MCID: CRN036
MIFTS: 62

Craniopharyngioma

Categories: Cancer diseases, Endocrine diseases, Eye diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Craniopharyngioma

MalaCards integrated aliases for Craniopharyngioma:

Name: Craniopharyngioma 11 19 58 53 5 43 14 16 71 75
Dysodontogenic Epithelial Tumor 19
Craniopharyngeal Duct Tumor 19
Neoplasm of Rathke's Pouch 11
Adamantinomatous Tumor 19
Rathke's Pouch Tumor 19

Characteristics:


Prevelance:

1-9/100000 (Europe) 1-9/1000000 (United States, Sweden) 58

Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare endocrine diseases


Summaries for Craniopharyngioma

GARD: 19 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). 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.

MalaCards based summary: Craniopharyngioma, also known as dysodontogenic epithelial tumor, is related to papillary craniopharyngioma and adamantinous craniopharyngioma, and has symptoms including visual disturbance An important gene associated with Craniopharyngioma is APC (APC Regulator Of WNT Signaling Pathway), and among its related pathways/superpathways are Signal Transduction and Nanog in Mammalian ESC Pluripotency. The drugs Prednisone and Hydrocortisone succinate have been mentioned in the context of this disorder. Affiliated tissues include pituitary, hypothalamus and brain, and related phenotypes are abnormal hypothalamus morphology and cerebral calcification

Orphanet: 58 Craniopharyngiomas are benign slow growing tumours that are located within the sellar and parasellar regions of the central nervous system.

Wikipedia: 75 A 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 621)
# Related Disease Score Top Affiliating Genes
1 papillary craniopharyngioma 32.8 KRT7 CTNNB1 BRAF
2 adamantinous craniopharyngioma 32.1 SOX2 PROP1 LEF1 CTNNB1
3 central diabetes insipidus 31.3 TRH PRL IGF1 GNRH1
4 leptin deficiency or dysfunction 31.0 TRH LEPR INS IL6 IGF1 GNRH1
5 ameloblastoma 30.9 KRT8 KRT14 CTNNB1
6 eating disorder 30.8 LEPR INS IGF1 GHRL
7 pituitary adenoma 30.8 TRH PRL POU1F1 IGF1 GNRH1 GHRL
8 adult syndrome 30.8 INS IL6 IGF1
9 sleep disorder 30.8 INS IL6 IGF1 GHRL
10 hyperinsulinism 30.7 LEPR INS IGF1 GNRH1 GHRL
11 abdominal obesity-metabolic syndrome 1 30.6 LEPR INS GHRL
12 apnea, obstructive sleep 30.6 INS IL6 IGF1 GHRL
13 adenoma 30.6 TRH PRL POU1F1 KRT7 IGF1 CTNNB1
14 pilomatrixoma 30.5 LEF1 KRT14 CTNNB1
15 galactorrhea 30.5 PRL IGF1
16 fatty liver disease 30.5 LEPR INS IL6 IGF1 GHRL
17 hyperprolactinemia 30.5 TRH PRL INS IGF1 GNRH1
18 lipid metabolism disorder 30.4 LEPR INS IL6 IGF1 GHRL
19 teratoma 30.4 SOX2 KRT7 INS CTNNB1
20 amenorrhea 30.4 TRH PRL INS IGF1 GNRH1 GHRL
21 pituitary hormone deficiency, combined, 2 30.4 TRH PROP1 PRL POU1F1 IGF1 GNRH1
22 sleep apnea 30.4 LEPR INS IL6 IGF1 GHRL
23 prediabetes syndrome 30.3 LEPR INS IGF1 GHRL
24 hypoglycemia 30.3 TRH PRL INS IGF1 GHRL
25 body mass index quantitative trait locus 11 30.3 TRH PRL LEPR INS IL6 IGF1
26 functioning pituitary adenoma 30.2 TRH PRL POU1F1 IGF1
27 insulin-like growth factor i 30.2 PRL INS IL6 IGF1 GHRL
28 type 1 diabetes mellitus 30.2 LEPR INS IL6 IGF1 GHRL
29 pituitary tumors 30.2 PROP1 PRL IGF1
30 hypopituitarism 30.2 TRH SOX2 PROP1 PRL POU1F1 INS
31 chromophobe adenoma 30.2 TRH PRL
32 liver cirrhosis 30.1 LEPR KRT8 INS IL6
33 isolated growth hormone deficiency 30.1 PROP1 POU1F1 IGF1
34 pituitary apoplexy 30.1 TRH PRL INS IL6 IGF1 GNRH1
35 thyroiditis 30.0 PRL INS IL6
36 hypothalamic disease 30.0 TRH PRL INS IGF1 GNRH1
37 celiac disease 1 30.0 INS IL6 IGF1 GHRL
38 pituitary gland disease 30.0 TRH PROP1 PRL POU1F1 INS IGF1
39 anorexia nervosa 30.0 PRL LEPR INS IGF1 GHRL
40 hypogonadism 30.0 PROP1 PRL LEPR INS IGF1 GNRH1
41 prolactinoma 29.8 TRH PRL POU1F1 INS IGF1 GNRH1
42 acromegaly 29.8 TRH PRL INS IGF1 GNRH1 GHRL
43 conn's syndrome 29.8 TRH PRL INS IL6 IGF1 GNRH1
44 prader-willi syndrome 29.7 TRH PRL LEPR INS IL6 IGF1
45 major depressive disorder 29.7 TRH PRL INS IL6 GHRL
46 hypothyroidism 29.7 TRH PROP1 PRL POU1F1 INS IGF1
47 hyperthyroidism 29.7 TRH PRL INS IL6 IGF1 GHRL
48 fibrous dysplasia 29.7 PRL IL6 IGF1 APC
49 alopecia 29.6 PRL KRT14 IL6 IGF1 CTNNB1
50 basal cell carcinoma 29.6 KRT8 KRT7 KRT14 IL6 ERCC2 CTNNB1

