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Sclerosing Epithelioid Fibrosarcoma

MedGen UID:
313689
•Concept ID:
C1710026
•
Neoplastic Process
Synonym: Sclerosing epithelioid fibrosarcoma
SNOMED CT: Sclerosing epithelioid fibrosarcoma (703618009); Sclerosing epithelioid fibrosarcoma (1290851003)

Definition

A well-circumscribed malignant fibroblastic neoplasm that usually arises from the soft tissue. It is characterized by the presence of nests of malignant epithelioid fibroblasts and sclerotic collagen stroma formation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSclerosing Epithelioid Fibrosarcoma

Professional guidelines

PubMed

Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I.
Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J
Cancer Treat Rev 2021 Sep;99:102259. Epub 2021 Jul 11 doi: 10.1016/j.ctrv.2021.102259. PMID: 34311246
In-depth Genetic Analysis of Sclerosing Epithelioid Fibrosarcoma Reveals Recurrent Genomic Alterations and Potential Treatment Targets.
Arbajian E, Puls F, Antonescu CR, Amary F, Sciot R, Debiec-Rychter M, Sumathi VP, Järås M, Magnusson L, Nilsson J, Hofvander J, Mertens F
Clin Cancer Res 2017 Dec 1;23(23):7426-7434. Epub 2017 Sep 22 doi: 10.1158/1078-0432.CCR-17-1856. PMID: 28939748
Sclerosing epithelioid fibrosarcoma: a study of five cases emphasizing diagnostic criteria.
Eyden BP, Manson C, Banerjee SS, Roberts IS, Harris M
Histopathology 1998 Oct;33(4):354-60. doi: 10.1046/j.1365-2559.1998.00530.x. PMID: 9822926

Recent clinical studies

Etiology

Sclerosing Epithelioid Fibrosarcoma (SEF) versus Low Grade Fibromyxoid Sarcoma (LGFMS): Presentation and outcome in the nationwide NETSARC+ series of 330 patients over 13 years.
Blay JY, Tlemsani C, Toulmonde M, Italiano A, Rios M, Bompas E, Valentin T, Duffaud F, Le Nail LR, Watson S, Firmin N, Dubray-Longeras P, Ropars M, Perrin C, Hervieu A, Lebbe C, Saada-Bouzid E, Soibinet P, Fiorenza F, Bertucci F, Boudou P, Vaz G, Bonvalot S, Honoré C, Marec-Berard P, Minard V, Cleirec M, Biau D, Meeus P, Babinet A, Dumaine V, Carriere S, Fau M, Decanter G, Gouin F, Ngo C, Le Loarer F, Karanian M, Meurgey A, Dufresne A, Brahmi M, Chemin-Airiau C, Ducimetiere F, Penel N, Le Cesne A; NETSARC/REPPS/RESOS and French Sarcoma Group- Groupe d′Etude des Tumeurs Osseuses (GSF-GETO) networks
Eur J Cancer 2024 Jan;196:113454. Epub 2023 Nov 23 doi: 10.1016/j.ejca.2023.113454. PMID: 38008029
Pediatric Abdominal Malignancies and Intravascular Extension: Contemporary Single-Center Experience.
Cairo SB, Urias AR, Murphy JT
J Surg Res 2022 Dec;280:396-403. Epub 2022 Aug 26 doi: 10.1016/j.jss.2022.06.046. PMID: 36037617
Sclerosing epithelioid fibrosarcoma: rare and serious.
Yahyaoui M, Benhammou M, Aharram S, Amghar J, Sadougui M, Agoumi O, Daoudi A
Pan Afr Med J 2020;36:131. Epub 2020 Jun 25 doi: 10.11604/pamj.2020.36.131.18668. PMID: 32849986Free PMC Article
What is new in epithelioid soft tissue tumors?
Agaimy A
Virchows Arch 2020 Jan;476(1):81-96. Epub 2019 Nov 4 doi: 10.1007/s00428-019-02677-8. PMID: 31686193
Clinical Characteristics and efficacy of chemotherapy in sclerosing epithelioid fibrosarcoma.
Chew W, Benson C, Thway K, Hayes A, Miah A, Zaidi S, Lee ATJ, Messiou C, Fisher C, van der Graaf WT, Jones RL
Med Oncol 2018 Sep 5;35(11):138. doi: 10.1007/s12032-018-1192-6. PMID: 30187231Free PMC Article

