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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

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLow grade fibromyxoid sarcoma

Professional guidelines

PubMed

Differential diagnosis of T2 hypointense masses in musculoskeletal MRI.
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
Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy.
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
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases.
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
Cytomorphologic findings of low-grade fibromyxoid sarcoma.
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
Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy.
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
Low-grade fibromyxoid sarcoma of acral sites: Case report and literature review.
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
Abdominopelvic and Retroperitoneal Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 13 Cases.
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

Diagnosis

Sclerosing Epithelioid Fibrosarcoma.
Warmke LM, Yu W, Meis JM
Surg Pathol Clin 2024 Mar;17(1):119-139. Epub 2023 Aug 5 doi: 10.1016/j.path.2023.06.009. PMID: 38278601
Superficial low-grade fibromyxoid sarcoma.
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
Sclerosing epithelioid fibrosarcoma.
Patterson JW, Tchernev G, Chokoeva AA, Wick MR
Wien Med Wochenschr 2017 Apr;167(5-6):120-123. Epub 2016 Sep 15 doi: 10.1007/s10354-016-0509-3. PMID: 27631873
Aggressive fibromatosis.
Fisher C, Thway K
Pathology 2014 Feb;46(2):135-40. doi: 10.1097/PAT.0000000000000045. PMID: 24378386
Low-grade fibromyxoid sarcoma.
Fukunaga M, Ushigome S, Fukunaga N
Virchows Arch 1996 Nov;429(4-5):301-3. doi: 10.1007/BF00198346. PMID: 8972766

Therapy

Low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, outcome of advanced disease: retrospective study from the Ultra-Rare Sarcoma Working Group.
Giani C, Denu RA, Ljevar S, Gronchi A, Napolitano A, Rosenbaum E, Salawu A, Bajpai J, Connolly EA, Lee ATJ, Trent JC, Koseła-Paterczyk H, Chia-Chen Li Z, Ogura K, Palmerini E, Baldi GG, Brunello A, Campos F, Cicala CM, Maki RG, Wagner AJ, Andelkovic V, Loong HH, Wong DD, Jones RL, Tap WD, Taverna SM, Lazar AJ, Demicco EG, Hong A, Bovee JVMG, Dei Tos AP, Fletcher CDM, Baumhoer D, Sbaraglia M, Schaefer IM, Miceli R, Stacchiotti S
ESMO Open 2024 Sep;9(9):103689. Epub 2024 Sep 11 doi: 10.1016/j.esmoop.2024.103689. PMID: 39265219Free PMC Article
A Case of Hypercalcemia from PTHrP-Producing Fibromyxoid Sarcoma Responsive to Glucocorticoid 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
Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy.
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
MRI and CT of Low-Grade Fibromyxoid Sarcoma in Children: A Report From Children's Oncology Group Study ARST0332.
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
Unusual histologic variant of a low-grade fibromyxoid sarcoma in a 3-year-old boy with complex chromosomal translocations involving 7q34, 10q11.2, and 16p11.2 and rearrangement of the FUS gene.
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

Superficial low-grade fibromyxoid sarcoma.
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
Cytomorphologic findings of low-grade fibromyxoid sarcoma.
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
Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy.
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
Abdominopelvic and Retroperitoneal Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 13 Cases.
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
A Rare Pathology: Low-Grade Fibromyxoid Sarcoma of the Maxilla.
Spalthoff S, Bredt M, Gellrich NC, Jehn P
J Oral Maxillofac Surg 2016 Jan;74(1):219.e1-10. Epub 2015 Sep 25 doi: 10.1016/j.joms.2015.09.018. PMID: 26455471

Clinical prediction guides

Differential diagnosis of T2 hypointense masses in musculoskeletal MRI.
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
MUC4 is expressed in alveolar rhabdomyosarcoma.
Forgó E, Hornick JL, Charville GW
Histopathology 2021 May;78(6):905-908. Epub 2021 Mar 16 doi: 10.1111/his.14321. PMID: 33368602Free PMC Article
Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature.
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
Low-grade fibromyxoid sarcoma: clinicopathologic case report with review of the literature.
Shidham VB, Ayala GE, Lahaniatis JE, Garcia FU
Am J Clin Oncol 1999 Apr;22(2):150-5. doi: 10.1097/00000421-199904000-00009. PMID: 10199449
Low-grade fibromyxoid sarcoma.
Fukunaga M, Ushigome S, Fukunaga N
Virchows Arch 1996 Nov;429(4-5):301-3. doi: 10.1007/BF00198346. PMID: 8972766

Recent systematic reviews

Supraclavicular Artery Island Flap for Treatment of Cervical Wound Defects and Persistent Cerebrospinal Fluid Leaks: A Technical Note and Systematic Review of the Literature.
Quiceno E, Soliman MAR, Khan A, Cavagnaro MJ, McSpadden RP, Pollina J, Levy EI, Mullin JP
World Neurosurg 2024 May;185:e915-e925. Epub 2024 Mar 6 doi: 10.1016/j.wneu.2024.02.151. PMID: 38458254
Low-Grade Fibromyxoid Sarcoma of the Distal Upper Extremity: A Systematic Review.
Kim M, Policherla RK, Linhares SM, Hui-Chou HG
Hand (N Y) 2024 Jul;19(5):701-708. Epub 2023 Jan 24 doi: 10.1177/15589447221150523. PMID: 36692100Free PMC Article

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

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