Graphical network of the top 20 diseases related to Craniopharyngioma:



Diseases related to Craniopharyngioma

Symptoms & Phenotypes for Craniopharyngioma

Human phenotypes related to Craniopharyngioma:

58 30 (show all 49)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal hypothalamus morphology 58 30 Obligate (100%) Obligate (100%)
HP:0012286
2 cerebral calcification 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002514
3 enlarged pituitary gland 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0012505
4 intracranial cystic lesion 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0010576
5 neoplasm of the anterior pituitary 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0011750
6 nausea and vomiting 58 30 Frequent (33%) Frequent (79-30%)
HP:0002017
7 obesity 58 30 Frequent (33%) Frequent (79-30%)
HP:0001513
8 headache 58 30 Frequent (33%) Frequent (79-30%)
HP:0002315
9 excessive daytime somnolence 58 30 Frequent (33%) Frequent (79-30%)
HP:0001262
10 pituitary hypothyroidism 58 30 Frequent (33%) Frequent (79-30%)
HP:0008245
11 central diabetes insipidus 58 30 Frequent (33%) Frequent (79-30%)
HP:0000863
12 central adrenal insufficiency 58 30 Frequent (33%) Frequent (79-30%)
HP:0011734
13 bitemporal hemianopia 58 30 Frequent (33%) Frequent (79-30%)
HP:0030521
14 increased circulating prolactin concentration 58 30 Frequent (33%) Frequent (79-30%)
HP:0000870
15 progressive visual field defects 58 30 Frequent (33%) Frequent (79-30%)
HP:0007987
16 abnormal visual field test 58 30 Frequent (33%) Frequent (79-30%)
HP:0030588
17 papilledema 58 30 Frequent (33%) Frequent (79-30%)
HP:0001085
18 slow decrease in visual acuity 58 30 Frequent (33%) Frequent (79-30%)
HP:0007924
19 hypogonadotropic hypogonadism 30 Frequent (33%) HP:0000044
20 sleep apnea 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0010535
21 hydrocephalus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000238
22 hearing impairment 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000365
23 type ii diabetes mellitus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0005978
24 increased intracranial pressure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002516
25 optic atrophy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000648
26 delayed puberty 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000823
27 cerebral ischemia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002637
28 increased susceptibility to fractures 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002659
29 polyphagia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002591
30 proportionate short stature 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003508
31 intellectual disability 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001249
32 seizure 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001250
33 global developmental delay 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001263
34 postnatal growth retardation 58 30 Very rare (1%) Very rare (<4-1%)
HP:0008897
35 myocardial infarction 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001658
36 vertigo 58 30 Very rare (1%) Very rare (<4-1%)
HP:0002321
37 recurrent infections 58 30 Very rare (1%) Very rare (<4-1%)
HP:0002719
38 coma 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001259
39 sudden loss of visual acuity 58 30 Very rare (1%) Very rare (<4-1%)
HP:0001117
40 abnormal nasal bone morphology 30 Very rare (1%) HP:0010939
41 abnormal frontal bone morphology 30 Very rare (1%) HP:0430000
42 sleep disturbance 58 Frequent (79-30%)
43 behavioral abnormality 58 Very rare (<4-1%)
44 growth delay 58 Occasional (29-5%)
45 hypogonadism 58 Frequent (79-30%)
46 low gonadotropins (secondary hypogonadism) 58 Frequent (79-30%)
47 hypopituitarism 58 Very frequent (99-80%)
48 abnormality of the nasal bone 58 Very rare (<4-1%)
49 abnormality of the frontal bone 58 Very rare (<4-1%)

