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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: 35715142Free PMC Article
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
Widemann BC, Italiano A
J Clin Oncol 2018 Jan 10;36(2):160-167. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.75.3467. PMID: 29220302Free PMC Article

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: 35715142Free 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
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
Carvalho SD, Pissaloux D, Crombé A, Coindre JM, Le Loarer F
Surg Pathol Clin 2019 Mar;12(1):63-105. Epub 2018 Dec 17 doi: 10.1016/j.path.2018.10.004. PMID: 30709449

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: 35715142Free PMC Article
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: [email protected]; 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
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
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
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
Carvalho SD, Pissaloux D, Crombé A, Coindre JM, Le Loarer F
Surg Pathol Clin 2019 Mar;12(1):63-105. Epub 2018 Dec 17 doi: 10.1016/j.path.2018.10.004. PMID: 30709449
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

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: 35715142Free 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
Dell'Aversana Orabona G, Iaconetta G, Abbate V, Piombino P, Romano A, Maglitto F, Salzano G, Califano L
J Med Case Rep 2014 Dec 29;8:468. doi: 10.1186/1752-1947-8-468. PMID: 25547541Free PMC Article
Brenn T
Adv Anat Pathol 2014 Mar;21(2):108-30. doi: 10.1097/PAP.0000000000000009. PMID: 24508694

Recent systematic reviews

Tibbo ME, Landau R, Markowitz MI, Abuodeh Y, Thomas Temple H, Crawford B
J Surg Oncol 2024 Sep;130(3):586-593. Epub 2024 Aug 19 doi: 10.1002/jso.27782. PMID: 39155707
Gusho C, Phillips R, Cook J, Evenski A
Iowa Orthop J 2023 Dec;43(2):52-59. PMID: 38213851Free PMC Article

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