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Myxofibrosarcoma

MedGen UID:
811359
Concept ID:
C3714524
Neoplastic Process
Synonyms: Fibromyxosarcoma; fibromyxosarcoma; MFS; myxofibrosarcoma; myxoid fibrous histiocytoma; Myxoid malignant fibrous histiocytoma; myxoid malignant fibrous histiocytoma; myxoid MFH
SNOMED CT: Myxofibrosarcoma (703609007); Myxoid malignant fibrous histiocytoma (703609007); Fibromyxosarcoma (703609007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019202
Orphanet: ORPHA79105

Definition

A rare soft tissue sarcoma characterized by a malignant, fibroblastic lesion with variably myxoid stroma, pleomorphism, and a distinctively curvilinear vascular pattern. The majority of tumors arise in the limbs including the limb girdles, more often in dermal/subcutaneous tissues than in the underlying fascia and skeletal muscle, and usually present as a slowly growing, painless mass. Depth of the lesion and tumor grade do not influence the high rate of local recurrence, while the percentage of metastasis and tumor-associated mortality are much higher in deep-seated and high-grade neoplasms. [from ORDO]

Professional guidelines

PubMed

Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Radaelli S, Pasquali S, Colombo C, Callegaro D, Sanfilippo R, Stacchiotti S, Provenzano S, Sangalli C, Morosi C, Barisella M, Miceli R, Fiore M, Gronchi A
Eur J Surg Oncol 2022 Aug;48(8):1723-1729. Epub 2022 Jan 21 doi: 10.1016/j.ejso.2022.01.003. PMID: 35153104
Finkelstein D, Foremny G, Singer A, Clifford P, Pretell-Mazzini J, Kerr DA, Subhawong TK
Skeletal Radiol 2021 Oct;50(10):1981-1994. Epub 2021 Mar 2 doi: 10.1007/s00256-021-03711-0. PMID: 33651128

Recent clinical studies

Etiology

Erdogan O, Çeli K A, Yildirim ANT, Tekçe E, Altun G, Demi Röz S, Güler Y, Ozkan K, Gurkan V
Acta Chir Orthop Traumatol Cech 2023;90(3):181-187. PMID: 37395425
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Díaz-Flores L, Gutiérrez R, García MP, González-Gómez M, Rodríguez-Rodriguez R, Hernández-León N, Díaz-Flores L Jr, Carrasco JL
Int J Mol Sci 2021 Jul 8;22(14) doi: 10.3390/ijms22147342. PMID: 34298962Free PMC Article
Nakata E, Fujiwara T, Kunisada T, Ito T, Takihira S, Ozaki T
Jpn J Clin Oncol 2021 Apr 1;51(4):523-537. doi: 10.1093/jjco/hyab005. PMID: 33611603
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082

Diagnosis

Erdogan O, Çeli K A, Yildirim ANT, Tekçe E, Altun G, Demi Röz S, Güler Y, Ozkan K, Gurkan V
Acta Chir Orthop Traumatol Cech 2023;90(3):181-187. PMID: 37395425
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Morag Y, Lucas DR
Skeletal Radiol 2022 Apr;51(4):691-700. Epub 2021 Jul 22 doi: 10.1007/s00256-021-03869-7. PMID: 34292352
Cancer Genome Atlas Research Network. Electronic address: elizabeth.demicco@sinaihealthsystem.ca; Cancer Genome Atlas Research Network
Cell 2017 Nov 2;171(4):950-965.e28. doi: 10.1016/j.cell.2017.10.014. PMID: 29100075Free PMC Article
Roland CL, Wang WL, Lazar AJ, Torres KE
Surg Oncol Clin N Am 2016 Oct;25(4):775-88. Epub 2016 Jul 30 doi: 10.1016/j.soc.2016.05.008. PMID: 27591498

Therapy

Wang D, Harris J, Kraybill WG, Eisenberg B, Kirsch DG, Ettinger DS, Kane JM 3rd, Barry PN, Naghavi A, Freeman CR, Chen YL, Hitchcock YJ, Bedi M, Salerno KE, Severin D, Godette KD, Larrier NA, Curran WJ Jr, Torres-Saavedra PA, Lucas DR
JAMA Oncol 2023 May 1;9(5):646-655. doi: 10.1001/jamaoncol.2023.0042. PMID: 36995690Free PMC Article
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Meric-Bernstam F, Sweis RF, Hodi FS, Messersmith WA, Andtbacka RHI, Ingham M, Lewis N, Chen X, Pelletier M, Chen X, Wu J, McWhirter SM, Müller T, Nair N, Luke JJ
Clin Cancer Res 2022 Feb 15;28(4):677-688. doi: 10.1158/1078-0432.CCR-21-1963. PMID: 34716197
Kenan S, Liang H, Goodman HJ, Jacobs AJ, Chan A, Grande DA, Levin AS
J Orthop Surg Res 2020 Mar 5;15(1):94. doi: 10.1186/s13018-020-01606-9. PMID: 32138774Free PMC Article
Udaka T, Yamamoto H, Shiomori T, Fujimura T, Suzuki H
J Laryngol Otol 2006 Oct;120(10):872-4. doi: 10.1017/S0022215106001113. PMID: 17038234

Prognosis

Haseli S, Mansoori B, Christensen D, Abadi A, Pooyan A, Shomal Zadeh F, Mau B, Khalili N, Murphey M, Chalian M
Radiographics 2023 Aug;43(8):e230005. doi: 10.1148/rg.230005. PMID: 37440448
Erdogan O, Çeli K A, Yildirim ANT, Tekçe E, Altun G, Demi Röz S, Güler Y, Ozkan K, Gurkan V
Acta Chir Orthop Traumatol Cech 2023;90(3):181-187. PMID: 37395425
Nakata E, Fujiwara T, Kunisada T, Ito T, Takihira S, Ozaki T
Jpn J Clin Oncol 2021 Apr 1;51(4):523-537. doi: 10.1093/jjco/hyab005. PMID: 33611603
Sambri A, De Paolis M, Spinnato P, Donati DM, Bianchi G
Oncol Res Treat 2020;43(6):314-322. Epub 2020 May 25 doi: 10.1159/000507334. PMID: 32450554
Mentzel T
Clin Dermatol 2011 Jan-Feb;29(1):80-90. doi: 10.1016/j.clindermatol.2010.07.011. PMID: 21146736

Clinical prediction guides

Erdogan O, Çeli K A, Yildirim ANT, Tekçe E, Altun G, Demi Röz S, Güler Y, Ozkan K, Gurkan V
Acta Chir Orthop Traumatol Cech 2023;90(3):181-187. PMID: 37395425
Kelly CM, Qin LX, Whiting KA, Richards AL, Avutu V, Chan JE, Chi P, Dickson MA, Gounder MM, Keohan ML, Movva S, Nacev BA, Rosenbaum E, Adamson T, Singer S, Bartlett EK, Crago AM, Yoon SS, Hwang S, Erinjeri JP, Antonescu CR, Tap WD, D'Angelo SP
Clin Cancer Res 2023 Jun 1;29(11):2043-2051. doi: 10.1158/1078-0432.CCR-22-3911. PMID: 36971773Free PMC Article
Crago AM, Cardona K, Koseła-Paterczyk H, Rutkowski P
Surg Oncol Clin N Am 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006. PMID: 35715142
Nakata E, Fujiwara T, Kunisada T, Ito T, Takihira S, Ozaki T
Jpn J Clin Oncol 2021 Apr 1;51(4):523-537. doi: 10.1093/jjco/hyab005. PMID: 33611603
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694

Recent systematic reviews

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