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
What is new in epithelioid soft tissue tumors?
Agaimy A
Virchows Arch 2020 Jan;476(1):81-96. Epub 2019 Nov 4 doi: 10.1007/s00428-019-02677-8. PMID: 31686193
Ear and Temporal Bone Pathology: Neural, Sclerosing and Myofibroblastic Lesions.
Flaman AN, Wasserman JK, Gravel DH, Purgina BM
Head Neck Pathol 2018 Sep;12(3):392-406. Epub 2018 Aug 1 doi: 10.1007/s12105-018-0891-9. PMID: 30069839Free 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
Fibrosarcoma: a review and update.
Folpe AL
Histopathology 2014 Jan;64(1):12-25. Epub 2013 Nov 22 doi: 10.1111/his.12282. PMID: 24266941

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
Examining Stripes on a Herd of Zebras: Impact of Genomic Matching for Ultrarare Sarcomas in Phase 1 Clinical Trials (SAMBA 102).
Moyers JT, Pestana RC, Roszik J, Hong DS, Naing A, Fu S, Piha-Paul S, Yap TA, Karp D, Rodon J, Livingston A, Zarzour MA, Ravi V, Patel S, Benjamin RS, Ludwig J, Herzog C, Ratan R, Somaiah N, Conley A, Gorlick R, Meric-Bernstam F, Subbiah V
Clin Cancer Res 2023 Jan 17;29(2):401-409. doi: 10.1158/1078-0432.CCR-22-2509. PMID: 36288393Free PMC Article
Clinical Benefit From Immune Checkpoint Blockade in Sclerosing Epithelioid Fibrosarcoma: A Translocation-Associated Sarcoma.
Doshi SD, Oza J, Remotti H, Remotti F, Moy MP, Schwartz GK, Ingham M
JCO Precis Oncol 2021 Nov;5:1-5. doi: 10.1200/PO.20.00201. PMID: 34994591
Clinical Characteristics and efficacy of chemotherapy in sclerosing epithelioid fibrosarcoma.
Chew W, Benson C, Thway K, Hayes A, Miah A, Zaidi S, Lee ATJ, Messiou C, Fisher C, van der Graaf WT, Jones RL
Med Oncol 2018 Sep 5;35(11):138. doi: 10.1007/s12032-018-1192-6. PMID: 30187231Free PMC Article
Sclerosing epithelioid fibrosarcoma of the pituitary.
Massier A, Scheithauer BW, Taylor HC, Clark C, Llerena L
Endocr Pathol 2007 Winter;18(4):233-8. doi: 10.1007/s12022-007-9010-2. PMID: 18097772

Prognosis

Sclerosing Epithelioid Fibrosarcoma (SEF) versus Low Grade Fibromyxoid Sarcoma (LGFMS): Presentation and outcome in the nationwide NETSARC+ series of 330 patients over 13 years.
Blay JY, Tlemsani C, Toulmonde M, Italiano A, Rios M, Bompas E, Valentin T, Duffaud F, Le Nail LR, Watson S, Firmin N, Dubray-Longeras P, Ropars M, Perrin C, Hervieu A, Lebbe C, Saada-Bouzid E, Soibinet P, Fiorenza F, Bertucci F, Boudou P, Vaz G, Bonvalot S, Honoré C, Marec-Berard P, Minard V, Cleirec M, Biau D, Meeus P, Babinet A, Dumaine V, Carriere S, Fau M, Decanter G, Gouin F, Ngo C, Le Loarer F, Karanian M, Meurgey A, Dufresne A, Brahmi M, Chemin-Airiau C, Ducimetiere F, Penel N, Le Cesne A; NETSARC/REPPS/RESOS and French Sarcoma Group- Groupe d′Etude des Tumeurs Osseuses (GSF-GETO) networks
Eur J Cancer 2024 Jan;196:113454. Epub 2023 Nov 23 doi: 10.1016/j.ejca.2023.113454. PMID: 38008029
Sclerosing epithelioid fibrosarcoma of bone: morphological, immunophenotypical, and molecular findings of 9 cases.
Kosemehmetoglu K, Ardic F, Kilpatrick SE, Aydingoz U, Sumathi VP, Michal M
Virchows Arch 2021 Apr;478(4):767-777. Epub 2020 Oct 21 doi: 10.1007/s00428-020-02953-y. PMID: 33084978
Sclerosing Epithelioid Fibrosarcoma: A Distinct Sarcoma With Aggressive Features.
Warmke LM, Meis JM
Am J Surg Pathol 2021 Mar 1;45(3):317-328. doi: 10.1097/PAS.0000000000001559. PMID: 32769431
Sclerosing epithelioid fibrosarcoma: rare and serious.
Yahyaoui M, Benhammou M, Aharram S, Amghar J, Sadougui M, Agoumi O, Daoudi A
Pan Afr Med J 2020;36:131. Epub 2020 Jun 25 doi: 10.11604/pamj.2020.36.131.18668. PMID: 32849986Free PMC Article
Clinical Characteristics and efficacy of chemotherapy in sclerosing epithelioid fibrosarcoma.
Chew W, Benson C, Thway K, Hayes A, Miah A, Zaidi S, Lee ATJ, Messiou C, Fisher C, van der Graaf WT, Jones RL
Med Oncol 2018 Sep 5;35(11):138. doi: 10.1007/s12032-018-1192-6. PMID: 30187231Free PMC Article

