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Odontogenic keratocysts of the jaw

MedGen UID:
313330
Concept ID:
C1708604
Neoplastic Process
Synonym: Keratocystic odontogenic tumor
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0010603
Monarch Initiative: MONDO:0018648
Orphanet: ORPHA447777

Definition

A benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and potential for aggressive, infiltrative behavior. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOdontogenic keratocysts of the jaw

Conditions with this feature

Gorlin syndrome
MedGen UID:
2554
Concept ID:
C0004779
Neoplastic Process
Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by the development of multiple jaw keratocysts, frequently beginning in the second decade of life, and/or basal cell carcinomas (BCCs) usually from the third decade onward. Approximately 60% of individuals have a recognizable appearance with macrocephaly, frontal bossing, coarse facial features, and facial milia. Most individuals have skeletal anomalies (e.g., bifid ribs, wedge-shaped vertebrae). Ectopic calcification, particularly in the falx, is present in more than 90% of affected individuals by age 20 years. Cardiac and ovarian fibromas occur in approximately 2% and 20% of individuals respectively. Approximately 5% of all children with NBCCS develop medulloblastoma (primitive neuroectodermal tumor), generally the desmoplastic subtype. The risk of developing medulloblastoma is substantially higher in individuals with an SUFU pathogenic variant (33%) than in those with a PTCH1 pathogenic variant (<2%). Peak incidence is at age one to two years. Life expectancy in NBCCS is not significantly different from average.

Professional guidelines

PubMed

Winters R, Garip M, Meeus J, Coropciuc R, Politis C
Br J Oral Maxillofac Surg 2023 Jun;61(5):331-336. Epub 2023 Apr 25 doi: 10.1016/j.bjoms.2023.04.006. PMID: 37248124
Buchbender M, Neukam FW, Lutz R, Schmitt CM
Oral Surg Oral Med Oral Pathol Oral Radiol 2018 May;125(5):399-406. Epub 2017 Dec 29 doi: 10.1016/j.oooo.2017.12.010. PMID: 29396318
Sharif FN, Oliver R, Sweet C, Sharif MO
Cochrane Database Syst Rev 2015 Nov 5;2015(11):CD008464. doi: 10.1002/14651858.CD008464.pub3. PMID: 26545201Free PMC Article

Recent clinical studies

Etiology

Pylkkö J, Willberg J, Suominen A, Laine HK, Rautava J
Clin Exp Dent Res 2023 Oct;9(5):894-898. Epub 2023 Oct 5 doi: 10.1002/cre2.796. PMID: 37794837Free PMC Article
Winters R, Garip M, Meeus J, Coropciuc R, Politis C
Br J Oral Maxillofac Surg 2023 Jun;61(5):331-336. Epub 2023 Apr 25 doi: 10.1016/j.bjoms.2023.04.006. PMID: 37248124
Boffano P, Cavarra F, Agnone AM, Brucoli M, Ruslin M, Forouzanfar T, Ridwan-Pramana A, Rodríguez-Santamarta T, de Vicente JC, Starch-Jensen T, Pechalova P, Pavlov N, Doykova I, Gospodinov D, Konstantinovic VS, Jovanović M, Barrabé A, Louvrier A, Meyer C, Tamme T, Andrianov A, Dovšak T, Birk A, Masu L, Rocchetti V
J Craniomaxillofac Surg 2022 Jan;50(1):1-6. Epub 2021 Oct 4 doi: 10.1016/j.jcms.2021.09.022. PMID: 34625371
Rajendra Santosh AB, Ogle OE
Dent Clin North Am 2020 Jan;64(1):121-138. Epub 2019 Oct 18 doi: 10.1016/j.cden.2019.08.008. PMID: 31735223
Rajendra Santosh AB
Dent Clin North Am 2020 Jan;64(1):105-119. Epub 2019 Oct 18 doi: 10.1016/j.cden.2019.08.002. PMID: 31735221

Diagnosis

Rayamajhi S, Shrestha S, Shakya S, Bhandari S, Twayana AR, Shahi K
JNMA J Nepal Med Assoc 2022 Jul 1;60(251):657-660. doi: 10.31729/jnma.7566. PMID: 36705195Free PMC Article
Soluk-Tekkesin M, Wright JM
Turk Patoloji Derg 2022;38(2):168-184. doi: 10.5146/tjpath.2022.01573. PMID: 35578902Free PMC Article
Bilodeau EA, Hunter KD
Head Neck Pathol 2021 Mar;15(1):71-84. Epub 2021 Mar 15 doi: 10.1007/s12105-020-01284-3. PMID: 33723756Free PMC Article
Rajendra Santosh AB, Ogle OE
Dent Clin North Am 2020 Jan;64(1):121-138. Epub 2019 Oct 18 doi: 10.1016/j.cden.2019.08.008. PMID: 31735223
Bartoš V, Kullová M, Adamicová K, Paučinová I
Klin Onkol 2019 Spring;32(2):124-128. doi: 10.14735/amko2019124. PMID: 30995852