UMLS symptoms related to Craniopharyngioma:


visual disturbance

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

25 (show all 15)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.19 APC BRAF CTNNB1 ERCC2 GHRL GNRH1
2 no effect GR00402-S-2 10.19 APC BRAF CTNNB1 ERCC2 GHRL GNRH1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-126 10.15 KRT8
4 Increased shRNA abundance (Z-score > 2) GR00366-A-133 10.15 APC
5 Increased shRNA abundance (Z-score > 2) GR00366-A-135 10.15 KRT7 KRT8
6 Increased shRNA abundance (Z-score > 2) GR00366-A-180 10.15 KRT8
7 Increased shRNA abundance (Z-score > 2) GR00366-A-192 10.15 APC KRT7 KRT8
8 Increased shRNA abundance (Z-score > 2) GR00366-A-202 10.15 APC
9 Increased shRNA abundance (Z-score > 2) GR00366-A-40 10.15 KRT8
10 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.15 CTNNB1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-68 10.15 KRT7
12 Increased shRNA abundance (Z-score > 2) GR00366-A-72 10.15 KRT7
13 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.15 KRT7 KRT8
14 Increased shRNA abundance (Z-score > 2) GR00366-A-91 10.15 KRT7
15 Decreased viability in pancreas lineage GR00235-A 8.85 KRT7

MGI Mouse Phenotypes related to Craniopharyngioma:

45 (show all 25)
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.52 APC BRAF CTNNB1 ERCC2 GHRL GNRH1
2 nervous system MP:0003631 10.51 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
3 growth/size/body region MP:0005378 10.48 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
4 endocrine/exocrine gland MP:0005379 10.47 APC BRAF CTNNB1 ERCC2 GHRL GNRH1
5 neoplasm MP:0002006 10.4 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
6 cellular MP:0005384 10.34 APC BRAF CTNNB1 ERCC2 IGF1 IL6
7 immune system MP:0005387 10.31 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
8 renal/urinary system MP:0005367 10.3 APC BRAF CTNNB1 GNRH1 IGF1 IL6
9 digestive/alimentary MP:0005381 10.3 APC BRAF CTNNB1 GHRL GNRH1 IL6
10 adipose tissue MP:0005375 10.27 APC BRAF ERCC2 GHRL IGF1 IL6
11 behavior/neurological MP:0005386 10.27 APC BRAF CTNNB1 ERCC2 GHRL IL6
12 reproductive system MP:0005389 10.27 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
13 normal MP:0002873 10.26 APC BRAF CTNNB1 GHRL GNRH1 IGF1
14 liver/biliary system MP:0005370 10.24 APC BRAF CTNNB1 GNRH1 IL6 INS
15 pigmentation MP:0001186 10.22 APC BRAF CTNNB1 ERCC2 KRT14 LEF1
16 cardiovascular system MP:0005385 10.22 APC BRAF CTNNB1 IGF1 IL6 INS
17 muscle MP:0005369 10.19 APC BRAF CTNNB1 IGF1 IL6 INS
18 skeleton MP:0005390 10.13 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
19 hearing/vestibular/ear MP:0005377 10.1 APC BRAF CTNNB1 IGF1 KRT14 POU1F1
20 craniofacial MP:0005382 10.05 APC BRAF CTNNB1 KRT14 LEF1 POU1F1
21 respiratory system MP:0005388 10.01 BRAF CTNNB1 IGF1 IL6 KRT14 LEPR
22 hematopoietic system MP:0005397 10 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1
23 vision/eye MP:0005391 9.96 APC BRAF CTNNB1 ERCC2 IL6 INS
24 mortality/aging MP:0010768 9.83 APC BRAF CTNNB1 ERCC2 IGF1 IL6
25 integument MP:0010771 9.44 APC BRAF CTNNB1 ERCC2 GNRH1 IGF1

