all 192)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754 657311
2
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
3
Butyric Acid Experimental Phase 4 107-92-6 264
4 glucocorticoids Phase 4,Phase 1
5 Cortisol succinate Phase 4
6 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
7 Anesthetics Phase 4,Phase 3
8 Hormones Phase 4,Phase 3,Phase 2,Phase 1
9 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1
10 Hydrocortisone 17-butyrate 21-propionate Phase 4
11 Hydrocortisone acetate Phase 4
12 Hydrocortisone-17-butyrate Phase 4
13 Anti-Inflammatory Agents Phase 4,Phase 1
14 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 1
15
Diazoxide Approved Phase 3,Phase 2 364-98-7 3019
16
Donepezil Approved Phase 3,Phase 2 120014-06-4 3152
17
Exenatide Approved, Investigational Phase 3,Phase 2,Phase 1 141758-74-9 15991534
18
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
19
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 1 50-02-2 5743
20
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
21
Ondansetron Approved Phase 3 99614-02-5 4595
22
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
23 Liver Extracts Phase 3,Phase 1,Phase 2
24 pancreatic polypeptide Phase 3,Phase 1
25 Analgesics Phase 3
26 Cholinergic Agents Phase 3,Phase 2
27 Adjuvants, Anesthesia Phase 3
28 Cholinesterase Inhibitors Phase 3,Phase 2
29 Narcotics Phase 3
30 Gastrointestinal Agents Phase 3,Phase 2,Phase 1
31 Neurotransmitter Agents Phase 3,Phase 2,Phase 1
32 Nootropic Agents Phase 3,Phase 2
33 Analgesics, Opioid Phase 3
34 Anesthetics, General Phase 3
35 Anesthetics, Intravenous Phase 3
36 Peripheral Nervous System Agents Phase 3,Phase 1
37 Vasodilator Agents Phase 3,Phase 2
38 Hypoglycemic Agents Phase 3,Phase 2,Phase 1
39 Incretins Phase 3,Phase 2,Phase 1
40 Antihypertensive Agents Phase 3,Phase 2
41 Central Nervous System Depressants Phase 3,Phase 1
42 glucagon Phase 3
43 Glucagon-Like Peptide 1 Phase 3
44 Dexamethasone acetate Phase 3,Phase 1 1177-87-3
45 Dexamethasone 21-phosphate Phase 3,Phase 1
46 Emetics Phase 3
47 Antiemetics Phase 3,Phase 1
48 BB 1101 Phase 3,Phase 1
49 Autonomic Agents Phase 3,Phase 1
50 Cola Nutraceutical Phase 3,Phase 1

Interventional clinical trials:

