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Dermatofibrosarcomas

MedGen UID:
104907
Concept ID:
C0206647
Neoplastic Process
Synonyms: Darier Ferrand Tumor; Darier Hoffmann Tumor; Darier-Ferrand Tumor; Darier-Hoffmann Tumor; Dermatofibrosarcoma; Dermatofibrosarcoma Protuberan; Dermatofibrosarcoma Protuberans; Protuberan, Dermatofibrosarcoma; Protuberans, Dermatofibrosarcoma; Tumor, Darier-Ferrand; Tumor, Darier-Hoffmann

Definition

A sarcoma of the deep layers of the skin. The tumors are locally aggressive tends to recur but rarely metastatic. It can be classified into variants depending on the cell type tumors are derived from or by its characteristics: Pigmented variant from MELANIN-containing DERMAL DENDRITIC CELLS; Myxoid variant, myxoid STROMAL CELLS; Giant cell variant characterized by GIANT CELLS in the tumors; and Fibrosarcomatous variant chracterized by tumor areas histologically indistinguishable from FIBROSARCOMA. [from MeSH]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDermatofibrosarcomas

Professional guidelines

PubMed

Kolk A, Wolff KD, Smeets R, Kesting M, Hein R, Eckert AW
Cancer Treat Rev 2014 Aug;40(7):819-37. Epub 2014 Apr 16 doi: 10.1016/j.ctrv.2014.04.002. PMID: 24814015

Recent clinical studies

Etiology

Bini A, Derka S, Stavrianos S
J Craniomaxillofac Surg 2024 Aug;52(8):877-883. Epub 2024 Jan 11 doi: 10.1016/j.jcms.2024.01.005. PMID: 38443189
Amadeo B, Penel N, Coindre JM, Ray-Coquard I, Plouvier S, Delafosse P, Bouvier AM, Gallet J, Lacourt A, Galvin A, Coureau G, Monnereau A, Blay JY, Desandes E, Mathoulin-Pélissier S; French Network of Cancer Registries (FRANCIM)
Cancer 2022 Jul 1;128(13):2483-2492. Epub 2022 Apr 6 doi: 10.1002/cncr.34217. PMID: 35385134
Molina AS, Duprat Neto JP, Bertolli E, da Cunha IW, Fregnani JHTG, Figueiredo PHM, Soares FA, Macedo MP, Pinto Lopes CA, de Abranches Oliveira Santos Filho ID
J Surg Oncol 2018 Apr;117(5):845-850. Epub 2018 Mar 6 doi: 10.1002/jso.25039. PMID: 29509956
Kolk A, Wolff KD, Smeets R, Kesting M, Hein R, Eckert AW
Cancer Treat Rev 2014 Aug;40(7):819-37. Epub 2014 Apr 16 doi: 10.1016/j.ctrv.2014.04.002. PMID: 24814015
Tsujimoto M, Aozasa K, Ueda T, Aoki Y, Orihara M, Mizushima T, Tsujimura T, Honda M
Jpn J Clin Oncol 1988 Jun;18(2):135-42. PMID: 3379783

Diagnosis

Lanic MD, Le Loarer F, Rainville V, Sater V, Viennot M, Beaussire L, Viailly PJ, Angot E, Hostein I, Jardin F, Ruminy P, Laé M
Mod Pathol 2022 May;35(5):649-663. Epub 2022 Jan 24 doi: 10.1038/s41379-021-00980-x. PMID: 35075283
Dadone-Montaudié B, Alberti L, Duc A, Delespaul L, Lesluyes T, Pérot G, Lançon A, Paindavoine S, Di Mauro I, Blay JY, de la Fouchardière A, Chibon F, Karanian M, MacGrogan G, Kubiniek V, Keslair F, Cardot-Leccia N, Michot A, Perrin V, Zekri Y, Coindre JM, Tirode F, Pedeutour F, Ranchère-Vince D, Le Loarer F, Pissaloux D
Mod Pathol 2018 Nov;31(11):1683-1693. Epub 2018 Jun 28 doi: 10.1038/s41379-018-0089-4. PMID: 29955147
Jung I, Gurzu S, Turdean S, Ciortea D, Sahlean DI, Golea M, Bara T
Int J Clin Exp Pathol 2015;8(2):1776-82. Epub 2015 Feb 1 PMID: 25973067Free PMC Article
Sartore L, Venezia ED, Della Puppa A, Bedogni A, Campana L, Giatsidis G
Head Neck 2015 Jan;37(1):E8-11. Epub 2014 Jul 21 doi: 10.1002/hed.23754. PMID: 24816728
Kolk A, Wolff KD, Smeets R, Kesting M, Hein R, Eckert AW
Cancer Treat Rev 2014 Aug;40(7):819-37. Epub 2014 Apr 16 doi: 10.1016/j.ctrv.2014.04.002. PMID: 24814015

