MCID: CRN036
MIFTS: 65

Craniopharyngioma

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

Aliases & Classifications for Craniopharyngioma

MalaCards integrated aliases for Craniopharyngioma:

Name: Craniopharyngioma 12 53 59 55 6 44 15 17 72
Dysodontogenic Epithelial Tumor 53
Craniopharyngeal Duct Tumor 53
Neoplasm of Rathke's Pouch 12
Adamantinomatous Tumor 53
Rathke's Pouch Tumor 53

Characteristics:

Orphanet epidemiological data:

59
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 44 D003397
NCIt 50 C2964
SNOMED-CT 68 40009002
MESH via Orphanet 45 D003397
ICD10 via Orphanet 34 D44.4
UMLS via Orphanet 73 C0010276
Orphanet 59 ORPHA54595
UMLS 72 C0010276

Summaries for Craniopharyngioma

NIH Rare Diseases : 53 Craniopharyngioma is a slow-growing, non-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, hearing loss, and changes in mood or behavior. 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 involve surgery to remove the tumor, radiation therapy, chemotherapy, biologic therapy, and/or hormone therapy to replace various hormones no longer produced or secreted due to the tumor or its treatment.

MalaCards based summary : Craniopharyngioma, also known as dysodontogenic epithelial tumor, is related to papillary craniopharyngioma and ameloblastoma, and has symptoms including visual disturbance An important gene associated with Craniopharyngioma is CTNNB1 (Catenin Beta 1), and among its related pathways/superpathways are Nanog in Mammalian ESC Pluripotency and PI3K-Akt signaling pathway. The drugs Prednisone and Hydrocortisone acetate have been mentioned in the context of this disorder. Affiliated tissues include brain, pituitary and hypothalamus, and related phenotypes are abnormal hypothalamus morphology and cerebral calcification

Wikipedia : 75 Craniopharyngioma is a rare type of brain tumor derived from pituitary gland embryonic tissue that... more...

Related Diseases for Craniopharyngioma

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

(show top 50) (show all 452)
# Related Disease Score Top Affiliating Genes
1 papillary craniopharyngioma 34.9 KRT7 CTNNB1 BRAF
2 ameloblastoma 31.4 PTCH1 KRT8 KRT14
3 growth hormone deficiency 31.2 IGF1 GHRL GH1
4 familial adenomatous polyposis 1 31.1 CTNNB1 APC
5 galactorrhea 30.8 PRL IGF1
6 hypogonadotropism 30.7 PRL GNRH1
7 amenorrhea 30.5 TRH PRL GNRH1
8 sleep apnea 30.5 INS IGF1 GHRL
9 adenocarcinoma 30.4 EPCAM CTNNB1 BRAF APC
10 colorectal adenoma 30.4 IGF1 CTNNB1 APC
11 hyperinsulinism 30.4 INS IGF1 GHRL GH1
12 pituitary apoplexy 30.3 PRL INS GNRH1
13 adenoma 30.0 PRL IGF1 GH1 CTNNB1 BRAF APC
14 ameloblastic carcinoma 29.9 MKI67 KRT14
15 chiasmal syndrome 29.8 PRL GNRH1
16 pituitary adenoma, prolactin-secreting 29.6 TRH PRL IGF1 GH1
17 hyperprolactinemia 29.6 TRH PRL IGF1 GNRH1 GH1
18 functioning pituitary adenoma 29.5 TRH PRL GH1
19 hypothalamic disease 29.5 PRL GNRH1 GH1
20 insulin-like growth factor i 29.5 PRL INS IGF1 GH1
21 pituitary hormone deficiency, combined, 2 29.5 PROP1 PRL POU1F1 IGF1 GH1
22 prader-willi syndrome 29.4 INS IGF1 GNRH1 GHRL GH1
23 anorexia nervosa 29.4 PRL IGF1 GHRL GH1
24 isolated growth hormone deficiency 29.3 PROP1 POU1F1 IGF1 GH1
25 pituitary tumors 29.0 PROP1 PRL POU1F1 IGF1 GH1
26 chromophobe adenoma 29.0 TRH PRL INS GNRH1 GH1
27 hyperthyroidism 28.9 TRH PRL INS GHRL GH1
28 acromegaly 28.7 TRH PRL INS IGF1 GHRL GH1
29 hypopituitarism 28.7 PROP1 PRL POU1F1 INS IGF1 GNRH1
30 hypothyroidism 28.6 TRH PROP1 PRL POU1F1 IGF1 GH1
31 empty sella syndrome 28.6 TRH PRL IGF1 GNRH1 GH1
32 hypothyroidism, congenital, nongoitrous, 4 28.0 TRH PROP1 PRL POU1F1 IGF1 GH1
33 pituitary gland disease 27.8 TRH PROP1 PRL POU1F1 INS IGF1
34 breast cancer 27.3 PRL MKI67 KRT8 KRT14 INS IGF1
35 adamantinous craniopharyngioma 12.5
36 cystadenoma 11.2
37 diabetes insipidus 10.9
38 proliferating trichilemmal cyst 10.8 KRT8 KRT7
39 endolymphatic sac tumor 10.8 KRT8 KRT7
40 benign breast adenomyoepithelioma 10.8 KRT7 KRT14
41 calcifying epithelial odontogenic tumor 10.7 PTCH1 KRT8
42 desmoid disease, hereditary 10.7 CTNNB1 APC
43 hydrocephalus 10.7
44 congenital hydrocephalus 10.7
45 apocrine sweat gland neoplasm 10.7 KRT8 KRT7 KRT14
46 lung acinar adenocarcinoma 10.7 KRT7 CTNNB1
47 large intestine adenocarcinoma 10.7 KRT7 CTNNB1
48 oncocytic breast carcinoma 10.7 KRT7 KRT14
49 pregnancy adenoma 10.7 KRT8 KRT7 KRT14
50 basaloid squamous cell carcinoma 10.7 KRT8 KRT7 KRT14

