KTOC
MCID: KRT063
MIFTS: 40

Keratocystic Odontogenic Tumor (KTOC)

Categories: Oral diseases, Rare diseases

Aliases & Classifications for Keratocystic Odontogenic Tumor

MalaCards integrated aliases for Keratocystic Odontogenic Tumor:

Name: Keratocystic Odontogenic Tumor 59 72
Tumor, Keratocystic Odontogenic 40
Odontogenic Keratocystoma 59
Ktoc 59

Characteristics:

Orphanet epidemiological data:

59
keratocystic odontogenic tumor
Inheritance: Not applicable;

Classifications:

Orphanet: 59  
Rare odontological diseases


External Ids:

ICD10 via Orphanet 34 D16.4
Orphanet 59 ORPHA447777
UMLS 72 C1708604

Summaries for Keratocystic Odontogenic Tumor

MalaCards based summary : Keratocystic Odontogenic Tumor, also known as tumor, keratocystic odontogenic, is related to basal cell nevus syndrome and ameloblastoma, and has symptoms including pain An important gene associated with Keratocystic Odontogenic Tumor is PTCH1 (Patched 1), and among its related pathways/superpathways are Cytokine Signaling in Immune system and G-Beta Gamma Signaling. Affiliated tissues include bone, endothelial and b cells, and related phenotypes are digestive/alimentary and cardiovascular system

Related Diseases for Keratocystic Odontogenic Tumor

Diseases related to Keratocystic Odontogenic Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 132)
# Related Disease Score Top Affiliating Genes
1 basal cell nevus syndrome 32.8 SUFU SMO SHH PTCH1 GLI1
2 ameloblastoma 31.8 TNFSF11 SMO PTCH1 GLI1
3 basal cell carcinoma 30.7 TP53 SUFU SMO SHH PTCH1 GLI1
4 oral cancer 30.2 TP53 PTGS2 MMP9 CCND1
5 squamous cell carcinoma 29.4 TP53 PTHLH PTGS2 PTCH1 PDPN MKI67
6 medulloblastoma 28.6 TP53 SUFU SMO SHH PTCH1 HPSE
7 9q22.3 microdeletion 11.6
8 brain ependymoma 10.7 TP53 MKI67
9 calcifying epithelial odontogenic tumor 10.7 TP53 PTCH1
10 clear cell cystadenofibroma 10.7 TP53 CALB2
11 vulvar intraepithelial neoplasia 10.7 TP53 BIRC5
12 basal cell carcinoma, multiple 10.6 SMO PTCH1
13 protoplasmic astrocytoma 10.6 TP53 PTGS2
14 bone resorption disease 10.6
15 cervical carcinosarcoma 10.6 TP53 BIRC5 BCL2
16 soft tissue sarcoma 10.6 TP53 MKI67 BCL2
17 midline interhemispheric variant of holoprosencephaly 10.6 SHH PTCH1
18 septopreoptic holoprosencephaly 10.5 SHH PTCH1
19 recurrent respiratory papillomatosis 10.5 TP53 PTGS2
20 bladder transitional cell papilloma 10.5 TP53 BIRC5
21 hemophilic arthropathy 10.5 TNFSF11 PTGS2
22 ovarian serous cystadenocarcinoma 10.5 TP53 BIRC5 BCL2
23 focal dermal hypoplasia 10.5
24 focal epithelial hyperplasia 10.5 TP53 SHH GLI1
25 oral leukoplakia 10.5 TP53 MKI67 CCND1
26 villonodular synovitis 10.5 TNFSF11 MMP9
27 gastric tubular adenocarcinoma 10.4 SHH PTGS2 GLI1
28 epidermoid cysts 10.4
29 b-cell lymphoma 10.4
30 spitz nevus 10.4 TP53 MKI67
31 suppressor of tumorigenicity 3 10.4 TP53 FHIT BCL2
32 inverted papilloma 10.4 TP53 MKI67 CCND1
33 bowenoid papulosis 10.4 PTGS2 CCND1
34 intestinal benign neoplasm 10.4 TP53 PTGS2 CCND1
35 barrett's adenocarcinoma 10.4 TP53 PTGS2 FHIT
36 cervical intraepithelial neoplasia 10.4 MKI67 FHIT BIRC5
37 colonic disease 10.4 TP53 PTGS2 CCND1
38 differentiated thyroid carcinoma 10.4 TP53 FHIT BCL2
39 intestinal disease 10.3 TP53 PTGS2 CCND1
40 hard palate cancer 10.3
41 paresthesia 10.3
42 hidradenocarcinoma 10.3 TP53 MKI67 CCND1 BCL2
43 pigmented villonodular synovitis 10.3 TP53 TNFSF11 MMP9
44 tooth ankylosis 10.3 TNFSF11 PTHLH
45 cervix disease 10.3 TP53 FHIT CCND1
46 nervous system cancer 10.3 TP53 SHH PTCH1 CCND1
47 brachydactyly, type e1 10.3 PTHLH PTGS2 PTCH1
48 adenocarcinoma 10.2 TP53 PTGS2 CCND1 BIRC5
49 central nervous system tuberculosis 10.2 SHH MMP9 GLI1
50 ductal carcinoma in situ 10.2 TP53 PTGS2 MKI67 CCND1