Therapy

Winters R, Garip M, Meeus J, Coropciuc R, Politis C
Br J Oral Maxillofac Surg 2023 Jun;61(5):331-336. Epub 2023 Apr 25 doi: 10.1016/j.bjoms.2023.04.006. PMID: 37248124
Candotto V, Oberti L, Gabrione F, Scarano A, Rossi D, Romano M
J Biol Regul Homeost Agents 2019 May-Jun;33(3 Suppl. 1):169-172. DENTAL SUPPLEMENT. PMID: 31538464
Gupta K, Chaturvedi TP, Gupta J, Agrawal R
Biotech Histochem 2019 Jul;94(5):348-351. Epub 2019 Feb 26 doi: 10.1080/10520295.2019.1571226. PMID: 30806083
Sharif FN, Oliver R, Sweet C, Sharif MO
Cochrane Database Syst Rev 2015 Nov 5;2015(11):CD008464. doi: 10.1002/14651858.CD008464.pub3. PMID: 26545201Free PMC Article
Johnson NR, Batstone MD, Savage NW
Oral Surg Oral Med Oral Pathol Oral Radiol 2013 Oct;116(4):e271-6. Epub 2012 Jul 6 doi: 10.1016/j.oooo.2011.12.028. PMID: 22771402

Prognosis

Heiliczer S, Shmuly T, Avishai G, Zlotogorski-Hurvitz A, Vered M, Mamber L, Kaplan I
Oral Dis 2023 Nov;29(8):3306-3312. Epub 2022 Dec 15 doi: 10.1111/odi.14415. PMID: 36305228
Garg A, Malhotra R, Urs AB
J Oral Biosci 2022 Jun;64(2):202-209. Epub 2022 Apr 6 doi: 10.1016/j.job.2022.03.005. PMID: 35398253
Bilodeau EA, Hunter KD
Head Neck Pathol 2021 Mar;15(1):71-84. Epub 2021 Mar 15 doi: 10.1007/s12105-020-01284-3. PMID: 33723756Free PMC Article
Bartoš V, Kullová M, Adamicová K, Paučinová I
Klin Onkol 2019 Spring;32(2):124-128. doi: 10.14735/amko2019124. PMID: 30995852
Trosman SJ, Krakovitz PR
Otolaryngol Clin North Am 2015 Feb;48(1):101-19. doi: 10.1016/j.otc.2014.09.008. PMID: 25442129

Clinical prediction guides

Ruddocks LA, Fitzpatrick SG, Bhattacharyya I, Islam MN, Cohen DM
J Am Dent Assoc 2022 May;153(5):421-428. Epub 2022 Feb 4 doi: 10.1016/j.adaj.2021.10.002. PMID: 35125168
Polak K, Jędrusik-Pawłowska M, Drozdzowska B, Morawiec T
Dent Med Probl 2019 Oct-Dec;56(4):433-436. doi: 10.17219/dmp/110682. PMID: 31689015
Bonavolontà P, Dell'Aversana Orabona G, Friscia M, Sani L, Abbate V, Iaconetta G, Califano L
J Craniofac Surg 2019 Oct;30(7):e658-e661. doi: 10.1097/SCS.0000000000005725. PMID: 31261346
Rojo R, Prados-Frutos JC, Gutierrez Lázaro I, Herguedas Alonso JA
J Stomatol Oral Maxillofac Surg 2017 Apr;118(2):122-124. Epub 2017 Feb 11 doi: 10.1016/j.jormas.2016.10.007. PMID: 28345512
Moura BS, Cavalcante MA, Hespanhol W
Rev Col Bras Cir 2016 Dec;43(6):466-471. doi: 10.1590/0100-69912016006013. PMID: 28273221

Recent systematic reviews

Sachdev SS, Chettiankandy TJ, Adhane YB, Sardar MA, Trimukhe A, Jain R
Sultan Qaboos Univ Med J 2023 Aug;23(3):288-296. Epub 2023 Aug 28 doi: 10.18295/squmj.5.2023.021. PMID: 37655071Free PMC Article
Winters R, Garip M, Meeus J, Coropciuc R, Politis C
Br J Oral Maxillofac Surg 2023 Jun;61(5):331-336. Epub 2023 Apr 25 doi: 10.1016/j.bjoms.2023.04.006. PMID: 37248124
Buchbender M, Neukam FW, Lutz R, Schmitt CM
Oral Surg Oral Med Oral Pathol Oral Radiol 2018 May;125(5):399-406. Epub 2017 Dec 29 doi: 10.1016/j.oooo.2017.12.010. PMID: 29396318
Musu D, Rossi-Fedele G, Campisi G, Cotti E
Oral Surg Oral Med Oral Pathol Oral Radiol 2016 Jul;122(1):e19-29. Epub 2016 Apr 7 doi: 10.1016/j.oooo.2016.03.022. PMID: 27260284
Johnson NR, Batstone MD, Savage NW
Oral Surg Oral Med Oral Pathol Oral Radiol 2013 Oct;116(4):e271-6. Epub 2012 Jul 6 doi: 10.1016/j.oooo.2011.12.028. PMID: 22771402

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