Graphical network of the top 20 diseases related to Craniopharyngioma:



Diseases related to Craniopharyngioma

Symptoms & Phenotypes for Craniopharyngioma

Human phenotypes related to Craniopharyngioma:

59 32 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal hypothalamus morphology 59 32 obligate (100%) Obligate (100%) HP:0012286
2 cerebral calcification 59 32 hallmark (90%) Very frequent (99-80%) HP:0002514
3 neoplasm of the anterior pituitary 59 32 hallmark (90%) Very frequent (99-80%) HP:0011750
4 enlarged pituitary gland 59 32 hallmark (90%) Very frequent (99-80%) HP:0012505
5 intracranial cystic lesion 59 32 hallmark (90%) Very frequent (99-80%) HP:0010576
6 obesity 59 32 frequent (33%) Frequent (79-30%) HP:0001513
7 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
8 headache 59 32 frequent (33%) Frequent (79-30%) HP:0002315
9 papilledema 59 32 frequent (33%) Frequent (79-30%) HP:0001085
10 slow decrease in visual acuity 59 32 frequent (33%) Frequent (79-30%) HP:0007924
11 abnormal visual field test 59 32 frequent (33%) Frequent (79-30%) HP:0030588
12 pituitary hypothyroidism 59 32 frequent (33%) Frequent (79-30%) HP:0008245
13 excessive daytime somnolence 59 32 frequent (33%) Frequent (79-30%) HP:0001262
14 bitemporal hemianopia 59 32 frequent (33%) Frequent (79-30%) HP:0030521
15 prolactin excess 59 32 frequent (33%) Frequent (79-30%) HP:0000870
16 central diabetes insipidus 59 32 frequent (33%) Frequent (79-30%) HP:0000863
17 progressive visual field defects 59 32 frequent (33%) Frequent (79-30%) HP:0007987
18 central adrenal insufficiency 59 32 frequent (33%) Frequent (79-30%) HP:0011734
19 hypogonadotrophic hypogonadism 32 frequent (33%) HP:0000044
20 hydrocephalus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000238
21 sleep apnea 59 32 occasional (7.5%) Occasional (29-5%) HP:0010535
22 hearing impairment 59 32 occasional (7.5%) Occasional (29-5%) HP:0000365
23 increased intracranial pressure 59 32 occasional (7.5%) Occasional (29-5%) HP:0002516
24 type ii diabetes mellitus 59 32 occasional (7.5%) Occasional (29-5%) HP:0005978
25 optic atrophy 59 32 occasional (7.5%) Occasional (29-5%) HP:0000648
26 delayed puberty 59 32 occasional (7.5%) Occasional (29-5%) HP:0000823
27 cerebral ischemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002637
28 polyphagia 59 32 occasional (7.5%) Occasional (29-5%) HP:0002591
29 increased susceptibility to fractures 59 32 occasional (7.5%) Occasional (29-5%) HP:0002659
30 proportionate short stature 59 32 occasional (7.5%) Occasional (29-5%) HP:0003508
31 intellectual disability 59 32 very rare (1%) Very rare (<4-1%) HP:0001249
32 seizures 59 32 very rare (1%) Very rare (<4-1%) HP:0001250
33 global developmental delay 59 32 very rare (1%) Very rare (<4-1%) HP:0001263
34 myocardial infarction 59 32 very rare (1%) Very rare (<4-1%) HP:0001658
35 postnatal growth retardation 59 32 very rare (1%) Very rare (<4-1%) HP:0008897
36 vertigo 59 32 very rare (1%) Very rare (<4-1%) HP:0002321
37 coma 59 32 very rare (1%) Very rare (<4-1%) HP:0001259
38 recurrent infections 59 32 very rare (1%) Very rare (<4-1%) HP:0002719
39 sudden loss of visual acuity 59 32 very rare (1%) Very rare (<4-1%) HP:0001117
40 abnormality of the nasal bone 59 32 very rare (1%) Very rare (<4-1%) HP:0010939
41 abnormality of the frontal bone 59 32 very rare (1%) Very rare (<4-1%) HP:0430000
42 sleep disturbance 59 Frequent (79-30%)
43 behavioral abnormality 59 Very rare (<4-1%)
44 growth delay 59 Occasional (29-5%)
45 hypogonadism 59 Frequent (79-30%)
46 low gonadotropins (secondary hypogonadism) 59 Frequent (79-30%)
47 hypopituitarism 59 Very frequent (99-80%)

UMLS symptoms related to Craniopharyngioma:


visual disturbance

MGI Mouse Phenotypes related to Craniopharyngioma:

