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Epignathus

MedGen UID:
540117
•Concept ID:
C0266725
•
Neoplastic Process
Synonyms: epignathus; epignathus (disease); Oropharyngeal teratoma; oropharyngeal teratoma; Palatine teratoma
SNOMED CT: Epignathus (31248004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0030767
Monarch Initiative: MONDO:0015386
Orphanet: ORPHA141077

Definition

Epignathus is a teratoma originating from the upper jaw, usually connected with the sphenoid bone or hard palate. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpignathus
Follow this link to review classifications for Epignathus in Orphanet.

Professional guidelines

PubMed

Prenatal diagnosis of cervical masses by magnetic resonance imaging and 3D virtual models: perinatal and long-term follow-up outcomes.
Paula Pinho Matos A, Teixeira Castro P, de Barros Duarte L, Dutra Moraes Barbosa A, Daltro P, Fazecas T, Nogueira R, Werner H, Araujo Júnior E
J Matern Fetal Neonatal Med 2020 Jul;33(13):2181-2189. Epub 2018 Nov 20 doi: 10.1080/14767058.2018.1543393. PMID: 30458651
Prenatal diagnosis of a giant epignathus teratoma in the third trimester of pregnancy using three-dimensional ultrasound and magnetic resonance imaging. Case report.
Santana EF, Helfer TM, Piassi Passos J, Araujo Júnior E
Med Ultrason 2014 Jun;16(2):168-71. doi: 10.11152/mu.201.3.2066.162.efms1. PMID: 24791849
Cervical and oral teratoma in the fetus: a systematic review of etiology, pathology, diagnosis, treatment and prognosis.
Tonni G, De Felice C, Centini G, Ginanneschi C
Arch Gynecol Obstet 2010 Oct;282(4):355-61. Epub 2010 May 15 doi: 10.1007/s00404-010-1500-7. PMID: 20473617

Recent clinical studies

Etiology

Three-Month Old with a Tongue Mass.
Schubert G, Reed A, Arora R
J Pediatr 2017 Aug;187:332-332.e1. Epub 2017 May 8 doi: 10.1016/j.jpeds.2017.04.006. PMID: 28495177
Fetal airway management on placental support: limitations and ethical considerations in seven cases.
Barthod G, Teissier N, Bellarbi N, Viala P, Oury JF, Dray G, Vuillard E, Michel J, Van Den Abbeele T
J Obstet Gynaecol 2013 Nov;33(8):787-94. doi: 10.3109/01443615.2013.823924. PMID: 24219714
Perinatal management of congenital oropharyngeal tumors: the ex utero intrapartum treatment (EXIT) approach.
Laje P, Howell LJ, Johnson MP, Hedrick HL, Flake AW, Adzick NS
J Pediatr Surg 2013 Oct;48(10):2005-10. doi: 10.1016/j.jpedsurg.2013.02.031. PMID: 24094948
Massive immature teratoma in a neonate.
Sumiyoshi S, Machida J, Yamamoto T, Fukano H, Shimozato K, Fujimoto Y, Kaetsu A
Int J Oral Maxillofac Surg 2010 Oct;39(10):1020-3. Epub 2010 May 21 doi: 10.1016/j.ijom.2010.04.008. PMID: 20483564
Epignathus: two cases.
Freitas Rda S, Alonso N, Azzolini Tde F, Gianini-Romano G, Tolazzi AR, Busato L, Grande CV
Br J Oral Maxillofac Surg 2008 Jun;46(4):317-9. Epub 2007 Aug 22 doi: 10.1016/j.bjoms.2007.06.012. PMID: 17716791

Diagnosis

Epignathus Teratoma.
Akhtar R, Riffat M, Mehmood Z
J Coll Physicians Surg Pak 2016 May;26(5):438-40. PMID: 27225155
Prenatal diagnosis of severe epignathus in a twin: case report and review of the literature.
Tonni G, Centini G, Inaudi P, Rosignoli L, Ginanneschi C, De Felice C
Cleft Palate Craniofac J 2010 Jul;47(4):421-5. doi: 10.1597/08-224.1. PMID: 20590464
Cervical and oral teratoma in the fetus: a systematic review of etiology, pathology, diagnosis, treatment and prognosis.
Tonni G, De Felice C, Centini G, Ginanneschi C
Arch Gynecol Obstet 2010 Oct;282(4):355-61. Epub 2010 May 15 doi: 10.1007/s00404-010-1500-7. PMID: 20473617
Prenatal diagnosis of an epignathus: a case report and review of the literature.
Clement K, Chamberlain P, Boyd P, Molyneux A
Ultrasound Obstet Gynecol 2001 Aug;18(2):178-81. doi: 10.1046/j.1469-0705.2001.00456.x. PMID: 11530004
Epignathus teratoma: report of three cases with a review of the literature.
Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P, Nuyts JP, Cuisset JM, Soto-Ares G
Cleft Palate Craniofac J 2000 Jan;37(1):83-91. doi: 10.1597/1545-1569_2000_037_0083_etrotc_2.3.co_2. PMID: 10670895