Graphical network of the top 20 diseases related to Keratocystic Odontogenic Tumor:



Diseases related to Keratocystic Odontogenic Tumor

Symptoms & Phenotypes for Keratocystic Odontogenic Tumor

UMLS symptoms related to Keratocystic Odontogenic Tumor:


pain

MGI Mouse Phenotypes related to Keratocystic Odontogenic Tumor:

46 (show all 24)
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 10.44 BCL2 CCND1 CTNNBIP1 FHIT GLI1 MMP9
2 cardiovascular system MP:0005385 10.42 BCL2 CCND1 CTNNBIP1 HPSE MMP9 PDPN
3 cellular MP:0005384 10.42 BCL2 BIRC5 CCND1 CTNNBIP1 GLI1 HPSE
4 growth/size/body region MP:0005378 10.41 BCL2 CCND1 CTNNBIP1 GLI1 MMP9 PDPN
5 behavior/neurological MP:0005386 10.4 BCL2 CALB2 CCND1 GLI1 MMP9 PDPN
6 hematopoietic system MP:0005397 10.4 BCL2 BIRC5 CCND1 CTNNBIP1 FHIT HPSE
7 mortality/aging MP:0010768 10.4 BCL2 BIRC5 CCND1 CTNNBIP1 FHIT GLI1
8 homeostasis/metabolism MP:0005376 10.39 BCL2 BIRC5 CCND1 FHIT MMP9 PDPN
9 immune system MP:0005387 10.38 BCL2 BIRC5 CCND1 FHIT HPSE MMP9
10 craniofacial MP:0005382 10.34 BCL2 CCND1 CTNNBIP1 GLI1 PTCH1 PTHLH
11 integument MP:0010771 10.34 BCL2 CCND1 FHIT HPSE MMP9 PDPN
12 endocrine/exocrine gland MP:0005379 10.32 BCL2 BIRC5 CCND1 GLI1 HPSE PTCH1
13 embryo MP:0005380 10.28 BCL2 BIRC5 CTNNBIP1 GLI1 PTCH1 PTGS2
14 nervous system MP:0003631 10.25 BCL2 CALB2 CCND1 CTNNBIP1 GLI1 MMP9
15 neoplasm MP:0002006 10.23 BCL2 CCND1 FHIT GLI1 MMP9 PTCH1
16 normal MP:0002873 10.18 BIRC5 CALB2 CCND1 GLI1 MKI67 PTCH1
17 limbs/digits/tail MP:0005371 10.16 GLI1 MMP9 PTCH1 PTHLH SHH SMO
18 muscle MP:0005369 10.13 BCL2 MMP9 PDPN PTCH1 PTGS2 SHH
19 no phenotypic analysis MP:0003012 10.01 GLI1 MKI67 PTCH1 PTGS2 SHH SUFU
20 respiratory system MP:0005388 10 CCND1 CTNNBIP1 GLI1 HPSE MMP9 PDPN
21 renal/urinary system MP:0005367 9.97 BCL2 CTNNBIP1 GLI1 MMP9 PTCH1 PTGS2
22 reproductive system MP:0005389 9.96 BCL2 CCND1 GLI1 MMP9 PTCH1 PTGS2
23 skeleton MP:0005390 9.73 CCND1 CTNNBIP1 MMP9 PDPN PTCH1 PTGS2
24 vision/eye MP:0005391 9.4 BCL2 CCND1 CTNNBIP1 MMP9 PTCH1 PTGS2