Drugs & Therapeutics for Craniopharyngioma

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

(show top 50) (show all 70)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
2
Hydrocortisone succinate Approved Phase 4 2203-97-6 3643
3
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
4
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 3640 5754
5 Antineoplastic Agents, Hormonal Phase 4
6 Hormones Phase 4
7 Hormone Antagonists Phase 4
8 glucocorticoids Phase 4
9 Anti-Inflammatory Agents Phase 4
10 Anesthetics Phase 4
11 Hydrocortisone 17-butyrate 21-propionate Phase 4
12
Diazoxide Approved Phase 3 364-98-7 3019
13
Exenatide Approved, Investigational Phase 3 141758-74-9 45588096
14
Glucagon Approved Phase 3 16941-32-5 16133228 16186314
15 Antihypertensive Agents Phase 3
16 Vasodilator Agents Phase 3
17 Hypoglycemic Agents Phase 3
18 Glucagon-Like Peptide 1 Phase 3
19 Incretins Phase 3
20 Anti-Obesity Agents Phase 3
21
Metformin Approved Phase 2 1115-70-4, 657-24-9 4091
22
Irinotecan Approved, Investigational Phase 2 97682-44-5, 100286-90-6 60838
23
Oxytocin Approved, Vet_approved Phase 2 50-56-6 439302 53477758
24
Nivolumab Approved Phase 2 946414-94-4
25
Trametinib Approved Phase 2 871700-17-3 11707110
26
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760
27
Vemurafenib Approved Phase 2 918504-65-1 42611257
28
Memantine Approved, Investigational Phase 2 41100-52-1, 19982-08-2 4054
29
Dopamine Approved Phase 2 62-31-7, 51-61-6 681
30
Peginterferon alfa-2b Approved Phase 2 215647-85-1, 99210-65-8
31
Racemethionine Approved, Experimental, Investigational, Nutraceutical Phase 2 59-51-8, 63-68-3, 348-67-4 6137
32 Insulin, Globin Zinc Phase 2
33
Insulin Phase 2
34 Angiogenesis Inhibitors Phase 2
35 CKD732 Phase 2
36 topoisomerase I inhibitors Phase 2
37 Immune Checkpoint Inhibitors Phase 2
38 Antineoplastic Agents, Immunological Phase 2
39 Protein Kinase Inhibitors Phase 2
40 Fluorodeoxyglucose F18 Phase 2
41 Neurotransmitter Agents Phase 2
42 Dopamine Agents Phase 2
43 Antiparkinson Agents Phase 2
44 Excitatory Amino Acid Antagonists Phase 2
45 Antiviral Agents Phase 2
46 Anti-Infective Agents Phase 2
47
Vorinostat Approved, Investigational Phase 1 149647-78-9 5311
48
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
49
Lenograstim Approved, Investigational Phase 1 135968-09-1
50
Temozolomide Approved, Investigational Phase 1 85622-93-1 5394

Interventional clinical trials:

(show all 50)
# 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 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
4 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
5 Combined Diazoxide and Metformin Therapy in Children With Hypothalamic Obesity Secondary to Craniopharyngioma: A Pilot Study Completed NCT00892073 Phase 2 Diazoxide;Metformin
6 Phase II Study on the Management of Pediatric Craniopharyngioma by a Combination of Partial Surgical Resection, and Protontherapy Completed NCT02842723 Phase 2
7 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
8 Phase II Trial of Irinotecan in Children With Refractory Solid Tumors Completed NCT00004078 Phase 2 irinotecan hydrochloride
9 Effects of Exenatide on Body Weight in Patients With Hypothalamic Obesity Completed NCT01484873 Phase 2 Exenatide
10 Intranasal Oxytocin to Promote Weight Loss in Children, Adolescents, and Adults With Brain Tumors and Hypothalamic Obesity Syndrome Completed NCT02849743 Phase 2 Syntocinon;Placebo (for Syntocinon)
11 Nivolumab and DAY101 for the Treatment of Newly Diagnosed or Recurrent Craniopharyngioma in Children and Young Adults Recruiting NCT05465174 Phase 2 Nivolumab;DAY101
12 A Phase II Trial of Intensity-Modulated Proton Therapy for Incompletely Resected Craniopharyngioma and Observation for Craniopharyngioma After Radical Resection Recruiting NCT02792582 Phase 2
13 Neoadjuvant and Postoperative Treatment With Dabrafenib and Trametinib in BRAF Mutated Papillary Craniopharyngioma Recruiting NCT05525273 Phase 2 Oral dabrafenib and trametinib
14 Methionine PET/CT Studies In Patients With Cancer Recruiting NCT00840047 Phase 2 Methionine
15 Phase II Trial of BRAF/MEK Inhibitors in Papillary Craniopharyngiomas Recruiting NCT03224767 Phase 2 Vemurafenib;Cobimetinib
16 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
17 Memantine for Prevention of Cognitive Late Effects in Pediatric Patients Receiving Cranial Radiation Therapy for Localized Brain Tumors: A Pilot Study Active, not recruiting NCT03194906 Phase 2 Memantine
18 Phase 2 Study of Systemic IL-6 Receptor Antagonist ACTEMRA® (Tocilizumab) for the Treatment of Progressive/Recurrent Pediatric Adamantinomatous Craniopharyngioma Not yet recruiting NCT05233397 Phase 2 Tocilizumab
19 Phase 2 Study of the MEK Inhibitor MEKTOVI® (Binimetinib) for the Treatment of Pediatric Adamantinomatous Craniopharyngioma Not yet recruiting NCT05286788 Phase 2 Binimetinib Oral Tablet [Mektovi]
20 Phase II Study of Peginterferon Alfa-2b (Sylatron) for Pediatric Patients With Unresectable or Recurrent Craniopharyngioma Terminated NCT01964300 Phase 2
21 Phase I Pilot Study of Stereotaxic Radiosurgery in Patients With Intracranial Neoplasms Completed NCT00019578 Phase 1
22 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
23 Phase I Trial of Temozolomide and O6-Benzylguanine in Pediatric Patients With Recurrent Brain Tumors Completed NCT00052780 Phase 1 O6-benzylguanine;temozolomide
24 A Phase I Trial of CC-5013 (Lenalidomide) in Pediatric Patients With Recurrent or Refractory Primary CNS Tumors Completed NCT00100880 Phase 1 lenalidomide
25 Phase I Study of Cilengitide (EMD 121974) in Children With Refractory Brain Tumors Completed NCT00063973 Phase 1 cilengitide
26 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
27 A PHASE 1 STUDY OF ISPINESIB (SB-715992) IN PEDIATRIC PATIENTS WITH RELAPSED OR REFRACTORY SOLID TUMORS Completed NCT00363272 Phase 1 ispinesib
28 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
29 A PHASE I STUDY OF DEPSIPEPTIDE (NSC#630176, IND# 51810) IN PEDIATRIC PATIENTS WITH REFRACTORY SOLID TUMORS AND LEUKEMIAS Completed NCT00053963 Phase 1 romidepsin
30 Phase 1 Trial of RO4929097 in Combination With Standard Radiotherapy and Temozolomide for Newly Diagnosed Malignant Glioma: A Pharmacokinetic and Pharmacodynamic Study Completed NCT01119599 Phase 1 Gamma-Secretase Inhibitor RO4929097;Temozolomide
31 Microenvironment: Imaging/Implications in Brain Tumors; A Preliminary Investigation of the Biodistribution of [F-18]-EF5 in Patients With Brain Tumors Terminated NCT00110032 Phase 1 EF5
32 A PHASE 1/2 STUDY OF RO4929097, AN ORAL SMALL MOLECULE INHIBITOR OF GAMMA-SECRETASE, IN CHILDREN WITH RELAPSED/REFRACTORY SOLID OR CNS TUMORS, LYMPHOMA, OR T-CELL LEUKEMIA Terminated NCT01088763 Phase 1 gamma-secretase/Notch signalling pathway inhibitor RO4929097;dexamethasone
33 Tumor Classification and Its Application in Surgical Treatment of Craniopharyngioma Unknown status NCT00949156
34 Examining Sleep and Family Functioning in Pediatric Craniopharyngioma Using Ecological Momentary Assessment Completed NCT03330080
35 Weight Gain in Surgically Treated Adult-onset Craniopharyngioma Completed NCT01968603
36 Clinical Features and Risk Factors of Craniopharyngioma With Tumoral Hemorrhage Completed NCT04937335
37 Prospective, Multi-Center Survey Study of Children and Adolescents With Craniopharyngioma Completed NCT00258453
38 Social Participation After Childhood Craniopharyngioma Completed NCT03139526
39 Intraoperative Use of a MR Tomography System Combined With Neuronavigation - Analysis of the Craniopharyngioma Subgroup ("Intraoperative Nutzung Eines Kernspintomographie-Systems in Direkter Kopplung Mit Einem Neuronavigations-System") Completed NCT01206543
40 KRANIOPHARYNGEOM 2007 - Multicenter Prospective Study of Children and Adolescents With Craniopharyngioma Completed NCT01272622
41 Cross Sectional Study of Sleep-wake and Melatonin Patterns in Patients Treated for Craniopharyngiomas Compared to Matched Controls Completed NCT01881854
42 A Children's Oncology Group Protocol for Collecting and Banking Pediatric Brain Tumor Research Specimens Completed NCT00919750
43 KRANIOPHARYNGEOM Registry 2019 Multicenter Registry for Patients With Childhood-onset Craniopharyngioma, Xanthogranuloma, Cysts of Rathke's Pouch, Meningioma, Pituitary Adenoma, Arachnoid Cysts Recruiting NCT04158284
44 A Phase 0/Feasibility Trial of Tocilizumab in Children and Adolescents With Newly- Diagnosed or Recurrent/Progressive Adamantinomatous Craniopharyngioma Recruiting NCT03970226 Early Phase 1 Tocilizumab
45 Proton Therapy Research Infrastructure- ProTRAIT- Neuro-oncology Recruiting NCT04648462
46 A Prospective Longitudinal Quality of Life Study in Patients Undergoing Endoscopic Endonasal Skull Base Surgery Recruiting NCT04087902
47 Molecular-guided Therapy for the Treatment of Patients With Relapsed and Refractory Childhood Cancers Active, not recruiting NCT02162732
48 Advancing Treatment for Pediatric Craniopharyngioma: Prospective Pilot Study Identifying Clinically Relevant Biological Targets for Medical Therapy Enrolling by invitation NCT03610906
49 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452
50 Deep Brain Stimulation for Hypothalamic Obesity: A Surgical & Neuroimaging Study Withdrawn NCT03708913