46 (show all 26)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.51 APC BRAF CTNNB1 EPCAM GHRL GNRH1
2 endocrine/exocrine gland MP:0005379 10.49 APC BRAF CTNNB1 EPCAM GHRL GNRH1
3 growth/size/body region MP:0005378 10.46 APC BRAF CTNNB1 EPCAM GNRH1 IGF1
4 cellular MP:0005384 10.4 APC BRAF CTNNB1 EPCAM IGF1 INS
5 immune system MP:0005387 10.39 APC BRAF CTNNB1 EPCAM GNRH1 IGF1
6 digestive/alimentary MP:0005381 10.38 APC BRAF CTNNB1 EPCAM GHRL GNRH1
7 behavior/neurological MP:0005386 10.36 APC BRAF CTNNB1 EPCAM GHRL INS
8 hematopoietic system MP:0005397 10.35 APC BRAF CTNNB1 EPCAM GNRH1 IGF1
9 mortality/aging MP:0010768 10.32 APC BRAF CTNNB1 EPCAM IGF1 INS
10 nervous system MP:0003631 10.3 APC BRAF CTNNB1 EPCAM GNRH1 IGF1
11 integument MP:0010771 10.28 APC BRAF CTNNB1 GNRH1 IGF1 INS
12 hearing/vestibular/ear MP:0005377 10.27 APC BRAF CTNNB1 IGF1 KRT14 KRT8
13 embryo MP:0005380 10.25 APC BRAF CTNNB1 EPCAM INS KRT8
14 craniofacial MP:0005382 10.21 APC BRAF CTNNB1 KRT14 POU1F1 PTCH1
15 adipose tissue MP:0005375 10.17 APC BRAF GHRL IGF1 INS KRT14
16 normal MP:0002873 10.14 APC BRAF CTNNB1 GHRL GNRH1 IGF1
17 neoplasm MP:0002006 10.13 APC BRAF CTNNB1 GNRH1 IGF1 KRT14
18 muscle MP:0005369 10.1 APC BRAF CTNNB1 IGF1 INS PTCH1
19 reproductive system MP:0005389 10.1 APC BRAF CTNNB1 GNRH1 IGF1 INS
20 liver/biliary system MP:0005370 10.07 APC BRAF CTNNB1 GNRH1 INS KRT8
21 no phenotypic analysis MP:0003012 10.03 CTNNB1 EPCAM INS MKI67 PTCH1 SOX2
22 renal/urinary system MP:0005367 9.97 APC BRAF CTNNB1 GNRH1 IGF1 INS
23 pigmentation MP:0001186 9.91 APC BRAF CTNNB1 KRT14 POU1F1 PTCH1
24 respiratory system MP:0005388 9.76 BRAF CTNNB1 IGF1 KRT14 PROP1 PTCH1
25 skeleton MP:0005390 9.65 APC BRAF CTNNB1 GNRH1 IGF1 INS
26 vision/eye MP:0005391 9.23 APC BRAF CTNNB1 INS KRT14 PTCH1

Drugs & Therapeutics for Craniopharyngioma

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

(show top 50) (show all 207)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
3
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754
4 Hydrocortisone-17-butyrate Phase 4
5 Hydrocortisone hemisuccinate Phase 4
6 Hydrocortisone 17-butyrate 21-propionate Phase 4
7
Diazoxide Approved Phase 3 364-98-7 3019
8
Exenatide Approved, Investigational Phase 3 141758-74-9 15991534
9
Glucagon Approved Phase 3 16941-32-5
10
Donepezil Approved Phase 3 120014-06-4 3152
11
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
12
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
13
Ondansetron Approved Phase 3 99614-02-5 4595
14
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
15 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
16 Antihypertensive Agents Phase 3
17 Vasodilator Agents Phase 3
18 Hypoglycemic Agents Phase 3
19 Anti-Obesity Agents Phase 3
20 Incretins Phase 3
21 Glucagon-Like Peptide 1 Phase 3
22 Central Nervous System Depressants Phase 3
23 Anesthetics Phase 3
24 Cholinesterase Inhibitors Phase 3
25 Cholinergic Agents Phase 3
26 Nootropic Agents Phase 3
27 Liver Extracts Phase 3
28 Peripheral Nervous System Agents Phase 3
29 Emetics Phase 3
30 Cola Phase 3
31 Analgesics Phase 3
32 Anesthetics, Intravenous Phase 3
33 Narcotics Phase 3
34 Analgesics, Opioid Phase 3
35 Adjuvants, Anesthesia Phase 3
36 Anesthetics, General Phase 3
37
Peginterferon alfa-2b Approved Phase 2 99210-65-8, 215647-85-1
38
Metformin Approved Phase 2 657-24-9 4091 14219
39
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
40
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
41
Lenograstim Approved, Investigational Phase 1, Phase 2 135968-09-1
42
Histamine Approved, Investigational Phase 2 51-45-6 774
43
Megestrol acetate Approved, Investigational, Vet_approved Phase 2 595-33-5 11683
44
Cyproheptadine Approved Phase 2 129-03-3 2913
45
Oxytocin Approved, Vet_approved Phase 2 50-56-6 439302 53477758
46
Vemurafenib Approved Phase 2 918504-65-1 42611257 23252090
47
Memantine Approved, Investigational Phase 2 19982-08-2 4054
48
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
49
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
50
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690

Interventional clinical trials:

(show top 50) (show all 107)
# Name Status NCT ID Phase Drugs
1 Effect of Perioperative Glucocorticoid Replacement on Prognosis of Surgical Patients With Sellar Lesions Unknown status NCT02190994 Phase 4 Hydrocortisone;Prednisone
2 Efficacy of Stereotactic Conformal Radiotherapy (SCRT) Compared to Conventional Radiotherapy in Minimising Late Sequelae in Children and Young Adults With Brain Tumours: a Randomised Clinical Trial Unknown status NCT00517959 Phase 3
3 Clinical Assessment of the Treatment With Diazoxide in Children Suffering From Obesity and Hyperinsulinemia Secondary to Surgery of Hypothalamic-pituitary Lesions Completed NCT00306683 Phase 3 DIAZOXIDE
4 Multicentre Double-blind Randomized Clinical Trial Assessing Efficacy and Safety of Exenatide in the Treatment of Hypothalamic Obesity After Craniopharyngioma Therapy Completed NCT02860923 Phase 3 Exenatide;Placebo
5 Phase III Double Blind, Placebo Controlled Study of Donepezil in the Irradiated Brain Completed NCT00369785 Phase 3 donepezil hydrochloride;Placebo
6 Randomized Controlled Trial of Acupressure to Control Chemotherapy-Induced Nausea (CIN) in Children Receiving Highly Emetogenic Chemotherapy Completed NCT01346267 Phase 3
7 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
8 Effects of Exenatide on Hypothalamic Obesity Unknown status NCT01061775 Phase 1, Phase 2 Exenatide
9 A Prospective, Multicentre Trial on the Value of 18F-FET PET in the Post-therapeutic Evaluation of Childhood Brain Tumours Unknown status NCT03216148 Phase 2
10 Phase II Study of Peginterferon Alfa-2b (Sylatron) for Pediatric Patients With Unresectable or Recurrent Craniopharyngioma Completed NCT01964300 Phase 2
11 Combined Diazoxide and Metformin Therapy in Children With Hypothalamic Obesity Secondary to Craniopharyngioma: A Pilot Study Completed NCT00892073 Phase 2 Diazoxide;Metformin
12 Effects of Exenatide on Body Weight in Patients With Hypothalamic Obesity Completed NCT01484873 Phase 2 Exenatide
13 Randomized, Double-Blind, Placebo Controlled, Phase 2a Trial of ZGN-440 (Subcutaneous Beloranib in Suspension), A Novel Methionine Aminopeptidase 2 Inhibitor, in Obese Subjects With Hypothalamic Injury to Evaluate Weight Reduction and Safety Over 4 Weeks Followed by an Optional 4-Week Open-Label Extension Completed NCT02063295 Phase 2 ZGN-440 sterile diluent;ZGN-440 for injectable suspension
14 A Phase II Study of Image-Guided Radiation Therapy for Pediatric CNS Tumors and Quantification of Radiation-Related CNS Effects Completed NCT00187226 Phase 2
15 Phase II Trial of Irinotecan in Children With Refractory Solid Tumors Completed NCT00004078 Phase 2 irinotecan hydrochloride
16 Phase II Study of Antineoplastons A10 and AS2-1 in Children With Brain Tumors Completed NCT00003458 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
17 A Phase II Trial of Rebeccamycin Analogue (NSC #655649) in Children With Solid Tumors Completed NCT00006102 Phase 2 becatecarin
18 Phase II Treatment of Children With Newly Diagnosed Malignant Central Nervous System Tumors With Temozolomide Prior to Radiation Therapy Completed NCT00005955 Phase 2 temozolomide
19 Phase I Study of At-Labeled Anti-Tenascin Human/Mouse Chimeric Monoclonal Antibody 81C6 (ch81C6) Via Surgically Created Cystic Resection Cavity in the Treatment of Patients With Primary or Metastatic Brain Tumors Completed NCT00003461 Phase 1, Phase 2
20 Donepezil and EGb761 in Improving Neurocognitive Function in Patients Who Have Previously Undergone Radiation Therapy for Primary Brain Tumor or Brain Metastases Completed NCT00070161 Phase 2 donepezil hydrochloride
21 A Phase I/II Trial of Temodar in Pediatric Patients and Young Adults With High-Risk or Recurrent Solid Tumors Completed NCT00005952 Phase 1, Phase 2 temozolomide
22 The Effect of Cyproheptadine Hydrochloride (Periactin) and Megestrol Acetate (Megace) on Weight in Children With Cancer/Treatment Related Cachexia Completed NCT00066248 Phase 2 cyproheptadine hydrochloride;megestrol acetate
23 A Phase I/II Study of Reirradiation for Recurrent Pediatric Brain and Spinal Cord Tumors and Primary Glioblastoma Multiforme Using the Photon Radiosurgery System Completed NCT00179907 Phase 1, Phase 2
24 Intranasal Oxytocin to Promote Weight Loss in Children, Adolescents, and Adults With Brain Tumors and Hypothalamic Obesity Syndrome Recruiting NCT02849743 Phase 2 Syntocinon;Placebo (for Syntocinon)
25 A Phase II Trial of Intensity-Modulated Proton Therapy for Incompletely Resected Craniopharyngioma and Observation for Craniopharyngioma After Radical Resection Recruiting NCT02792582 Phase 2