Clinical prediction guides

Sclerosing Epithelioid Fibrosarcoma (SEF) versus Low Grade Fibromyxoid Sarcoma (LGFMS): Presentation and outcome in the nationwide NETSARC+ series of 330 patients over 13 years.
Blay JY, Tlemsani C, Toulmonde M, Italiano A, Rios M, Bompas E, Valentin T, Duffaud F, Le Nail LR, Watson S, Firmin N, Dubray-Longeras P, Ropars M, Perrin C, Hervieu A, Lebbe C, Saada-Bouzid E, Soibinet P, Fiorenza F, Bertucci F, Boudou P, Vaz G, Bonvalot S, Honoré C, Marec-Berard P, Minard V, Cleirec M, Biau D, Meeus P, Babinet A, Dumaine V, Carriere S, Fau M, Decanter G, Gouin F, Ngo C, Le Loarer F, Karanian M, Meurgey A, Dufresne A, Brahmi M, Chemin-Airiau C, Ducimetiere F, Penel N, Le Cesne A; NETSARC/REPPS/RESOS and French Sarcoma Group- Groupe d′Etude des Tumeurs Osseuses (GSF-GETO) networks
Eur J Cancer 2024 Jan;196:113454. Epub 2023 Nov 23 doi: 10.1016/j.ejca.2023.113454. PMID: 38008029
Metastatic pediatric sclerosing epithelioid fibrosarcoma.
Woods AD, Purohit R, Mitchell LC, Collier JR, Collier KA, Lathara M, Learned K, Vaske O, Geiger H, Wrzeszczynski KO, Jobanputra V, Srinivasa G, Rudzinski ER, Whelan K, Beierle E, Spunt SL, Keller C, Wadhwa A
Cold Spring Harb Mol Case Stud 2021 Oct;7(5) Epub 2021 Oct 19 doi: 10.1101/mcs.a006093. PMID: 34362827Free PMC Article
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
Primary sclerosing epithelioid fibrosarcoma of bone: analysis of a series.
Wojcik JB, Bellizzi AM, Dal Cin P, Bredella MA, Fletcher CD, Hornicek FJ, Deshpande V, Hornick JL, Nielsen GP
Am J Surg Pathol 2014 Nov;38(11):1538-44. doi: 10.1097/PAS.0000000000000265. PMID: 24921641
Selected topics in the pathology of epithelioid soft tissue tumors.
Folpe AL
Mod Pathol 2014 Jan;27 Suppl 1:S64-79. doi: 10.1038/modpathol.2013.175. PMID: 24384854

Recent systematic reviews

Sclerosing epithelioid fibrosarcoma: case presentation and a systematic review.
Ossendorf C, Studer GM, Bode B, Fuchs B
Clin Orthop Relat Res 2008 Jun;466(6):1485-91. Epub 2008 Mar 14 doi: 10.1007/s11999-008-0205-8. PMID: 18340502Free PMC Article

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