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Plexiform neurofibroma

MedGen UID:
64640
Concept ID:
C0206728
Neoplastic Process
Synonyms: Elephantiasis Neuromatoses; Elephantiasis Neuromatosis; Neurofibroma, Plexiform; Neurofibromas, Plexiform; Neuroma, Plexiform; Neuromas, Plexiform; Pachydermatocele; Pachydermatoceles; Plexiform Neurofibroma; Plexiform Neurofibromas; Plexiform Neuroma; Plexiform Neuromas; Tumor Royale
SNOMED CT: Plexiform neurofibroma (403818001); Plexiform neurofibroma (41252002); Plexiform neuroma (41252002)
 
HPO: HP:0009732

Definition

A neurofibroma in which Schwann cells proliferate inside the nerve sheath, producing an irregularly thickened, distorted, tortuous structure. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPlexiform neurofibroma

Recent clinical studies

Etiology

Clemens RK, Lillis AP, Perez-Rossello J, Shaikh R, Thalhammer C, Amann-Vesti BR, Alomari AI
Int Angiol 2016 Dec;35(6):613-621. Epub 2016 Feb 11 PMID: 26868134
Wehmeijer LM, Hage JJ, Eberz B, van Beurden M
J Low Genit Tract Dis 2015 Jul;19(3):e55-7. doi: 10.1097/LGT.0000000000000100. PMID: 25658713
Dagalakis U, Lodish M, Dombi E, Sinaii N, Sabo J, Baldwin A, Steinberg SM, Stratakis CA, Widemann BC
J Pediatr 2014 Mar;164(3):620-4. Epub 2013 Dec 8 doi: 10.1016/j.jpeds.2013.10.081. PMID: 24321536Free PMC Article
Friedrich RE, Rother J, Christ G, Lehmann M, Eulenburg CG, Giese M, Scheuer HA
Anticancer Res 2013 Mar;33(3):1081-90. PMID: 23482785
Friedrich RE, Giese M, Stelljes C, Froeder C, Scheuer HA
Anticancer Res 2012 May;32(5):2207-14. PMID: 22593511

Diagnosis

Michal M, Kazakov DV, Hadravský L, Michalová K, Rychlý B, Michal M
Hum Pathol 2017 Feb;60:167-173. Epub 2016 Nov 2 doi: 10.1016/j.humpath.2016.10.010. PMID: 27816720
Czuczwar P, Stepniak A, Szkodziak P, Korolczuk A, Paszkowski T, Wozniak S
J Obstet Gynaecol Res 2016 Nov;42(11):1618-1622. Epub 2016 Sep 19 doi: 10.1111/jog.13116. PMID: 27641440
Lee KH, Yoo SH, Noh GT, Heo WS, Ko BS, Chio JA, Cho HJ, Choi JY, Kim HJ, Sohn W, Park SJ, Park YM
Clin Mol Hepatol 2016 Jun;22(2):276-80. Epub 2016 May 20 doi: 10.3350/cmh.2015.0021. PMID: 27209645Free PMC Article
Wehmeijer LM, Hage JJ, Eberz B, van Beurden M
J Low Genit Tract Dis 2015 Jul;19(3):e55-7. doi: 10.1097/LGT.0000000000000100. PMID: 25658713
Shi L, Liu FJ, Jia QH, Guan H, Lu ZJ
World J Gastroenterol 2014 May 7;20(17):5153-6. doi: 10.3748/wjg.v20.i17.5153. PMID: 24803834Free PMC Article

Therapy

Azizi AA, Slavc I, Theisen BE, Rausch I, Weber M, Happak W, Aszmann O, Hojreh A, Peyrl A, Amann G, Benkoe TM, Wadsak W, Kasprian G, Staudenherz A, Hacker M, Traub-Weidinger T
Pediatr Blood Cancer 2018 Jan;65(1) Epub 2017 Aug 3 doi: 10.1002/pbc.26733. PMID: 28771999
Michal M, Kazakov DV, Hadravský L, Michalová K, Rychlý B, Michal M
Hum Pathol 2017 Feb;60:167-173. Epub 2016 Nov 2 doi: 10.1016/j.humpath.2016.10.010. PMID: 27816720
Varin J, Poulain L, Hivelin M, Nusbaum P, Hubas A, Laurendeau I, Lantieri L, Wolkenstein P, Vidaud M, Pasmant E, Chapuis N, Parfait B
Oncotarget 2016 Jun 14;7(24):35753-35767. doi: 10.18632/oncotarget.7099. PMID: 26840085Free PMC Article
Staser K, Yang FC, Clapp DW
Annu Rev Pathol 2012;7:469-95. Epub 2011 Nov 7 doi: 10.1146/annurev-pathol-011811-132441. PMID: 22077553Free PMC Article
Citak EC, Oguz A, Karadeniz C, Okur A, Memis L, Boyunaga O
Pediatr Hematol Oncol 2008 Sep;25(7):673-8. doi: 10.1080/08880010802315983. PMID: 18850480