26 Phase II Trial of BRAF/MEK Inhibitors in Papillary Craniopharyngiomas Recruiting NCT03224767 Phase 2 Vemurafenib;Cobimetinib
27 Memantine for Prevention of Cognitive Late Effects in Pediatric Patients Receiving Cranial Radiation Therapy for Localized Brain Tumors: A Pilot Study Recruiting NCT03194906 Phase 2 Memantine
28 Methionine PET/CT Studies In Patients With Cancer Recruiting NCT00840047 Phase 2 Methionine
29 Phase II Study on the Management of Pediatric Craniopharyngioma by a Combination of Partial Surgical Resection, and Protontherapy Active, not recruiting NCT02842723 Phase 2
30 A Phase II Trial of Limited Surgery and Proton Therapy for Craniopharyngioma and Observation for Craniopharyngioma After Radical Resection Active, not recruiting NCT01419067 Phase 2 ^1^8F-fluorodeoxyglucose;^1^1C-methionine
31 Dasatinib With Ifosfamide, Carboplatin, Etoposide: A Pediatric Phase I/II Trial Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
32 A Randomized, Phase II Placebo-controlled Study of the Use of Extended-release Methylphenidate or Modafinil for the Treatment of Excessive Daytime Sleepiness in Children Following Cancer Therapy Terminated NCT01348607 Phase 2 methylphenidate hydrochloride;modafinil;placebo
33 A Phase II Study of Photodynamic Therapy (PDT) With Photofrin® (IND 104,613) For Recurrent High Grade Gliomas in Adults Terminated NCT01966809 Phase 2 Photofrin photodynamic therapy.
34 Photodynamic Therapy For Childhood Brain Tumors, A Phase I Study Unknown status NCT00002647 Phase 1 verteporfin
35 Phase I Studies of TARCEVA™ (ERLOTINIB HYDROCHLORIDE, OSI-774) as Single Agent in Children With Refractory and Relapsed Malignant Brain Tumors and in Combination With Irradiation in Newly Diagnosed Brain Stem Glioma Unknown status NCT00360854 Phase 1 erlotinib hydrochloride
36 Phase I Pilot Study of Stereotaxic Radiosurgery in Patients With Intracranial Neoplasms Completed NCT00019578 Phase 1
37 A PHASE 1 STUDY OF ISPINESIB (SB-715992) IN PEDIATRIC PATIENTS WITH RELAPSED OR REFRACTORY SOLID TUMORS Completed NCT00363272 Phase 1 ispinesib
38 Phase I Trial Of Escalating Oral Doses Of SCH 66336 In Pediatric Patients With Refractory Or Recurrent Brain Tumors Completed NCT00015899 Phase 1 lonafarnib
39 A PHASE I STUDY OF DEPSIPEPTIDE (NSC#630176, IND# 51810) IN PEDIATRIC PATIENTS WITH REFRACTORY SOLID TUMORS AND LEUKEMIAS Completed NCT00053963 Phase 1 romidepsin
40 A Phase I Study of Vorinostat and Bortezomib in Children With Refractory or Recurrent Solid Tumors, Including CNS Tumors and Lymphomas Completed NCT00994500 Phase 1 vorinostat;bortezomib
41 A Phase I Study of SAHA and Temozolomide in Children With Relapsed or Refractory Primary Brain or Spinal Cord Tumors Completed NCT01076530 Phase 1 vorinostat;temozolomide
42 A Phase I Trial of CC-5013 (Lenalidomide) in Pediatric Patients With Recurrent or Refractory Primary CNS Tumors Completed NCT00100880 Phase 1 lenalidomide
43 Phase I Study Of Cloretazine (VNP40101M) In Children With Recurrent, Progressive Or Refractory Primary Brain Tumors Completed NCT00098761 Phase 1 laromustine
44 A Trial of 06-BG and BCNU in Children With CNS Tumors Completed NCT00003765 Phase 1 O6-benzylguanine;carmustine
45 Phase I Study of Anti-Tenascin Monoclonal Antibody I-Labeled 81C6 Via Surgically Created Cystic Resection Cavity in the Treatment of Patients With Primary Brain Tumors After External Beam Radiotherapy Completed NCT00003484 Phase 1 carmustine;irinotecan hydrochloride
46 Phase I Study of Cilengitide (EMD 121974) in Children With Refractory Brain Tumors Completed NCT00063973 Phase 1 cilengitide
47 High Dose Carboplatin Combined With Oral VP-16 In The Treatment Of Pediatric CNS Malignancies Completed NCT00053118 Phase 1 carboplatin;etoposide
48 A Phase I Study of ABT-888, an Oral Inhibitor of Poly (ADP-Ribose) Polymerase and Temozolomide in Children With Recurrent/Refractory CNS Tumors Completed NCT00946335 Phase 1 veliparib;temozolomide
49 A Pilot Feasibility, Dose-Escalation Study Using Intracerebral Microdialysis to Assess the Neuropharmacodynamics of Temsirolimus in Patients With Primary or Metastatic Brain Tumors Completed NCT00784914 Phase 1 temsirolimus
50 A Phase I Trial and Pharmacokinetic Study of Talabostat (PT-100, Val-Boro-Pro) in Combination With Temozolomide or Carboplatin in Pediatric Patients With Relapsed or Refractory Solid Tumors Including Brain Tumors Completed NCT00303940 Phase 1 carboplatin;talabostat mesylate;temozolomide