Drugs & Therapeutics for Keratocystic Odontogenic Tumor

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase 2, Single-center, Single-arm, Open Label Trial of Vismodegib in Patients With Keratocystic Odontogenic Tumors Active, not recruiting NCT02366312 Phase 2 vismodegib

Search NIH Clinical Center for Keratocystic Odontogenic Tumor

Genetic Tests for Keratocystic Odontogenic Tumor

Anatomical Context for Keratocystic Odontogenic Tumor

MalaCards organs/tissues related to Keratocystic Odontogenic Tumor:

41
Bone, Endothelial, B Cells, Colon

Publications for Keratocystic Odontogenic Tumor

Articles related to Keratocystic Odontogenic Tumor:

(show top 50) (show all 334)
# Title Authors PMID Year
1
Cell proliferation proteins and aggressiveness of histological variants of ameloblastoma and keratocystic odontogenic tumor. 38
30806083 2019
2
Cysts and tumors of the jaws treated by marsupialization: A description of 4 clinical cases. 38
31346379 2019
3
Orthokeratinized odontogenic cyst (OOC): Clinicopathological and radiological features of a series of 10 cases. 38
30947718 2019
4
Multidisciplinary oral rehabilitation of an adolescent suffering from juvenile Gorlin-Goltz syndrome - a case report. 38
30736811 2019
5
Cytokeratin and fibronectin expression in orthokeratinized odontogenic cyst: A comparative immunohistochemical study. 38
31110419 2019
6
Protein 53, B-Cell Lymphoma-2, Cyclooxygenase-2, and CD105 Reactivity in Keratocystic Odontogenic Tumors: An Immunohistochemical Analysis. 38
30820416 2019
7
Tetrad presentation of non-syndromic odontogenic keratocyst: An uphill diagnostic and therapeutic challenge. 38
30648369 2018
8
Pediatric case of squamous cell carcinoma arising from a keratocystic odontogenic tumor. 38
30055720 2018
9
Expression of WNT1 in ameloblastoma and its significance. 38
30008830 2018
10
Retrospective clinical study of multiple keratocystic odontogenic tumors in non-syndromic patients. 38
29963491 2018
11
A Comparative Study of Macrophage Density in Odontogenic Cysts and Tumors with Diverse Clinical Behavior. 38
29854889 2018
12
A Case of an Extensive Keratocystic Odontogenic Tumor in the Mandible Reconstructed with a Custom-Made Total Joint Prosthesis. 38
29892328 2018
13
Quantitative analysis of Argyrophilic Nucleolar organizer regions in odontogenic cysts and tumor - A comparative study. 38
29892529 2018
14
Amelogenin is a Potential Biomarker for the Aggressiveness in Odontogenic Tumors 38
29802703 2018
15
Volumetric analysis of keratocystic odontogenic tumors and non-neoplastic jaw cysts - Comparison and its clinical relevance. 38
29233700 2018
16
Management of odontogenic cysts by endonasal endoscopic techniques: A systematic review and case series. 38
29336289 2018
17
Role of HIF-1α and CASPASE-3 in cystogenesis of odontogenic cysts and tumors. 38
28238093 2018
18
Comparison of osteoclastogenesis and local invasiveness of ameloblastoma and keratocystic odontogenic tumor. 38
29657523 2018
19
Trigeminal neuralgia induced by odontogenic keratocyst associated with impacted supernumerary teeth: A rare case report. 38
29491624 2018
20
Keratocystic Odontogenic Tumor Involving the Orbital Apex. 38
28099232 2018
21
Squamous cell carcinoma arising from a keratocystic odontogenic tumor: a case report. 38
29191248 2017
22
Molecular analysis of keratocystic odontogenic tumor cell lines derived from sporadic and basal cell nevus syndrome patients. 38
29039452 2017
23
Sporadic versus syndromic keratocysts-Can we predict treatment outcome? A review of 102 cysts. 38
28449413 2017
24
Controversies in Oral and Maxillofacial Pathology. 38
28893467 2017
25