Therapy

Johnson S, Renz M, Wheeler L, Diver E, Dorigo O, Litkouhi B, Behbakht K, Howitt B, Karam A
Int J Gynecol Cancer 2020 Aug;30(8):1118-1123. Epub 2020 Jul 7 doi: 10.1136/ijgc-2020-001516. PMID: 32641392
Mattox AK, Mehta AI, Grossi PM, Cummings TJ, Adamson DC
J Neurosurg 2010 May;112(5):965-77. doi: 10.3171/2009.7.JNS09522. PMID: 19681684
Potti A, Ganti AK, Tendulkar K, Sholes K, Chitajallu S, Koch M, Kargas S
J Cancer Res Clin Oncol 2004 Jan;130(1):52-6. Epub 2003 Nov 5 doi: 10.1007/s00432-003-0504-0. PMID: 14600832
Szadowska A, Olborski B, Harezga-Bal B, Debiec-Rychter M
Pol J Pathol 1999;50(1):9-16. PMID: 10412269

Prognosis

Bini A, Derka S, Stavrianos S
J Craniomaxillofac Surg 2024 Aug;52(8):877-883. Epub 2024 Jan 11 doi: 10.1016/j.jcms.2024.01.005. PMID: 38443189
Amadeo B, Penel N, Coindre JM, Ray-Coquard I, Plouvier S, Delafosse P, Bouvier AM, Gallet J, Lacourt A, Galvin A, Coureau G, Monnereau A, Blay JY, Desandes E, Mathoulin-Pélissier S; French Network of Cancer Registries (FRANCIM)
Cancer 2022 Jul 1;128(13):2483-2492. Epub 2022 Apr 6 doi: 10.1002/cncr.34217. PMID: 35385134
Johnson S, Renz M, Wheeler L, Diver E, Dorigo O, Litkouhi B, Behbakht K, Howitt B, Karam A
Int J Gynecol Cancer 2020 Aug;30(8):1118-1123. Epub 2020 Jul 7 doi: 10.1136/ijgc-2020-001516. PMID: 32641392
Molina AS, Duprat Neto JP, Bertolli E, da Cunha IW, Fregnani JHTG, Figueiredo PHM, Soares FA, Macedo MP, Pinto Lopes CA, de Abranches Oliveira Santos Filho ID
J Surg Oncol 2018 Apr;117(5):845-850. Epub 2018 Mar 6 doi: 10.1002/jso.25039. PMID: 29509956
Kolk A, Wolff KD, Smeets R, Kesting M, Hein R, Eckert AW
Cancer Treat Rev 2014 Aug;40(7):819-37. Epub 2014 Apr 16 doi: 10.1016/j.ctrv.2014.04.002. PMID: 24814015

Clinical prediction guides

Buffo TH, Stelini RF, Serrano JYM, Pontes LT, Magalhães RF, de Moraes AM
An Bras Dermatol 2023 Jan-Feb;98(1):36-46. Epub 2022 Nov 8 doi: 10.1016/j.abd.2022.01.009. PMID: 36369200Free PMC Article
Molina AS, Duprat Neto JP, Bertolli E, da Cunha IW, Fregnani JHTG, Figueiredo PHM, Soares FA, Macedo MP, Pinto Lopes CA, de Abranches Oliveira Santos Filho ID
J Surg Oncol 2018 Apr;117(5):845-850. Epub 2018 Mar 6 doi: 10.1002/jso.25039. PMID: 29509956
Jung I, Gurzu S, Turdean S, Ciortea D, Sahlean DI, Golea M, Bara T
Int J Clin Exp Pathol 2015;8(2):1776-82. Epub 2015 Feb 1 PMID: 25973067Free PMC Article
Hasegawa T, Matsuno Y, Shimoda T, Hirohashi S, Hirose T, Sano T
Jpn J Clin Oncol 1998 Feb;28(2):86-91. doi: 10.1093/jjco/28.2.86. PMID: 9544821
Rudolph P, Schubert B, Wacker HH, Parwaresch R, Schubert C
Am J Surg Pathol 1997 Jul;21(7):791-800. doi: 10.1097/00000478-199707000-00007. PMID: 9236835

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