(show top 50) (show all 100)
id Name Status NCT ID Phase
1 Effect of Perioperative Glucocorticoid Replacement on Prognosis of Surgical Patients With Sellar Lesions Unknown status NCT02190994 Phase 4
2 SCRT Versus Conventional RT in Children and Young Adults With Low Grade and Benign Brain Tumors Unknown status NCT00517959 Phase 3
3 Effect of Diazoxide on the Obesity Secondary to Hypothalamic-pituitary Lesions Completed NCT00306683 Phase 3
4 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3
5 Donepezil in Treating Patients Who Have Undergone Radiation Therapy for Brain Tumors Completed NCT00369785 Phase 3
6 Energy Balance & Weight Loss in Craniopharyngioma-related Hypothalamic Obesity Recruiting NCT02664441 Phase 3
7 90Yttrium Colloid for the Treatment of Cystic Sellar/Parasellar Tumors Recruiting NCT02081768 Phase 3
8 Acupressure in Controlling Nausea in Young Patients Receiving Highly Emetogenic Chemotherapy Active, not recruiting NCT01346267 Phase 3
9 Multicentre Double-blind Randomized Clinical Trial Assessing Efficacy and Safety of Exenatide in the Treatment of Hypothalamic Obesity After Craniopharyngioma Therapy Not yet recruiting NCT02860923 Phase 3
10 Hypothalamic Obesity Following Craniopharyngioma Surgery: A Pilot Trial of Combined Metformin and Diazoxide Therapy Completed NCT00892073 Phase 2
11 Weight Loss Study for Patients With Obesity Due to Craniopharyngioma or Other Brain Tumor Completed NCT01484873 Phase 2
12 A Study for Image-Guided Radiation Therapy in Pediatric Brain Tumors and Side Effects Completed NCT00187226 Phase 2
13 An Efficacy, Safety, and Pharmacokinetics Study of Beloranib in Obese Subjects With Hypothalamic Injury Completed NCT02063295 Phase 2
14 Irinotecan in Treating Children With Refractory Solid Tumors Completed NCT00004078 Phase 2
15 Antineoplaston Therapy in Treating Children With Brain Tumors Completed NCT00003458 Phase 2
16 Temozolomide Plus Peripheral Stem Cell Transplantation in Treating Children With Newly Diagnosed Malignant Glioma or Recurrent CNS or Other Solid Tumors Completed NCT00005952 Phase 1, Phase 2
17 Radiolabeled Monoclonal Antibody Therapy in Treating Patients With Primary or Metastatic Brain Tumors Completed NCT00003461 Phase 1, Phase 2
18 Rebeccamycin Analogue in Treating Children With Solid Tumors or Non-Hodgkin's Lymphoma Completed NCT00006102 Phase 2
19 Temozolomide Followed by Radiation Therapy in Treating Children With Newly Diagnosed Malignant CNS Tumors Completed NCT00005955 Phase 2
20 Phase II Studies Of Donepezil And Ginkgo Biloba In Irradiated Brain Tumor Completed NCT00070161 Phase 2
21 Cyproheptadine and Megestrol in Preventing Weight Loss in Children With Cachexia Caused By Cancer or Cancer Treatment Completed NCT00066248 Phase 2
22 A Phase II Trial of Intensity-Modulated Proton Therapy for Incompletely Resected Craniopharyngioma and Observation for Craniopharyngioma After Radical Resection Recruiting NCT02792582 Phase 2
23 Peginterferon Alfa-2b in Treating Younger Patients With Craniopharyngioma That is Recurrent or Cannot Be Removed By Surgery Recruiting NCT01964300 Phase 2
24 Intranasal Oxytocin in Hypothalamic Obesity Recruiting NCT02849743 Phase 2
25 Methionine PET/CT Studies In Patients With Cancer Recruiting NCT00840047 Phase 2
26 Photodynamic Therapy (PDT) For Recurrent High Grade Gliomas Recruiting NCT01966809 Phase 2
27 A Phase II Trial of Limited Surgery and Proton Therapy for Craniopharyngioma or Observation After Radical Resection Active, not recruiting NCT01419067 Phase 2
28 Management of Pediatric Craniopharyngioma by a Combination of Partial Surgical Resection, and Protontherapy (Craniopharyngioma) Active, not recruiting NCT02842723 Phase 2
29 Effects of Exenatide on Hypothalamic Obesity Active, not recruiting NCT01061775 Phase 1, Phase 2
30 Crizotinib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Anaplastic Large Cell Lymphoma Active, not recruiting NCT00939770 Phase 1, Phase 2
31 Dasatinib, Ifosfamide, Carboplatin, and Etoposide in Treating Young Patients With Metastatic or Recurrent Malignant Solid Tumors Active, not recruiting NCT00788125 Phase 1, Phase 2
32 Dabrafenib in Treating Patients With BRAF Mutated Ameloblastoma Not yet recruiting NCT02367859 Phase 2
33 Methylphenidate HCl or Modafinil in Treating Young Patients With Excessive Daytime Sleepiness After Cancer Therapy Terminated NCT01348607 Phase 2
34 Tumor Classification and Its Application in Surgical Treatment of Craniopharyngioma Unknown status NCT00949156 Phase 1
35 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1
36 Peripheral Stem Cell Transplantation Plus Chemotherapy in Treating Patients With Malignant Solid Tumors Unknown status NCT00007813 Phase 1
37 Erlotinib Alone or in Combination With Radiation Therapy in Treating Young Patients With Refractory or Relapsed Malignant Brain Tumors or Newly Diagnosed Brain Stem Glioma Unknown status NCT00360854 Phase 1
38 Stereotactic Radiosurgery in Treating Patients With Brain Tumors Completed NCT00019578 Phase 1
39 Cilengitide in Treating Children With Refractory Primary Brain Tumors Completed NCT00063973 Phase 1
40 Temozolomide and O6-Benzylguanine in Treating Children With Recurrent Brain Tumors Completed NCT00052780 Phase 1
41 ABT-888 and Temozolomide in Treating Young Patients With Recurrent or Refractory CNS Tumors Completed NCT00946335 Phase 1
42 Ispinesib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Lymphoma Completed NCT00363272 Phase 1
43 Lenalidomide in Treating Young Patients With Recurrent, Progressive, or Refractory CNS Tumors Completed NCT00100880 Phase 1
44 FR901228 in Treating Children With Refractory or Recurrent Solid Tumors or Leukemia Completed NCT00053963 Phase 1
45 RO4929097, Temozolomide, and Radiation Therapy in Treating Patients With Newly Diagnosed Malignant Glioma Completed NCT01119599 Phase 1
46 Vorinostat and Temozolomide in Treating Young Patients With Relapsed or Refractory Primary Brain Tumors or Spinal Cord Tumors Completed NCT01076530 Phase 1
47 Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma Completed NCT00994500 Phase 1
48 SCH 66336 in Treating Children With Recurrent or Progressive Brain Tumors Completed NCT00015899 Phase 1
49 O6-benzylguanine and Carmustine in Treating Children With Refractory CNS Tumors Completed NCT00003765 Phase 1
50 Ixabepilone in Treating Young Patients With Solid Tumors or Leukemia That Haven't Responded to Therapy Completed NCT00030108 Phase 1