Frequent oncogenic BRAF V600E mutation in odontogenic keratocyst. 38
29103753 2017
26
Reconstruction of Mandibular Segmental Detects Using Transport Disk Distraction Osteogenesis. 38
28968331 2017
27
Kallikrein-related peptidase expression in odontogenic cysts and tumors: An immunohistochemical comparative study. 38
28054463 2017
28
Ortho Keratinized Odontogenic Cyst with Dentinoid Formation. 38
28969743 2017
29
Immunohistochemical evaluation of inducible nitric oxide synthase in the epithelial lining of odontogenic cysts: A qualitative and quantitative analysis. 38
29391711 2017
30
FAK is overexpressed in keratocystic odontogenic tumor: a preliminary study. 38
27935113 2017
31
[Expression of receptor activator of nuclear factor-κB ligand in keratocystic odontogenic tumor before and after decompression]. 38
29199341 2017
32
Computer-aided diagnosis of periapical cyst and keratocystic odontogenic tumor on cone beam computed tomography. 38
28688493 2017
33
A study of the distribution of B-cell lymphoma/leukemia-2 in odontogenic cyst and tumors: Histochemical study. 38
28862228 2017
34
Retrospective analysis of nonendodontic periapical lesions misdiagnosed as endodontic apical periodontitis lesions in a population of Taiwanese patients. 38
27853924 2017
35
Decompression of keratocystic odontogenic tumors leading to increased fibrosis, but without any change in epithelial proliferation. 38
28377093 2017
36
Orthokeratinized Odontogenic Cyst with an Associated Keratocystic Odontogenic Tumor Component and Ghost Cell Keratinization and Calcifications in a Patient with Gardner Syndrome. 38
27501963 2017
37
Correlation between ploidy status using flow cytometry and nucleolar organizer regions in benign and malignant epithelial odontogenic tumors. 38
28222389 2017
38
The anecdote of viral etiopathogenia in ameloblastoma and odontogenic keratocyst: Why don't we let it go? 38
28706783 2017
39
Corrigendum to "malignant transformation of keratocystic odontogenic tumor: Two case reports" [American journal of otolaryngology 34 (2013) 357-361]. 38
28341089 2017
40
P53 Protein Expression in Dental Follicle, Dentigerous Cyst, Odontogenic Keratocyst, and Inflammatory Subtypes of Cysts: An Immunohistochemical Study. 38
28584604 2017
41
Use of refrigerant spray of a propane/butane/isobutane gas mixture in the management of keratocystic odontogenic tumors: a preliminary study. 38
27873145 2017
42
Assessing alpha-tocopherol levels in patients with keratocystic odontogenic tumor: A cross-sectional study. 38
28611319 2017
43
Topical 5-Fluorouracil is a Novel Targeted Therapy for the Keratocystic Odontogenic Tumor. 38
27789270 2017
44
Odontogenic Cysts and Neoplasms. 38
28153133 2017
45
Clinicopathological and cell proliferation evaluation of ameloblastomas and keratocystic odontogenic tumors: a 10 year retrospective study. 38
27747382 2017
46
Collagen and Its Role in predicting the Biological Behavior of Odontogenic Lesions. 38
28174367 2017
47
Expression of astrocyte elevated gene-1 protein in ameloblastomas, keratocystic odontogenic tumors, and dentigerous cysts. 38
27381451 2017
48
Automated classification of maxillofacial cysts in cone beam CT images using contourlet transformation and Spherical Harmonics. 38
28187891 2017
49
Expression of YAP/TAZ in Keratocystic Odontogenic Tumors and Its Possible Association with Proliferative Behavior. 38
28512636 2017
50
Orthokeratinized Odontogenic Cyst of the Maxilla: Report of a Case and Review of the Literature. 38
25110243 2017

Variations for Keratocystic Odontogenic Tumor

Expression for Keratocystic Odontogenic Tumor

Search GEO for disease gene expression data for Keratocystic Odontogenic Tumor.

