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Ectopic pancreatic tissue

MedGen UID:
203053
Concept ID:
C0994638
Finding
Synonyms: Abnormal pancreas location; Structure of ectopic pancreatic tissue
SNOMED CT: Ectopic pancreatic tissue (264304000); Structure of ectopic pancreatic tissue (264304000)
 
HPO: HP:0006278

Definition

The presence of pancreatic tissue outside the normal pancreas, in many cases along the foregut and proximal midgut. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Ectopic pancreatic tissue

Conditions with this feature

Fryns syndrome
MedGen UID:
65088
Concept ID:
C0220730
Disease or Syndrome
Fryns syndrome is characterized by diaphragmatic defects (diaphragmatic hernia, eventration, hypoplasia, or agenesis); characteristic facial appearance (coarse facies, wide-set eyes, a wide and depressed nasal bridge with a broad nasal tip, long philtrum, low-set and anomalous ears, tented vermilion of the upper lip, wide mouth, and a small jaw); short distal phalanges of the fingers and toes (the nails may also be small); pulmonary hypoplasia; and associated anomalies (polyhydramnios, cloudy corneas and/or microphthalmia, orofacial clefting, renal dysplasia / renal cortical cysts, and/or malformations involving the brain, cardiovascular system, gastrointestinal system, and/or genitalia). Survival beyond the neonatal period is rare. Data on postnatal growth and psychomotor development are limited; however, severe developmental delay and intellectual disability are common.

Professional guidelines

PubMed

Perdomo CM, Avilés-Olmos I, Dicker D, Frühbeck G
Rev Endocr Metab Disord 2023 Oct;24(5):795-807. Epub 2023 May 10 doi: 10.1007/s11154-023-09797-2. PMID: 37162651Free PMC Article
Griffin BA
Proc Nutr Soc 2020 Feb;79(1):1-3. doi: 10.1017/S0029665119001149. PMID: 31985390
Villarroya F, Domingo P, Giralt M
Biochim Biophys Acta 2010 Mar;1801(3):392-9. Epub 2009 Sep 30 doi: 10.1016/j.bbalip.2009.09.018. PMID: 19800025

Recent clinical studies

Etiology

Chen Q, Gao Z, Zhang L, Zhang Y, Pan T, Cai D, Xiong Q, Shu Q, Qian Y
J Pediatr Surg 2018 Apr;53(4):676-681. Epub 2017 Dec 5 doi: 10.1016/j.jpedsurg.2017.11.059. PMID: 29331260
Lin XK, Huang XZ, Bao XZ, Zheng N, Xia QZ, Chen CD
Medicine (Baltimore) 2017 Aug;96(32):e7760. doi: 10.1097/MD.0000000000007760. PMID: 28796070Free PMC Article
Betzler A, Mees ST, Pump J, Schölch S, Zimmermann C, Aust DE, Weitz J, Welsch T, Distler M
BMC Surg 2017 May 8;17(1):53. doi: 10.1186/s12893-017-0250-x. PMID: 28482873Free PMC Article
Huang CC, Lai MW, Hwang FM, Yeh YC, Chen SY, Kong MS, Lai JY, Chen JC, Ming YC
Pediatr Neonatol 2014 Oct;55(5):369-75. Epub 2014 Mar 28 doi: 10.1016/j.pedneo.2013.12.005. PMID: 24685339
Rokach A, Izbicki G, Deeb M, Bogot N, Arish N, Hadas-Halperen I, Azulai H, Bohadana A, Golomb E
Diagn Pathol 2013 Oct 23;8:176. doi: 10.1186/1746-1596-8-176. PMID: 24152726Free PMC Article