Search NIH Clinical Center for Craniopharyngioma

Cochrane evidence based reviews: craniopharyngioma

Genetic Tests for Craniopharyngioma

Anatomical Context for Craniopharyngioma

Organs/tissues related to Craniopharyngioma:

MalaCards : Pituitary, Hypothalamus, Brain, Bone, Spinal Cord, Temporal Lobe, Pineal

Publications for Craniopharyngioma

Articles related to Craniopharyngioma:

(show top 50) (show all 3679)
# Title Authors PMID Year
1
Prospective enterprise-level molecular genotyping of a cohort of cancer patients. 5
25157968 2014
2
Novel immunohistochemical markers in the diagnosis of nonglial tumors of nervous system. 53 62
20179436 2010
3
The differential diagnosis of central nervous system tumors: a critical examination of some recent immunohistochemical applications. 53 62
18318590 2008
4
Expression of human MCM6 and DNA Topo II alpha in craniopharyngiomas and its correlation with recurrence of the tumor. 53 62
17410335 2007
5
Acquired prolactin deficiency in patients with disorders of the hypothalamic-pituitary axis. 53 62
17556861 2007
6
[Expression of minichromosome maintenance protein 6 in craniopharyngioma and its correlation with prognosis]. 53 62
17294730 2007
7
Mutation in exon 7 of PTCH deregulates SHH/PTCH/SMO signaling: possible linkage to WNT. 53 62
16596257 2006
8
Hypersomnolence and increased REM sleep with low cerebrospinal fluid hypocretin level in a patient after removal of craniopharyngioma. 53 62
16084764 2005
9
Expression and growth dependency of the insulin-like growth factor I receptor in craniopharyngioma cells: a novel therapeutic approach. 53 62
16000560 2005
10
Beta-catenin mutations in craniopharyngiomas and pituitary adenomas. 53 62
15980970 2005
11
Common mutations of beta-catenin in adamantinomatous craniopharyngiomas but not in other tumours originating from the sellar region. 53 62
15891929 2005
12
The clinical, metabolic and endocrine features and the quality of life in adults with childhood-onset craniopharyngioma compared with adult-onset craniopharyngioma. 53 62
15817911 2005
13
Possible linkage between specific histological structures and aberrant reactivation of the Wnt pathway in adamantinomatous craniopharyngioma. 53 62
15221941 2004
14
Beta-catenin mutations are frequent in calcifying odontogenic cysts, but rare in ameloblastomas. 53 62
14578169 2003
15
Growth without growth hormone. 53 62
12510977 2002
16
Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. 53 62
12466115 2002
17
Differential expression of cytokeratins 8 and 20 distinguishes craniopharyngioma from rathke cleft cyst. 53 62
12296753 2002
18
Craniopharyngioma: a case report and comparative galectin histochemical analysis. 53 62
12495217 2002
19
Pseudotumor of the pituitary due to PROP-1 deletion. 53 62
11822586 2002
20
A study of the growth-promoting and metabolic effects of growth hormone (GH) in a patient with the "growth without GH" syndrome. 53 62
11735238 2001
21
Different keratin profiles in craniopharyngioma subtypes and ameloblastomas. 53 62
11770017 2001
22
The use of insulin-like growth factor 1 reference values for the diagnosis of growth hormone deficiency in prepubertal children. 53 62
10848878 2000
23
A boy with normal growth in spite of growth hormone deficiency after resection of a suprasellar teratoma. 53 62
10890195 2000
24
Somatostatin receptor tumor imaging (Tc 99m P829) in pituitary adenoma. 53 62
10659562 1999
25
Serum IGF-I and IGFBP-3 concentrations do not accurately predict growth hormone deficiency in children with brain tumours. 53 62
10469014 1999
26
The prevalence of severe growth hormone deficiency in adults who received growth hormone replacement in childhood [see comment]. 53 62
8729528 1996
27
Effective use of magnetic resonance imaging in the assessment of children with possible growth hormone deficiency. 53 62
15251557 1996
28
Could craniopharyngiomas produce pituitary hormones? 53 62
8098858 1993
29
Discontinuation of growth hormone therapy in growth-hormone-deficient patients: assessment of body fat mass using bioelectrical impedance. 53 62
8314202 1993
30
Pulsatile prolactin secretion in hyperprolactinemia due to presumed pituitary stalk interruption. 53 62
1955511 1991
31
Demonstration of insulin-like growth factors I, II and heterogeneous insulin-like growth factor binding proteins in the cyst fluid of patients with craniopharyngioma. 53 62
1719442 1991
32
Extended endoscopic transsphenoidal approach for suprasellar craniopharyngiomas. 62
36460851 2022
33
Neuroendoscopic surgery combined with Ommaya reservoir placement for cystic craniopharyngiomas: 11 years of experience in a single institution. 62
36469601 2022
34
Limited surgery and conformal photon radiation therapy for pediatric craniopharyngioma: long-term results from the RT1 protocol. 62
35556133 2022
35
Lesion network mapping of ectopic craniopharyngioma identifies potential cause of psychosis: a case report. 62
36109364 2022
36
Combined pre-retrochiasmatic resection of third ventricle craniopharyngioma by endoscopic endonasal approach. 62
36074182 2022
37
Commentary: Resection of Giant Craniopharyngioma: Contending With Multiple Compartments and Myriad Perforating Arteries: 2-Dimensional Operative Video. 62
36251415 2022
38
Inhibition of integrated stress response protects against lipid-induced senescence in hypothalamic neural stem cells in adamantinomatous craniopharyngioma. 62
36454228 2022
39
Endoscopic endonasal surgery for craniopharyngioma in an 18-month-old boy. 62
35717300 2022
40
Natural pregnancy after gross total resection of a large craniopharyngioma with hypothalamic involvement: a case report. 62
34406611 2022
41
Clinical, Radiographic and Histopathological Analysis of Craniopharyngiomas and Ameloblastomas: A Systematic Review. 62
35920974 2022
42
Posterior hypothalamic involvement on pre-operative MRI predicts hypothalamic obesity in craniopharyngiomas. 62
36462067 2022
43
Endocrine outcomes after limited surgery and conformal photon radiation therapy for pediatric craniopharyngioma: Long-term results from the RT1 protocol. 62
35481829 2022
44
Outcomes of endoscopic endonasal resection of pediatric craniopharyngiomas. 62
35485414 2022
45
Multidisciplinary Management of Craniopharyngiomas in Children: A Single Center Experience. 62
36359587 2022
46
Minimally invasive endoscopic approaches to pediatric skull base pathologies. 62
36206699 2022
47
Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL-6/STAT3 signaling. 62
36328767 2022
48
A Latent Profile Analysis of Sleep, Anxiety, and Mood in Youth with Craniopharyngioma. 62
34719997 2022
49
Safety and Efficacy of Pediatric Growth Hormone Therapy: Results From the Full KIGS Cohort. 62
36102184 2022
50
The importance of preserving the superior hypophyseal artery infundibular branch in craniopharyngioma surgery. 62
36355231 2022