Search NIH Clinical Center for Craniopharyngioma

Cochrane evidence based reviews: craniopharyngioma

Genetic Tests for Craniopharyngioma

Anatomical Context for Craniopharyngioma

MalaCards organs/tissues related to Craniopharyngioma:

39
Pituitary, Brain, Hypothalamus, Bone, Testes, Endothelial, Skin

Publications for Craniopharyngioma

Articles related to Craniopharyngioma:

(show top 50) (show all 736)
id Title Authors Year
1
Multi-modality, Multi-directional Resection of Craniopharyngioma: versatility in alternating the principal and auxiliary surgical corridors and visualization modalities. ( 28344177 )
2017
2
Preclinical transgenic and patient-derived xenograft models recapitulate the radiological features of human adamantinomatous craniopharyngioma. ( 28481062 )
2017
3
MAPK pathway activation in the embryonic pituitary results in stem cell compartment expansion, differentiation defects and provides insights into the pathogenesis of papillary craniopharyngioma. ( 28506993 )
2017
4
Anorexia-cachexia syndrome-like hypothalamic neuroendocrine dysfunction in a patient with a papillary craniopharyngioma. ( 28469924 )
2017
5
Hypothalamic obesity after craniopharyngioma surgery: Treatment with a long acting glucagon like peptide 1 derivated. ( 28440760 )
2017
6
Review of management and morbidity of pediatric craniopharyngioma patients in a low-middle-income country: a 12-year experience. ( 28455541 )
2017
7
Interleukina896 induces an epitheliala89mesenchymal transition phenotype in human adamantinomatous craniopharyngioma cells and promotes tumor cell migration. ( 28487953 )
2017
8
Papillary craniopharyngioma: A clinicopathologic study of a rare entity from a major tertiary care center in Pakistan. ( 28488624 )
2017
9
Genomic Alterations of Adamantinomatous and Papillary Craniopharyngioma. ( 28069929 )
2017
10
Proteomics in pediatric cystic craniopharyngioma. ( 28414889 )
2017
11
Norman M. Dott, master of hypothalamic craniopharyngioma surgery: the decisive mentoring of Harvey Cushing and Percival Bailey at Peter Bent Brigham Hospital. ( 28059655 )
2017
12
Models of human adamantinomatous craniopharyngioma tissue: Steps toward an effective adjuvant treatment. ( 28414888 )
2017
13
Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN. ( 28499018 )
2017
14
Malignant Transformation of Craniopharyngioma without Radiation Therapy: Case Report and Review of the Literature. ( 28061501 )
2017
15
Genetically engineered mouse models of craniopharyngioma: an opportunity for therapy development and understanding of tumor biology. ( 28414891 )
2017
16
The purine receptor P2X7R regulates the release of pro-inflammatory cytokines in human craniopharyngioma. ( 28389503 )
2017
17
Microstructure alterations in the Hypothalamus in cranially radiated Childhood Leukemia survivors but not in Craniopharyngioma patients unaffected by hypothalamic damage. ( 28502079 )
2017
18
Confounding features of ectopic craniopharyngioma: A differential for tumors showing diffusion restriction. ( 28413554 )
2017
19
The neuroendocrine sequelae of paediatric craniopharyngioma: a 40 year meta-data analysis of 185 cases from three UK centres. ( 28073908 )
2017
20
Psychological well-being and independent living of young adults with childhood-onset craniopharyngioma. ( 28485516 )
2017
21
BRAF-V600E mutant papillary craniopharyngioma dramatically responds to combination BRAF and MEK inhibitors. ( 28425764 )
2017
22
Endocrine Disorders Developing after Surgical Intervention of Craniopharyngioma in Children. ( 28508600 )
2017
23
Primary Ectopic Ethmoidal Craniopharyngioma. ( 28403130 )
2017
24
Oxytocin inA survivors of childhood-onset craniopharyngioma. ( 27585663 )
2016
25
Removal of craniopharyngioma via fronto-basal interhemispheric approach. ( 27347116 )
