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Low grade fibromyxoid sarcoma

MedGen UID:
224814
Concept ID:
C1275282
Neoplastic Process
Synonyms: Low Grade Fibromyxoid Sarcoma; low grade fibromyxoid sarcoma; Low-grade fibromyxoid sarcoma; low-grade fibromyxoid sarcoma
SNOMED CT: Low-grade fibromyxoid sarcoma (404088004); Low grade fibromyxoid sarcoma (703617004)
 
Monarch Initiative: MONDO:0006272

Definition

A low-grade, late-metastasizing variant of fibrosarcoma characterized by alternating fibrous and myxoid areas and a whorling growth pattern. The neoplastic cells have a spindle morphology, and lack hyperchromasia or significant nuclear atypia. Approximately 40% of cases show the focal presence of collagen rosettes. A t(7;16)(q33;p11) translocation has been identified in the majority of cases, associated with the presence of FUS-CREB3L2 fusion protein. Rare cases carry the t(11;16)(p11;p11) translocation which is associated with the presence of the FUS-CREB3L1 fusion protein. [from NCI]

Term Hierarchy

Professional guidelines

PubMed

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
Chamberlain F, Engelmann B, Al-Muderis O, Messiou C, Thway K, Miah A, Zaidi S, Constantinidou A, Benson C, Gennatas S, Jones RL
In Vivo 2020 Jan-Feb;34(1):239-245. doi: 10.21873/invivo.11766. PMID: 31882484Free PMC Article
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Recent clinical studies

Etiology

Gjorgova Gjeorgjievski S, Fritchie K, Thangaiah JJ, Folpe AL, Din NU
Head Neck Pathol 2022 Jun;16(2):434-443. Epub 2021 Sep 24 doi: 10.1007/s12105-021-01380-y. PMID: 34559373Free PMC Article
Mustafa S, VandenBussche CJ, Ali SZ, Siddiqui MT, Wakely PE Jr
J Am Soc Cytopathol 2020 May-Jun;9(3):191-201. Epub 2020 Feb 22 doi: 10.1016/j.jasc.2020.01.006. PMID: 32197967
Chamberlain F, Engelmann B, Al-Muderis O, Messiou C, Thway K, Miah A, Zaidi S, Constantinidou A, Benson C, Gennatas S, Jones RL
In Vivo 2020 Jan-Feb;34(1):239-245. doi: 10.21873/invivo.11766. PMID: 31882484Free PMC Article
Scheer M, Vokuhl C, Veit-Friedrich I, Münter M, von Kalle T, Greulich M, Loff S, Stegmaier S, Sparber-Sauer M, Niggli F, Ladenstein R, Kazanowska B, Ljungman G, Jahnukainen K, Fuchs J, Bielack SS, Koscielniak E, Klingebiel T; Cooperative Weichteilsarkom Studiengruppe (CWS)
Pediatr Blood Cancer 2020 Feb;67(2):e28009. Epub 2019 Nov 17 doi: 10.1002/pbc.28009. PMID: 31736251
Saab-Chalhoub MW, Al-Rohil RN
J Cutan Pathol 2019 Apr;46(4):271-276. Epub 2019 Feb 7 doi: 10.1111/cup.13413. PMID: 30632203

Diagnosis

Ronen S, Ko JS, Rubin BP, Kilpatrick SE, Wang WL, Lazar AJ, Goldblum JR, Billings SD
J Cutan Pathol 2023 Feb;50(2):147-154. Epub 2022 Nov 2 doi: 10.1111/cup.14325. PMID: 36074249Free PMC Article
Mustafa S, VandenBussche CJ, Ali SZ, Siddiqui MT, Wakely PE Jr
J Am Soc Cytopathol 2020 May-Jun;9(3):191-201. Epub 2020 Feb 22 doi: 10.1016/j.jasc.2020.01.006. PMID: 32197967
Cowan ML, Thompson LD, Leon ME, Bishop JA
Head Neck Pathol 2016 Jun;10(2):161-6. Epub 2015 Aug 15 doi: 10.1007/s12105-015-0647-8. PMID: 26276044Free PMC Article
Fisher C, Thway K
Pathology 2014 Feb;46(2):135-40. doi: 10.1097/PAT.0000000000000045. PMID: 24378386
Fukunaga M, Ushigome S, Fukunaga N
Virchows Arch 1996 Nov;429(4-5):301-3. doi: 10.1007/BF00198346. PMID: 8972766