Prognosis

Azizi AA, Slavc I, Theisen BE, Rausch I, Weber M, Happak W, Aszmann O, Hojreh A, Peyrl A, Amann G, Benkoe TM, Wadsak W, Kasprian G, Staudenherz A, Hacker M, Traub-Weidinger T
Pediatr Blood Cancer 2018 Jan;65(1) Epub 2017 Aug 3 doi: 10.1002/pbc.26733. PMID: 28771999
Michal M, Kazakov DV, Hadravský L, Michalová K, Rychlý B, Michal M
Hum Pathol 2017 Feb;60:167-173. Epub 2016 Nov 2 doi: 10.1016/j.humpath.2016.10.010. PMID: 27816720
Wehmeijer LM, Hage JJ, Eberz B, van Beurden M
J Low Genit Tract Dis 2015 Jul;19(3):e55-7. doi: 10.1097/LGT.0000000000000100. PMID: 25658713
Shi L, Liu FJ, Jia QH, Guan H, Lu ZJ
World J Gastroenterol 2014 May 7;20(17):5153-6. doi: 10.3748/wjg.v20.i17.5153. PMID: 24803834Free PMC Article
Gleiss J, Demestre M, Spyra M, Eschenhagen T, Hagel C, Mautner VF, Kluwe L, Friedrich RE
In Vivo 2013 Nov-Dec;27(6):723-7. PMID: 24292574

Clinical prediction guides

Azizi AA, Slavc I, Theisen BE, Rausch I, Weber M, Happak W, Aszmann O, Hojreh A, Peyrl A, Amann G, Benkoe TM, Wadsak W, Kasprian G, Staudenherz A, Hacker M, Traub-Weidinger T
Pediatr Blood Cancer 2018 Jan;65(1) Epub 2017 Aug 3 doi: 10.1002/pbc.26733. PMID: 28771999
Michal M, Kazakov DV, Hadravský L, Michalová K, Rychlý B, Michal M
Hum Pathol 2017 Feb;60:167-173. Epub 2016 Nov 2 doi: 10.1016/j.humpath.2016.10.010. PMID: 27816720
Hirbe AC, Dahiya S, Miller CA, Li T, Fulton RS, Zhang X, McDonald S, DeSchryver K, Duncavage EJ, Walrath J, Reilly KM, Abel HJ, Pekmezci M, Perry A, Ley TJ, Gutmann DH
Clin Cancer Res 2015 Sep 15;21(18):4201-11. Epub 2015 Apr 29 doi: 10.1158/1078-0432.CCR-14-3049. PMID: 25925892Free PMC Article
Shi L, Liu FJ, Jia QH, Guan H, Lu ZJ
World J Gastroenterol 2014 May 7;20(17):5153-6. doi: 10.3748/wjg.v20.i17.5153. PMID: 24803834Free PMC Article
Stricker TP, Henriksen KJ, Tonsgard JH, Montag AG, Krausz TN, Pytel P
Mod Pathol 2013 Jul;26(7):930-43. Epub 2013 Feb 1 doi: 10.1038/modpathol.2012.242. PMID: 23370767

Recent systematic reviews

Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free PMC Article
Tovmassian D, Abdul Razak M, London K
Int J Surg Oncol 2016;2016:6162182. Epub 2016 Dec 12 doi: 10.1155/2016/6162182. PMID: 28058117Free PMC Article
Plotkin SR, Davis SD, Robertson KA, Akshintala S, Allen J, Fisher MJ, Blakeley JO, Widemann BC, Ferner RE, Marcus CL; REiNS International Collaboration.
Neurology 2016 Aug 16;87(7 Suppl 1):S13-20. doi: 10.1212/WNL.0000000000002933. PMID: 27527645Free PMC Article
Bernier A, Larbrisseau A, Perreault S
Pediatr Neurol 2016 Jul;60:24-29.e1. Epub 2016 Mar 19 doi: 10.1016/j.pediatrneurol.2016.03.003. PMID: 27212418
Arun D, Gutmann DH
Curr Opin Neurol 2004 Apr;17(2):101-5. PMID: 15021234

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