2016
26
Drug priming enhances radiosensitivity of adamantinomatous craniopharyngioma via downregulation of survivin. ( 27903123 )
2016
27
Risk-adapted, long-term management in childhood-onset craniopharyngioma. ( 27604996 )
2016
28
Craniopharyngioma adherence: a comprehensive topographical categorization and outcome-related risk stratification model based on the methodical examination of 500 tumors. ( 27903121 )
2016
29
Simultaneous Transventricular-Orbitocranial Resection of Large Suprasellar Craniopharyngioma as Inspired by Jackson's Maneuver from 1863. ( 27054052 )
2016
30
Dramatic Response of BRAF V600E Mutant Papillary Craniopharyngioma to Targeted Therapy. ( 26498373 )
2016
31
Aseptic Meningitis with Craniopharyngioma Resection: Consideration after Endoscopic Surgery. ( 27722072 )
2016
32
Fenestrated optic nerve in craniopharyngioma. ( 27366261 )
2016
33
Introduction: Craniopharyngioma: current and emerging treatment modalities. ( 27903127 )
2016
34
Bypass surgery to treat symptomatic fusiform dilation of the internal carotid artery following craniopharyngioma resection: report of 2 cases. ( 27903112 )
2016
35
Ectopic craniopharyngioma of the fourth ventricle in a patient with Gardner syndrome. ( 26995577 )
2016
36
Distinct patterns of primary and motile cilia in Rathke's cleft cysts and craniopharyngioma subtypes. ( 27562488 )
2016
37
Letter to the Editor: Electing the surgical approach in craniopharyngioma surgery. ( 27058203 )
2016
38
Cholesterol granulomas presenting as sellar masses: a similar, but clinically distinct entity from craniopharyngioma and Rathke's cleft cyst. ( 27837386 )
2016
39
Spontaneous leaking craniopharyngioma causing preoperative chemical meningitis. ( 27127706 )
2016
40
Periostin activates pathways involved in epithelial-mesenchymal transition in adamantinomatous craniopharyngioma. ( 26723972 )
2016
41
Evaluation of Macular Ganglion Cell Complex and Peripapillary Retinal Nerve Fiber Layer in Primary Craniopharyngioma by Fourier-Domain Optical Coherence Tomography. ( 27372909 )
2016
42
Visualization of 90Yttrium Colloid Within a Cystic Craniopharyngioma Using PET/CT/MRI Fusion. ( 27846921 )
2016
43
Expanded Endonasal Endoscopic Approach for Resection of an Infrasellar Craniopharyngioma. ( 27554305 )
2016
44
Visceral adiposity index as an indicator of cardiometabolic risk in patients treated for craniopharyngioma. ( 27981514 )
2016
45
Clinical and prognostic role of annexin A2 in adamantinomatous craniopharyngioma. ( 27640198 )
2016
46
HORMONE SUBSTITUTION AFTER GASTRIC BYPASS SURGERY IN PATIENTS WITH HYPOPITUITARISM SECONDARY TO CRANIOPHARYNGIOMA. ( 26789341 )
2016
47
Craniopharyngioma recurrence: the impact of tumor topography. ( 27494816 )
2016
48
Osteoblastic differentiation and cell calcification of adamantinomatous craniopharyngioma induced by bone morphogenetic protein-2. ( 27983534 )
2016
49
Management strategy for treatment of vasospasm following transsphenoidal excision of craniopharyngioma. ( 27638640 )
2016
50
Potential evolution of neurosurgical treatment paradigms for craniopharyngioma based on genomic and transcriptomic characteristics. ( 27903126 )
2016

Variations for Craniopharyngioma

ClinVar genetic disease variations for Craniopharyngioma:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 CTNNB1 NM_001904.3(CTNNB1): c.94G> T (p.Asp32Tyr) single nucleotide variant Pathogenic/Likely pathogenic rs28931588 GRCh37 Chromosome 3, 41266097: 41266097
2 CTNNB1 NM_001904.3(CTNNB1): c.100G> A (p.Gly34Arg) single nucleotide variant Likely pathogenic rs121913399 GRCh37 Chromosome 3, 41266103: 41266103
3 CTNNB1 NM_001904.3(CTNNB1): c.100G> C (p.Gly34Arg) single nucleotide variant Likely pathogenic rs121913399 GRCh37 Chromosome 3, 41266103: 41266103