Therapy

Niu I, Hsiao EC, Wustrack R, Wysolmerski JJ, Dann P, Masharani U
Calcif Tissue Int 2023 Aug;113(2):246-253. Epub 2023 Jun 26 doi: 10.1007/s00223-023-01099-8. PMID: 37358786Free PMC Article
Bissonnette C, Shilo K, Liebner D, Rogers A, Pollock RE, Iwenofu OH
Int J Surg Pathol 2021 Feb;29(1):109-116. Epub 2020 Jun 7 doi: 10.1177/1066896920929081. PMID: 32506986
Chamberlain F, Engelmann B, Al-Muderis O, Messiou C, Thway K, Miah A, Zaidi S, Constantinidou A, Benson C, Gennatas S, Jones RL
In Vivo 2020 Jan-Feb;34(1):239-245. doi: 10.21873/invivo.11766. PMID: 31882484Free PMC Article
Sargar K, Kao SC, Spunt SL, Hawkins DS, Parham DM, Coffin C, McCarville MB
AJR Am J Roentgenol 2015 Aug;205(2):414-20. doi: 10.2214/AJR.14.13972. PMID: 26204295Free PMC Article
Dobin SM, Malone VS, Lopez L, Donner LR
Pediatr Dev Pathol 2013 Mar-Apr;16(2):86-90. Epub 2012 Oct 17 doi: 10.2350/12-07-1225-CR.1. PMID: 23075075

Prognosis

Ronen S, Ko JS, Rubin BP, Kilpatrick SE, Wang WL, Lazar AJ, Goldblum JR, Billings SD
J Cutan Pathol 2023 Feb;50(2):147-154. Epub 2022 Nov 2 doi: 10.1111/cup.14325. PMID: 36074249Free PMC Article
Mustafa S, VandenBussche CJ, Ali SZ, Siddiqui MT, Wakely PE Jr
J Am Soc Cytopathol 2020 May-Jun;9(3):191-201. Epub 2020 Feb 22 doi: 10.1016/j.jasc.2020.01.006. PMID: 32197967
Chamberlain F, Engelmann B, Al-Muderis O, Messiou C, Thway K, Miah A, Zaidi S, Constantinidou A, Benson C, Gennatas S, Jones RL
In Vivo 2020 Jan-Feb;34(1):239-245. doi: 10.21873/invivo.11766. PMID: 31882484Free PMC Article
Scheer M, Vokuhl C, Veit-Friedrich I, Münter M, von Kalle T, Greulich M, Loff S, Stegmaier S, Sparber-Sauer M, Niggli F, Ladenstein R, Kazanowska B, Ljungman G, Jahnukainen K, Fuchs J, Bielack SS, Koscielniak E, Klingebiel T; Cooperative Weichteilsarkom Studiengruppe (CWS)
Pediatr Blood Cancer 2020 Feb;67(2):e28009. Epub 2019 Nov 17 doi: 10.1002/pbc.28009. PMID: 31736251
Ud Din N, Ahmad Z, Zreik R, Horvai A, Folpe AL, Fritchie K
Am J Clin Pathol 2018 Jan 29;149(2):128-134. doi: 10.1093/ajcp/aqx137. PMID: 29385413

Clinical prediction guides

Serinelli S, Mookerjee GG, Stock H, de la Roza G, Damron T, Gitto L, Zaccarini DJ
Appl Immunohistochem Mol Morphol 2022 Oct 1;30(9):640-646. Epub 2022 Sep 19 doi: 10.1097/PAI.0000000000001059. PMID: 36121293
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
Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY
Chin Med J (Engl) 2018 Jan 5;131(1):32-36. doi: 10.4103/0366-6999.221274. PMID: 29271377Free PMC Article
Vallejo-Benítez A, Rodríguez-Zarco E, Carrasco SP, Pereira-Gallardo S, Brugal Molina J, García-Escudero A, Robles Frías A, Marcilla D, González-Cámpora R
Ann Diagn Pathol 2017 Oct;30:8-11. Epub 2017 May 11 doi: 10.1016/j.anndiagpath.2017.05.002. PMID: 28965630
Fukunaga M, Ushigome S, Fukunaga N
Virchows Arch 1996 Nov;429(4-5):301-3. doi: 10.1007/BF00198346. PMID: 8972766

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