Pathways for Keratocystic Odontogenic Tumor

Pathways related to Keratocystic Odontogenic Tumor according to GeneCards Suite gene sharing:

(show all 36)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.28 TP53 TNFSF11 PTGS2 MMP9 CCND1 BIRC5
2
Show member pathways
12.91 SUFU SMO PTGS2 PTCH1 MMP9 GLI1
3
Show member pathways
12.85 TP53 PTGS2 MMP9 CCND1 BIRC5 BCL2
4
Show member pathways
12.8 TP53 MMP9 CCND1 BIRC5 BCL2
5
Show member pathways
12.66 TP53 TNFSF11 SHH CCND1 BCL2
6
Show member pathways
12.6 TP53 MMP9 FHIT CCND1 BCL2
7
Show member pathways
12.47 SUFU SMO SHH PTCH1 GLI1
8 12.42 TP53 SUFU SMO SHH PTGS2 PTCH1
9 12.4 TP53 PTGS2 MMP9 CCND1 BCL2
10 12.36 TP53 PTGS2 CCND1 BIRC5
11
Show member pathways
12.35 SMO SHH PTHLH PTCH1
12
Show member pathways
12.29 TP53 SUFU SMO SHH PTCH1 GLI1
13 12.12 SUFU SHH PTCH1 GLI1
14 12.07 TP53 PTGS2 MMP9 CCND1 BIRC5
15 12.01 TP53 SMO SHH PTCH1 MMP9 HPSE
16 11.87 TNFSF11 PTHLH BCL2
17 11.87 TP53 PTGS2 MMP9 CCND1
18 11.85 TNFSF11 PTGS2 BCL2
19 11.79 TP53 PTGS2 MMP9 CCND1 BIRC5 BCL2
20 11.78 PTGS2 PDPN MMP9
21 11.73 TP53 MMP9 BIRC5
22 11.73 TP53 PTGS2 FHIT CCND1 BCL2
23 11.71 MMP9 CTNNBIP1 CCND1
24 11.7 PTGS2 CCND1 BCL2
25 11.66 TP53 BIRC5 BCL2
26 11.66 TP53 MMP9 CCND1
27
Show member pathways
11.65 TNFSF11 BIRC5 BCL2
28
Show member pathways
11.58 PTGS2 MMP9 CCND1 BIRC5
29 11.57 PTHLH PTCH1 MMP9
30 11.44 TP53 PTGS2 CCND1 BIRC5
31 11.43 TP53 CCND1 BCL2
32 11.27 TP53 PTGS2 BIRC5
33
Show member pathways
11.18 SMO SHH PTHLH PTCH1
34 10.9 MMP9 HPSE
35 10.76 TNFSF11 SMO SHH PTHLH PTCH1 GLI1
36 10.49 SHH PTCH1

GO Terms for Keratocystic Odontogenic Tumor

Cellular components related to Keratocystic Odontogenic Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytoplasm GO:0005737 9.8 TP53 TNFSF11 SUFU SMO PTHLH PTGS2
2 caveola GO:0005901 9.33 SMO PTGS2 PTCH1
3 ciliary tip GO:0097542 8.8 SUFU SMO GLI1
4 cytosol GO:0005829 10.03 TP53 SUFU SHH PTHLH PDPN GLI1

Biological processes related to Keratocystic Odontogenic Tumor according to GeneCards Suite gene sharing:

(show all 47)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell proliferation GO:0008285 9.99 TP53 PTHLH PTGS2 PTCH1 PDPN BCL2
2 regulation of gene expression GO:0010468 9.97 SMO SHH PTHLH BCL2
3 response to estradiol GO:0032355 9.87 PTGS2 PTCH1 CCND1
4 anterior/posterior pattern specification GO:0009952 9.87 SMO SHH CTNNBIP1
5 lung development GO:0030324 9.87 SHH PDPN GLI1
6 negative regulation of apoptotic process GO:0043066 9.86 TP53 SMO SHH PTGS2 PDPN MMP9
7 determination of left/right symmetry GO:0007368 9.85 SUFU SMO SHH
8 negative regulation of DNA-binding transcription factor activity GO:0043433 9.83 SUFU PTCH1 CTNNBIP1
9 response to glucocorticoid GO:0051384 9.83 PTGS2 CCND1 BCL2
10 pattern specification process GO:0007389 9.83 SMO SHH PTCH1
11 heart looping GO:0001947 9.82 SUFU SMO SHH
12 liver regeneration GO:0097421 9.79 PTCH1 GLI1 CCND1
13 embryonic organ development GO:0048568 9.76 SMO SHH PTCH1
14 positive regulation of smoothened signaling pathway GO:0045880 9.75 SMO SHH GLI1
15 positive regulation of protein import into nucleus GO:0042307 9.74 SMO SHH PTGS2
16 developmental growth GO:0048589 9.73 SMO SHH BCL2
17 smoothened signaling pathway GO:0007224 9.73 SMO SHH PTCH1 GLI1
18 hair follicle morphogenesis GO:0031069 9.72 SMO SHH BCL2
19 renal system development GO:0072001 9.71 SMO SHH PTCH1
20 mammary gland alveolus development GO:0060749 9.7 TNFSF11 CCND1
21 cellular response to cholesterol GO:0071397 9.7 SMO PTCH1
22 ossification GO:0001503 9.7 TNFSF11 SMO BCL2
23 thalamus development GO:0021794 9.69 SMO SHH
24 mammary gland epithelial cell differentiation GO:0060644 9.68 SMO PTCH1
25 negative regulation of epithelial cell differentiation GO:0030857 9.68 SMO CCND1
26 commissural neuron axon guidance GO:0071679 9.68 SMO PTCH1
27 regulation of mitochondrial membrane permeability GO:0046902 9.68 TP53 BCL2
28 mammary gland epithelial cell proliferation GO:0033598 9.67 TNFSF11 CCND1
29 somite development GO:0061053 9.67 SMO SHH PTCH1
30 branching involved in ureteric bud morphogenesis GO:0001658 9.67 SHH PTCH1 CTNNBIP1 BCL2
31 mitotic G1 DNA damage checkpoint GO:0031571 9.65 TP53 CCND1
32 prostate gland development GO:0030850 9.65 SHH PTCH1 GLI1
33 lymphoid progenitor cell differentiation GO:0002320 9.63 SHH BCL2
34 spinal cord dorsal/ventral patterning GO:0021513 9.63 SUFU SHH
35 digestive tract morphogenesis GO:0048546 9.63 SHH GLI1 BCL2
36 epithelial-mesenchymal cell signaling GO:0060684 9.62 SMO SHH
37 cerebellar cortex morphogenesis GO:0021696 9.62 SMO GLI1
38 dorsal/ventral pattern formation GO:0009953 9.62 SMO SHH PTCH1 GLI1
39 ventral midline development GO:0007418 9.61 SHH GLI1
40 positive regulation of hh target transcription factor activity GO:0007228 9.6 SMO SHH
41 determination of left/right asymmetry in lateral mesoderm GO:0003140 9.59 SMO SHH
42 dorsal/ventral neural tube patterning GO:0021904 9.58 SMO SHH PTCH1
43 positive regulation of cell proliferation GO:0008284 9.56 SMO SHH PTHLH PTGS2 GLI1 CCND1
44 smoothened signaling pathway involved in regulation of cerebellar granule cell precursor cell proliferation GO:0021938 9.33 SMO SHH GLI1
45 cytokine-mediated signaling pathway GO:0019221 9.17 TP53 TNFSF11 PTGS2 MMP9 CCND1 BIRC5
46 multicellular organism development GO:0007275 10.18 TP53 TNFSF11 SUFU SMO SHH PDPN
47 negative regulation of transcription by RNA polymerase II GO:0000122 10.14 TP53 SUFU SMO SHH PTCH1 CCND1

Molecular functions related to Keratocystic Odontogenic Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.89 TP53 TNFSF11 SUFU SMO SHH PTGS2
2 chaperone binding GO:0051087 9.33 TP53 PDPN BIRC5
3 patched binding GO:0005113 8.8 SMO SHH PTCH1

Sources for Keratocystic Odontogenic Tumor

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