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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
Zhao YX, Hu XY, Zhong X, Shen H, Yuan Y
Dermatol Ther 2021 Mar;34(2):e14816. Epub 2021 Feb 4 doi: 10.1111/dth.14816. PMID: 33497505
Riouallon G, Larousserie F, Pluot E, Anract P
Orthop Traumatol Surg Res 2013 Jun;99(4):473-7. Epub 2013 Apr 18 doi: 10.1016/j.otsr.2012.11.020. PMID: 23602311

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
Wakely PE Jr
J Am Soc Cytopathol 2021 May-Jun;10(3):300-309. Epub 2020 Sep 19 doi: 10.1016/j.jasc.2020.09.004. PMID: 33041221
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
Tjarks BJ, Ko JS, Billings SD
J Cutan Pathol 2018 Feb;45(2):104-110. Epub 2017 Dec 5 doi: 10.1111/cup.13063. PMID: 29065228
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

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
Li H, Wang H, Wang D
Braz J Otorhinolaryngol 2022 Nov-Dec;88 Suppl 5(Suppl 5):S171-S178. Epub 2022 Feb 15 doi: 10.1016/j.bjorl.2022.02.001. PMID: 35256287Free 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
Clarke LE
Dermatol Clin 2012 Oct;30(4):643-56, vi. Epub 2012 Aug 11 doi: 10.1016/j.det.2012.06.005. PMID: 23021051
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

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
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694
Clarke LE
Dermatol Clin 2012 Oct;30(4):643-56, vi. Epub 2012 Aug 11 doi: 10.1016/j.det.2012.06.005. PMID: 23021051
Mentzel T
Clin Dermatol 2011 Jan-Feb;29(1):80-90. doi: 10.1016/j.clindermatol.2010.07.011. PMID: 21146736
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

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
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
Renn A, Adejolu M, Messiou C, Bhaludin B, Strauss DC, Thway K, Moskovic E
Clin Radiol 2021 Dec;76(12):940.e1-940.e16. Epub 2021 Oct 1 doi: 10.1016/j.crad.2021.08.011. PMID: 34607656
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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