Diagnosis

Chen Q, Gao Z, Zhang L, Zhang Y, Pan T, Cai D, Xiong Q, Shu Q, Qian Y
J Pediatr Surg 2018 Apr;53(4):676-681. Epub 2017 Dec 5 doi: 10.1016/j.jpedsurg.2017.11.059. PMID: 29331260
Rokach A, Izbicki G, Deeb M, Bogot N, Arish N, Hadas-Halperen I, Azulai H, Bohadana A, Golomb E
Diagn Pathol 2013 Oct 23;8:176. doi: 10.1186/1746-1596-8-176. PMID: 24152726Free PMC Article
Heller RS, Tsugu H, Nabeshima K, Madsen OD
Islets 2010 Mar-Apr;2(2):65-71. doi: 10.4161/isl.2.2.10580. PMID: 21099298
Nijs E, Callahan MJ, Taylor GA
Pediatr Radiol 2005 Apr;35(4):358-73; quiz 457. Epub 2004 Nov 5 doi: 10.1007/s00247-004-1326-1. PMID: 15536562
Macpherson RI
Radiographics 1993 Sep;13(5):1063-80. doi: 10.1148/radiographics.13.5.8210590. PMID: 8210590

Therapy

Kumar A, Baksi A, Yadavalli SD, Aggarwal S
Obes Surg 2021 May;31(5):2344-2345. Epub 2021 Jan 19 doi: 10.1007/s11695-020-05162-3. PMID: 33469860
Yang ZH, Gao JB, Yue SW, Yang XH, Guo H
J Korean Med Sci 2011 Jun;26(6):832-5. Epub 2011 May 18 doi: 10.3346/jkms.2011.26.6.832. PMID: 21655073Free PMC Article

Prognosis

Chen Y, Zhou N, Guo D, He X, Tang H, Wang L, Xu Y, Xu T
Diagn Pathol 2023 Mar 16;18(1):37. doi: 10.1186/s13000-023-01324-w. PMID: 36927376Free PMC Article
Chen Q, Gao Z, Zhang L, Zhang Y, Pan T, Cai D, Xiong Q, Shu Q, Qian Y
J Pediatr Surg 2018 Apr;53(4):676-681. Epub 2017 Dec 5 doi: 10.1016/j.jpedsurg.2017.11.059. PMID: 29331260
Zundler S, Erber R, Agaimy A, Hartmann A, Kiesewetter F, Strobel D, Neurath MF, Wildner D
BMC Cancer 2016 Feb 20;16:130. doi: 10.1186/s12885-016-2184-6. PMID: 26895632Free PMC Article
Borghol S, Diris B, Alberti N, Crombe A, Laurent F
Diagn Interv Imaging 2015 Nov;96(11):1233-6. Epub 2015 Apr 6 doi: 10.1016/j.diii.2015.01.009. PMID: 25857740
Rokach A, Izbicki G, Deeb M, Bogot N, Arish N, Hadas-Halperen I, Azulai H, Bohadana A, Golomb E
Diagn Pathol 2013 Oct 23;8:176. doi: 10.1186/1746-1596-8-176. PMID: 24152726Free PMC Article

Clinical prediction guides

Rodríguez-Seguel E, Villamayor L, Arroyo N, De Andrés MP, Real FX, Martín F, Cano DA, Rojas A
J Pathol 2020 Apr;250(4):362-373. Epub 2020 Jan 20 doi: 10.1002/path.5378. PMID: 31875961
Kotlyarov PM
Ter Arkh 2018 Feb 15;90(2):94-97. doi: 10.26442/terarkh201890294-97. PMID: 30701782
Chen Q, Gao Z, Zhang L, Zhang Y, Pan T, Cai D, Xiong Q, Shu Q, Qian Y
J Pediatr Surg 2018 Apr;53(4):676-681. Epub 2017 Dec 5 doi: 10.1016/j.jpedsurg.2017.11.059. PMID: 29331260
Rokach A, Izbicki G, Deeb M, Bogot N, Arish N, Hadas-Halperen I, Azulai H, Bohadana A, Golomb E
Diagn Pathol 2013 Oct 23;8:176. doi: 10.1186/1746-1596-8-176. PMID: 24152726Free PMC Article
Heller RS, Tsugu H, Nabeshima K, Madsen OD
Islets 2010 Mar-Apr;2(2):65-71. doi: 10.4161/isl.2.2.10580. PMID: 21099298

Recent systematic reviews

Hansen CC, Søreide K
Medicine (Baltimore) 2018 Aug;97(35):e12154. doi: 10.1097/MD.0000000000012154. PMID: 30170459Free PMC Article

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