Search NIH Clinical Center for Craniopharyngioma

Inferred drug relations via UMLS 72 / NDF-RT 51 :


L-Arginine

Cochrane evidence based reviews: craniopharyngioma

Genetic Tests for Craniopharyngioma

Anatomical Context for Craniopharyngioma

MalaCards organs/tissues related to Craniopharyngioma:

41
Brain, Pituitary, Hypothalamus, Bone, Testes, Liver, Spinal Cord

Publications for Craniopharyngioma

Articles related to Craniopharyngioma:

(show top 50) (show all 3080)
# Title Authors PMID Year
1
Oxytocin and Naltrexone Successfully Treat Hypothalamic Obesity in a Boy Post-Craniopharyngioma Resection. 38 6
29220529 2018
2
First experiences with neuropsychological effects of oxytocin administration in childhood-onset craniopharyngioma. 38 6
28213803 2017
3
Oxytocin in survivors of childhood-onset craniopharyngioma. 38 6
27585663 2016
4
Specific behaviour, mood and personality traits may contribute to obesity in patients with craniopharyngioma. 38 6
24923438 2015
5
Novel immunohistochemical markers in the diagnosis of nonglial tumors of nervous system. 9 38
20179436 2010
6
The differential diagnosis of central nervous system tumors: a critical examination of some recent immunohistochemical applications. 9 38
18318590 2008
7
Expression of human MCM6 and DNA Topo II alpha in craniopharyngiomas and its correlation with recurrence of the tumor. 9 38
17410335 2007
8
Acquired prolactin deficiency in patients with disorders of the hypothalamic-pituitary axis. 9 38
17556861 2007
9
[Expression of minichromosome maintenance protein 6 in craniopharyngioma and its correlation with prognosis]. 9 38
17294730 2007
10
Mutation in exon 7 of PTCH deregulates SHH/PTCH/SMO signaling: possible linkage to WNT. 9 38
16596257 2006
11
Hypersomnolence and increased REM sleep with low cerebrospinal fluid hypocretin level in a patient after removal of craniopharyngioma. 9 38
16084764 2005
12
Beta-catenin mutations in craniopharyngiomas and pituitary adenomas. 9 38
15980970 2005
13
Expression and growth dependency of the insulin-like growth factor I receptor in craniopharyngioma cells: a novel therapeutic approach. 9 38
16000560 2005
14
Common mutations of beta-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region. 9 38
15891929 2005
15
The clinical, metabolic and endocrine features and the quality of life in adults with childhood-onset craniopharyngioma compared with adult-onset craniopharyngioma. 9 38
15817911 2005
16
Possible linkage between specific histological structures and aberrant reactivation of the Wnt pathway in adamantinomatous craniopharyngioma. 9 38
15221941 2004
17
Beta-catenin mutations are frequent in calcifying odontogenic cysts, but rare in ameloblastomas. 9 38
14578169 2003
18
Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. 9 38
12466115 2002
19
Growth without growth hormone. 9 38
12510977 2002
20
Differential expression of cytokeratins 8 and 20 distinguishes craniopharyngioma from rathke cleft cyst. 9 38
12296753 2002
21
Craniopharyngioma: a case report and comparative galectin histochemical analysis. 9 38
12495217 2002
22
Pseudotumor of the pituitary due to PROP-1 deletion. 9 38
11822586 2002
23
A study of the growth-promoting and metabolic effects of growth hormone (GH) in a patient with the "growth without GH" syndrome. 9 38
11735238 2001
24
Different keratin profiles in craniopharyngioma subtypes and ameloblastomas. 9 38
11770017 2001
25
The use of insulin-like growth factor 1 reference values for the diagnosis of growth hormone deficiency in prepubertal children. 9 38
10848878 2000
26
A boy with normal growth in spite of growth hormone deficiency after resection of a suprasellar teratoma. 9 38
10890195 2000
27
Somatostatin receptor tumor imaging (Tc 99m P829) in pituitary adenoma. 9 38
10659562 1999
28
Serum IGF-I and IGFBP-3 concentrations do not accurately predict growth hormone deficiency in children with brain tumours. 9 38
10469014 1999
29
The prevalence of severe growth hormone deficiency in adults who received growth hormone replacement in childhood [see comment]. 9 38
8729528 1996
30
Effective use of magnetic resonance imaging in the assessment of children with possible growth hormone deficiency. 9 38
15251557 1996
31
Could craniopharyngiomas produce pituitary hormones? 9 38
8098858 1993
32
Discontinuation of growth hormone therapy in growth-hormone-deficient patients: assessment of body fat mass using bioelectrical impedance. 9 38
8314202 1993
33
Pulsatile prolactin secretion in hyperprolactinemia due to presumed pituitary stalk interruption. 9 38
1955511 1991
34
Demonstration of insulin-like growth factors I, II and heterogeneous insulin-like growth factor binding proteins in the cyst fluid of patients with craniopharyngioma. 9 38
1719442 1991
35
Eating behaviour and oxytocin in patients with childhood-onset craniopharyngioma and different grades of hypothalamic involvement. 38
31013553 2019
36
Subfrontal Infrachiasmatic Approach to a Craniopharyngioma Resection: 2-Dimensional Operative Video. 38
30726971 2019
37
Benefit of Intracystic Bleomycin for Symptomatic Recurrent Rathke Cleft Cyst. 38
30445570 2019
38
Is Gross Total Resection Reasonable in Adults with Craniopharyngiomas with Hypothalamic Involvement? 38
31203080 2019
39
Thromboelastography-Guided Therapy of Hemorrhagic Complications after Craniopharyngioma Resection: Case-Based Update. 38
31390646 2019
40
Identification and Functional Enrichment Analysis of Potential Diagnostic and Therapeutic Targets in Adamantinomatous Craniopharyngioma. 38
31424286 2019
41
Extended endoscopic endonasal resection of craniopharyngioma using intraoperative visual evoked potential monitoring: technical note. 38
31402418 2019
42
Commentary: A Clinical Rule for Preoperative Prediction of BRAF Mutation Status in Craniopharyngioma. 38
30541138 2019
43
Ectopic clival craniopharyngioma with intratumoral hemorrhage: A case report. 38
31194153 2019
44
Pediatric craniopharyngioma. 38
31385085 2019
45
Expanded Endonasal Endoscopic Surgery in Suprasellar Craniopharyngiomas: A Retrospective Analysis of 43 Surgeries Including Recurrent Cases. 38
30768130 2019
46
The Medical Therapy of Craniopharyngiomas: The Way Ahead. 38
31369091 2019
47
Outcomes after craniotomy for resection of craniopharyngiomas in adults: analysis of the National Surgical Quality Improvement Program (NSQIP). 38
31228138 2019
48
A Clinical Rule for Preoperative Prediction of BRAF Mutation Status in Craniopharyngiomas. 38
30481321 2019
49
Clinical significance of tumor-related edema of the optic tract affecting visual function in patients with sellar and suprasellar tumors. 38
31394363 2019
50
A new technique of endoscopic decompression of suprasellar craniopharyngioma cyst. 38
31377958 2019

Variations for Craniopharyngioma

ClinVar genetic disease variations for Craniopharyngioma:

6 (show all 23)
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 APC NM_000038.6(APC): c.3183_3187del (p.Lys1061_Gln1062insTer) deletion Pathogenic rs587779352 5:112174474-112174478 5:112838777-112838781
2 CTNNB1 NM_001904.4(CTNNB1): c.94G> T (p.Asp32Tyr) single nucleotide variant Pathogenic/Likely pathogenic rs28931588 3:41266097-41266097 3:41224606-41224606
3 CTNNB1 NM_001904.4(CTNNB1): c.100G> A (p.Gly34Arg) single nucleotide variant Likely pathogenic rs121913399 3:41266103-41266103 3:41224612-41224612
4 CTNNB1 NM_001904.4(CTNNB1): c.100G> C (p.Gly34Arg) single nucleotide variant Likely pathogenic rs121913399 3:41266103-41266103 3:41224612-41224612
5 ERCC2 NM_000400.3(ERCC2): c.1361_1363TCA[2] (p.Ile456del) short repeat Likely pathogenic 19:45860740-45860742 19:45357482-45357484
6 TSC2 NM_000548.5(TSC2): c.3803G> A (p.Arg1268His) single nucleotide variant Conflicting interpretations of pathogenicity rs200577441 16:2131788-2131788 16:2081787-2081787
7 TSC2 NM_000548.5(TSC2): c.3846_3855delinsG (p.Ser1282_Gly1285delinsArg) indel Conflicting interpretations of pathogenicity rs587778732 16:2132468-2132477 16:2082467-2082476
8 CTNNB1 NM_001904.4(CTNNB1): c.101G> T (p.Gly34Val) single nucleotide variant Conflicting interpretations of pathogenicity rs28931589 3:41266104-41266104 3:41224613-41224613
9 NF1 NM_000267.3(NF1): c.4773G> A (p.Arg1591=) single nucleotide variant Conflicting interpretations of pathogenicity rs1555533268 17:29652838-29652838 17:31325820-31325820
10 PTCH1 NM_001083602.2(PTCH1): c.4-1650G> A single nucleotide variant Conflicting interpretations of pathogenicity rs143494325 9:98270531-98270531 9:95508249-95508249
11 SMARCA4 NM_001128849.2(SMARCA4): c.442G> A (p.Gly148Arg) single nucleotide variant Conflicting interpretations of pathogenicity rs138689221 19:11096951-11096951 19:10986275-10986275
12 SUFU NM_016169.3(SUFU): c.839G> A (p.Arg280Gln) single nucleotide variant Conflicting interpretations of pathogenicity rs145704867 10:104356979-104356979 10:102597222-102597222
13 MSH6 NM_000179.2(MSH6): c.1657A> C (p.Thr553Pro) single nucleotide variant Uncertain significance 2:48026779-48026779 2:47799640-47799640
14 PHOX2B NM_003924.3(PHOX2B): c.556G> A (p.Glu186Lys) single nucleotide variant Uncertain significance 4:41748213-41748213 4:41746196-41746196
15 PTCH1 NM_001083602.2(PTCH1): c.5G> A (p.Gly2Glu) single nucleotide variant Uncertain significance 9:98268880-98268880 9:95506598-95506598
16 KRAS NM_004985.5(KRAS): c.547_552del (p.Thr183_Lys184del) deletion Uncertain significance 12:25362744-25362749 12:25209815-25209820
17 TSC1 NM_000368.4(TSC1): c.1231C> A (p.Leu411Ile) single nucleotide variant Uncertain significance rs397514840 9:135785990-135785990 9:132910603-132910603
18 CDH1 NM_004360.5(CDH1): c.1225T> C (p.Trp409Arg) single nucleotide variant Uncertain significance rs587778176 16:68847303-68847303 16:68813400-68813400
19 BRCA1 NM_007294.3(BRCA1): c.5123C> T (p.Ala1708Val) single nucleotide variant Uncertain significance rs28897696 17:41215920-41215920 17:43063903-43063903
20 TSC1 NM_000368.4(TSC1): c.1355G> C (p.Gly452Ala) single nucleotide variant Uncertain significance rs371093730 9:135782201-135782201 9:132906814-132906814
21 TSC2 NM_000548.5(TSC2): c.3038A> G (p.Asp1013Gly) single nucleotide variant Uncertain significance rs1060500961 16:2129104-2129104 16:2079103-2079103
22 BMPR1A NM_004329.2(BMPR1A): c.499A> T (p.Met167Leu) single nucleotide variant Uncertain significance rs200951235 10:88659852-88659852 10:86900095-86900095
23 PTPN11 NM_002834.4(PTPN11): c.925A> G (p.Ile309Val) single nucleotide variant Benign rs201787206 12:112915526-112915526 12:112477722-112477722

Cosmic variations for Craniopharyngioma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM5672 CTNNB1 pituitary,craniopharyngeal duct,craniopharyngioma,adamantinomatous c.94G>A p.D32N 3:41224606-41224606 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 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.1 SOX2 IGF1 GNRH1 GH1 CTNNB1 BRAF
2
Show member pathways
12.79 PRL INS IGF1 GH1 CTNNB1 APC
3
Show member pathways
12.73 INS IGF1 CTNNB1 BRAF APC
4 12.7 PTCH1 IGF1 CTNNB1 BRAF APC
5
Show member pathways
12.36 INS IGF1 BRAF APC
6 12.17 SOX9 PTCH1 GHRL BRAF
7 12.13 PTCH1 IGF1 CTNNB1 BRAF
8
Show member pathways
12.1 IGF1 GNRH1 GH1 CTNNB1 BRAF APC
9 12.06 SOX2 PTCH1 CTNNB1 APC
10
Show member pathways
11.91 INS IGF1 GHRL GH1 CTNNB1
11 11.88 SOX2 IGF1 CTNNB1 APC
12 11.83 INS IGF1 GH1 CTNNB1
13 11.73 KRT14 CTNNB1 BRAF
14 11.71 IGF1 CTNNB1 BRAF APC
15 11.66 PRL POU1F1 KRT14
16 11.63 SOX9 SOX2 IGF1
17 11.59 MKI67 KRT8 CTNNB1
18 11.48 SOX9 SOX2 INS IGF1 EPCAM
19 11.36 SOX2 INS IGF1
20 11.27 SOX9 PTCH1 IGF1 GH1
21 10.56 SOX9 SOX2 CTNNB1 APC