Therapy

Bilateral tension pneumothorax following rigid bronchoscopy: a report of an epignathus in a newborn delivered by the EXIT procedure with a fatal outcome.
Harar RP, Pratap R, Chadha N, Tolley N
J Laryngol Otol 2005 May;119(5):400-2. doi: 10.1258/0022215053945813. PMID: 15949108

Prognosis

The fatal fetal tumor: a geneticist's perspective.
Arora V, Bijarnia Mahay S, Rao S, Dimri N, Manocha A, Mansukhani C, Mishra R, Gupta S, Dua Puri R, Verma IC
J Matern Fetal Neonatal Med 2021 Mar;34(6):1006-1008. Epub 2019 Jun 3 doi: 10.1080/14767058.2019.1622671. PMID: 31113261
An autopsy case of epignathus (immature teratoma of the soft palate) with intracranial extension but without brain invasion: case report and literature review.
Kirishima M, Yamada S, Shinya M, Onishi S, Goto Y, Kitazono I, Hiraki T, Higashi M, Hida AI, Tanimoto A
Diagn Pathol 2018 Dec 22;13(1):99. doi: 10.1186/s13000-018-0776-y. PMID: 30579363Free PMC Article
Cervical and oral teratoma in the fetus: a systematic review of etiology, pathology, diagnosis, treatment and prognosis.
Tonni G, De Felice C, Centini G, Ginanneschi C
Arch Gynecol Obstet 2010 Oct;282(4):355-61. Epub 2010 May 15 doi: 10.1007/s00404-010-1500-7. PMID: 20473617
Neonatal oral tumors: congenital epulis and epignathus.
Kumar B, Sharma SB
J Pediatr Surg 2008 Sep;43(9):e9-11. doi: 10.1016/j.jpedsurg.2008.03.055. PMID: 18778989
Epignathus teratoma: report of three cases with a review of the literature.
Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P, Nuyts JP, Cuisset JM, Soto-Ares G
Cleft Palate Craniofac J 2000 Jan;37(1):83-91. doi: 10.1597/1545-1569_2000_037_0083_etrotc_2.3.co_2. PMID: 10670895

Clinical prediction guides

Surgical Management of Duplication of the Pituitary Gland-Plus Syndrome With Epignathus, Cleft Palate, Duplication of Mandible, and Lobulated Tongue.
Noguchi T, Sugiyama T, Sasaguri KI, Ono S, Maeda K, Nishino H, Jinbu Y, Mori Y
J Craniofac Surg 2017 Mar;28(2):e141-e144. doi: 10.1097/SCS.0000000000003324. PMID: 28027175
Oropharyngeal teratoma, oral duplication, cervical diplomyelia and anencephaly in a 22-week fetus: A review of the craniofacial teratoma syndrome.
Morlino S, Castori M, Servadei F, Laino L, Silvestri E; Pediatric Craniofacial Malformation (PECRAM) Study Group, Grammatico P
Birth Defects Res A Clin Mol Teratol 2015 Jun;103(6):554-66. Epub 2014 Oct 31 doi: 10.1002/bdra.23327. PMID: 25360518
Fetal airway management on placental support: limitations and ethical considerations in seven cases.
Barthod G, Teissier N, Bellarbi N, Viala P, Oury JF, Dray G, Vuillard E, Michel J, Van Den Abbeele T
J Obstet Gynaecol 2013 Nov;33(8):787-94. doi: 10.3109/01443615.2013.823924. PMID: 24219714
Clinical presentation of epignathus teratoma with cleft palate; and duplication of cranial base, tongue, mandible, and pituitary gland.
Maeda Y, Suenaga H, Sugiyama M, Saijo H, Hoshi K, Mori Y, Takato T
J Craniofac Surg 2013 Jul;24(4):1486-91. doi: 10.1097/SCS.0b013e3182953b1f. PMID: 23851840
Massive immature teratoma in a neonate.
Sumiyoshi S, Machida J, Yamamoto T, Fukano H, Shimozato K, Fujimoto Y, Kaetsu A
Int J Oral Maxillofac Surg 2010 Oct;39(10):1020-3. Epub 2010 May 21 doi: 10.1016/j.ijom.2010.04.008. PMID: 20483564

Recent systematic reviews

Cervical and oral teratoma in the fetus: a systematic review of etiology, pathology, diagnosis, treatment and prognosis.
Tonni G, De Felice C, Centini G, Ginanneschi C
Arch Gynecol Obstet 2010 Oct;282(4):355-61. Epub 2010 May 15 doi: 10.1007/s00404-010-1500-7. PMID: 20473617

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