Variations for Craniopharyngioma

ClinVar genetic disease variations for Craniopharyngioma:

5 (show all 12)
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 APC NM_000038.6(APC):c.3183_3187del (p.Lys1061_Gln1062insTer) DEL Pathogenic
88913 rs587779352 GRCh37: 5:112174471-112174475
GRCh38: 5:112838774-112838778
2 CTNNB1 NM_001904.4(CTNNB1):c.100G>A (p.Gly34Arg) SNV Likely Pathogenic
376232 rs121913399 GRCh37: 3:41266103-41266103
GRCh38: 3:41224612-41224612
3 ERCC2 NM_000400.4(ERCC2):c.1361TCA[2] (p.Ile456del) MICROSAT Likely Pathogenic
620626 rs750123656 GRCh37: 19:45860740-45860742
GRCh38: 19:45357482-45357484
4 CTNNB1 NM_001904.4(CTNNB1):c.101G>T (p.Gly34Val) SNV Likely Pathogenic
17582 rs28931589 GRCh37: 3:41266104-41266104
GRCh38: 3:41224613-41224613
5 CTNNB1 NM_001904.4(CTNNB1):c.94G>T (p.Asp32Tyr) SNV Likely Pathogenic
17581 rs28931588 GRCh37: 3:41266097-41266097
GRCh38: 3:41224606-41224606
6 CTNNB1 NM_001904.4(CTNNB1):c.100G>C (p.Gly34Arg) SNV Likely Pathogenic
376233 rs121913399 GRCh37: 3:41266103-41266103
GRCh38: 3:41224612-41224612
7 TSC1 NM_000368.5(TSC1):c.1355G>C (p.Gly452Ala) SNV Uncertain Significance
411263 rs371093730 GRCh37: 9:135782201-135782201
GRCh38: 9:132906814-132906814
8 PTCH1 NM_000264.5(PTCH1):c.113G>A (p.Gly38Glu) SNV Uncertain Significance
216367 rs143494325 GRCh37: 9:98270531-98270531
GRCh38: 9:95508249-95508249
9 PTCH1 NM_000264.5(PTCH1):c.203G>A (p.Gly68Glu) SNV Uncertain Significance
620601 rs757430199 GRCh37: 9:98268880-98268880
GRCh38: 9:95506598-95506598
10 TSC1 NM_000368.5(TSC1):c.1231C>A (p.Leu411Ile) SNV Uncertain Significance
64790 rs397514840 GRCh37: 9:135785990-135785990
GRCh38: 9:132910603-132910603
11 NF1 NM_001042492.3(NF1):c.4836G>A (p.Arg1612=) SNV Uncertain Significance
457720 rs1555533268 GRCh37: 17:29652838-29652838
GRCh38: 17:31325820-31325820
12 SUFU NM_016169.4(SUFU):c.839G>A (p.Arg280Gln) SNV Uncertain Significance
241089 rs145704867 GRCh37: 10:104356979-104356979
GRCh38: 10:102597222-102597222