GO Terms for Craniopharyngioma

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

# Name GO ID Score Top Affiliating Genes
1 keratin filament GO:0045095 9.61 KRT8 KRT7 KRT14
2 cell periphery GO:0071944 9.5 KRT8 KRT14 CTNNB1
3 lateral plasma membrane GO:0016328 9.43 EPCAM CTNNB1 APC
4 beta-catenin destruction complex GO:0030877 9.4 CTNNB1 APC
5 Wnt signalosome GO:1990909 9.37 CTNNB1 APC
6 transcription factor complex GO:0005667 9.35 SOX9 SOX2 PROP1 POU1F1 CTNNB1
7 nuclear transcription factor complex GO:0044798 9.33 SOX9 SOX2 CTNNB1
8 endosome lumen GO:0031904 8.8 PRL INS GH1

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

(show all 33)
# Name GO ID Score Top Affiliating Genes
1 regulation of gene expression GO:0010468 9.93 SOX2 IGF1 GNRH1 CTNNB1
2 negative regulation of cell proliferation GO:0008285 9.88 SOX9 PTCH1 POU1F1 GNRH1 CTNNB1 APC
3 positive regulation of MAPK cascade GO:0043410 9.81 SOX2 INS IGF1 CTNNB1
4 negative regulation of epithelial cell proliferation GO:0050680 9.8 SOX9 SOX2 PTCH1
5 negative regulation of apoptotic process GO:0043066 9.8 SOX9 PROP1 IGF1 GNRH1 GHRL CTNNB1
6 response to nutrient levels GO:0031667 9.77 PRL GHRL GH1
7 somatic stem cell population maintenance GO:0035019 9.76 SOX9 SOX2 BRAF
8 branching involved in ureteric bud morphogenesis GO:0001658 9.73 SOX9 PTCH1 CTNNB1
9 regulation of cell proliferation GO:0042127 9.72 SOX9 PTCH1 GHRL CTNNB1 BRAF
10 dorsal/ventral pattern formation GO:0009953 9.71 PTCH1 PROP1 CTNNB1
11 regulation of cell differentiation GO:0045595 9.7 SOX9 CTNNB1 APC
12 positive regulation of cell proliferation GO:0008284 9.7 SOX9 PRL POU1F1 INS IGF1 EPCAM
13 response to organic cyclic compound GO:0014070 9.67 TRH PTCH1 GNRH1
14 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.67 SOX9 INS IGF1 GH1
15 prostate gland development GO:0030850 9.66 SOX9 PTCH1
16 positive regulation of insulin-like growth factor receptor signaling pathway GO:0043568 9.65 IGF1 GH1
17 male sex determination GO:0030238 9.65 SOX9 GNRH1
18 JAK-STAT cascade involved in growth hormone signaling pathway GO:0060397 9.65 PRL GH1
19 positive regulation of multicellular organism growth GO:0040018 9.65 POU1F1 GHRL GH1
20 positive regulation of growth GO:0045927 9.64 GHRL GH1
21 mammary gland development GO:0030879 9.63 SOX9 PTCH1 PRL
22 positive regulation of epithelial cell proliferation GO:0050679 9.62 SOX9 IGF1
23 positive regulation of glucose transmembrane transport GO:0010828 9.61 GH1 BRAF
24 response to estradiol GO:0032355 9.61 PTCH1 GH1 CTNNB1
25 negative regulation of feeding behavior GO:2000252 9.6 TRH INS
26 positive regulation of epithelial cell differentiation GO:0030858 9.58 SOX9 CTNNB1
27 somatotropin secreting cell differentiation GO:0060126 9.55 PROP1 POU1F1
28 ovulation cycle GO:0042698 9.34 GNRH1
29 adenohypophysis development GO:0021984 9.13 SOX2 PROP1 POU1F1
30 cell fate specification GO:0001708 8.92 SOX9 POU1F1 CTNNB1 APC
31 positive regulation of transcription by RNA polymerase II GO:0045944 10.16 SOX9 SOX2 PROP1 POU1F1 IGF1 EPCAM
32 positive regulation of transcription, DNA-templated GO:0045893 10.04 SOX9 SOX2 PTCH1 POU1F1 IGF1 CTNNB1
33 positive regulation of gene expression GO:0010628 10.02 SOX9 INS IGF1 CTNNB1 BRAF

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

# Name GO ID Score Top Affiliating Genes
1 beta-catenin binding GO:0008013 9.43 SOX9 PROP1 APC
2 insulin-like growth factor receptor binding GO:0005159 9.32 INS IGF1
3 hormone activity GO:0005179 9.17 TRH PRL INS IGF1 GNRH1 GHRL
4 prolactin receptor binding GO:0005148 8.96 PRL GH1
5 protein binding GO:0005515 10.09 SOX9 SOX2 PTCH1 PROP1 PRL MKI67

Sources for Craniopharyngioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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