Cosmic variations for Craniopharyngioma:

9 (show all 20)
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM5672 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.94G>A p.D32N 8
2 COSM5677 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.98C>G p.S33C 8
3 COSM5679 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.110C>G p.S37C 8
4 COSM5684 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.100G>C p.G34R 8
5 COSM5668 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.94G>C p.D32H 8
6 COSM5683 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.97T>G p.S33A 8
7 COSM5663 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.133T>C p.S45P 8
8 COSM5676 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.122C>T p.T41I 8
9 COSM5669 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.98C>T p.S33F 8
10 COSM5686 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.100G>A p.G34R 8
11 COSM5661 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.94G>T p.D32Y 8
12 COSM5662 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.110C>T p.S37F 8
13 COSM5682 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.97T>C p.S33P 8
14 COSM5673 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.98C>A p.S33Y 8
15 COSM5664 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.121A>G p.T41A 8
16 COSM5681 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.95A>G p.D32G 8
17 COSM5701 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.122C>G p.T41S 8
18 COSM5758 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.127G>A p.A43T 8
19 COSM5687 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.109T>C p.S37P 8
20 COSM476 BRAF pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.1799T>A p.V600E 8

Expression for Craniopharyngioma

Search GEO for disease gene expression data for Craniopharyngioma.

Pathways for Craniopharyngioma

Pathways related to Craniopharyngioma according to GeneCards Suite gene sharing:

(show all 15)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.78 BRAF CTNNB1 IGF1 INS VEGFA
2 12.35 IGF1 INS PRL VEGFA
3
Show member pathways
12.19 BRAF CTNNB1 IGF1 INS VEGFA
4 11.87 BRAF CTNNB1 IGF1 VEGFA
5 11.85 IGF1 INS VEGFA
6 11.83 CTNNB1 IGF1 INS
7 11.72 BRAF CTNNB1 IGF1
8 11.68 BRAF IGF1 INS
9 11.63 IGF1 INS VEGFA
10 11.59 IGF1 INS SP7
11 11.55 BRAF CTNNB1 KRT14
12
Show member pathways
11.52 CTNNB1 GHRL IGF1 INS
13 11.46 KRT14 POU1F1 PRL
14 11.37 CTNNB1 KRT8 MKI67
15 10.48 IGF1 INS VEGFA

GO Terms for Craniopharyngioma

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

id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.86 GHRL GNRH1 IGF1 INS MMP20 PRL
2 endosome lumen GO:0031904 9.16 INS PRL
3 secretory granule GO:0030141 9.13 PRL TRH VEGFA
4 cell periphery GO:0071944 8.8 CTNNB1 KRT14 KRT8

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

(show all 14)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.83 BRAF CTNNB1 INS VEGFA
2 negative regulation of apoptotic process GO:0043066 9.72 BRAF GHRL GNRH1 IGF1 VEGFA
3 response to ethanol GO:0045471 9.69 GNRH1 PRL TRH
4 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.61 IGF1 INS VEGFA
5 positive regulation of MAPK cascade GO:0043410 9.58 CTNNB1 IGF1 INS
6 positive regulation of neuroblast proliferation GO:0002052 9.54 CTNNB1 VEGFA
7 positive regulation of glycogen biosynthetic process GO:0045725 9.49 IGF1 INS
8 cell fate specification GO:0001708 9.48 CTNNB1 POU1F1
9 positive regulation of glycolytic process GO:0045821 9.46 IGF1 INS
10 positive regulation of epithelial cell proliferation GO:0050679 9.43 IGF1 PRL VEGFA
11 ovulation cycle GO:0042698 9.37 GNRH1 PRL
12 response to organic cyclic compound GO:0014070 9.26 GNRH1 MKI67 PRL TRH
13 negative regulation of feeding behavior GO:2000252 9.16 INS TRH
14 positive regulation of cell proliferation GO:0008284 9.1 CTNNB1 IGF1 INS POU1F1 PRL VEGFA

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

id Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.1 GHRL GNRH1 IGF1 INS PRL TRH
2 insulin-like growth factor receptor binding GO:0005159 8.96 IGF1 INS

Sources for Craniopharyngioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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