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 30)
# Super pathways Score Top Affiliating Genes
1 13.15 TRH SOX2 LEPR LEF1 INS IL6
2
Show member pathways
13.11 SOX2 LEF1 IL6 IGF1 GNRH1 CTNNB1
3
Show member pathways
12.74 INS IL6 CTNNB1 BRAF APC
4
Show member pathways
12.62 LEF1 IGF1 CTNNB1 BRAF APC
5 12.52 LEF1 INS IL6 IGF1 CTNNB1 APC
6
Show member pathways
12.38 SOX2 LEF1 CTNNB1 APC
7
Show member pathways
12.34 APC BRAF CTNNB1 IGF1 LEF1
8
Show member pathways
12.24 IL6 IGF1 GNRH1 CTNNB1 BRAF APC
9 12.15 SOX2 LEF1 CTNNB1 APC
10
Show member pathways
12.11 INS IGF1 GHRL CTNNB1
11 11.96 SOX2 INS IL6 IGF1
12 11.94 INS IL6 IGF1 CTNNB1
13 11.87 LEF1 IL6 CTNNB1 BRAF APC
14 11.83 LEF1 CTNNB1 APC
15 11.82 INS IL6 IGF1 BRAF
16 11.8 INS IL6 IGF1
17
Show member pathways
11.78 LEF1 CTNNB1 APC
18 11.76 KRT14 CTNNB1 BRAF
19 11.65 LEF1 CTNNB1 APC
20 11.59 PRL POU1F1 KRT14 IL6
21 11.57 INS GNRH1 GHRL
22 11.51 IGF1 LEF1 SOX2
23 11.48 SOX2 INS IGF1
24 11.47 SOX2 LEF1 CTNNB1 APC
25 11.42 INS IL6 IGF1
26 11.4 PRL LEPR INS
27 11.39 LEPR INS IL6
28 11.08 SOX2 LEF1 CTNNB1 APC
29 10.75 APC CTNNB1 LEF1
30 10.23 PRL LEPR INS GHRL

GO Terms for Craniopharyngioma

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell population proliferation GO:0008284 10.2 PRL LEF1 INS IL6 IGF1 CTNNB1
2 positive regulation of gene expression GO:0010628 10.15 LEF1 INS IL6 IGF1 CTNNB1 BRAF
3 negative regulation of apoptotic process GO:0043066 10.1 BRAF CTNNB1 GHRL IGF1 IL6 LEF1
4 epithelial to mesenchymal transition GO:0001837 9.91 CTNNB1 IGF1 LEF1
5 positive regulation of epithelial to mesenchymal transition GO:0010718 9.88 CTNNB1 IL6 LEF1
6 positive regulation of cell differentiation GO:0045597 9.76 SOX2 LEF1 INS CTNNB1
7 positive regulation of MAPK cascade GO:0043410 9.4 SOX2 INS IL6 IGF1 GHRL CTNNB1
8 adenohypophysis development GO:0021984 9.35 SOX2 PROP1 POU1F1
9 hair cycle process GO:0022405 9.32 ERCC2 CTNNB1

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

# Name GO ID Score Top Affiliating Genes
1 beta-catenin binding GO:0008013 9.56 PROP1 LEF1 CTNNB1 APC
2 hormone activity GO:0005179 9.32 TRH PRL INS IGF1 GNRH1 GHRL

Sources for Craniopharyngioma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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