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Localized Fibrosarcoma

MedGen UID:
208946
•Concept ID:
C0855055
•
Neoplastic Process
Synonyms: Non-Metastatic Fibrosarcoma; Primary Fibrosarcoma

Definition

Fibrosarcoma that is confined to a specific site without evidence of spread to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocalized Fibrosarcoma

Professional guidelines

PubMed

Surgical management of dermatofibrosarcoma protuberans.
Rust DJ, Kwinta BD, Geskin LJ, Samie FH, Remotti F, Yoon SS
J Surg Oncol 2023 Jul;128(1):87-96. Epub 2023 Mar 31 doi: 10.1002/jso.27258. PMID: 36999599
Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options.
Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
Management of dermatofibrosarcoma protuberans.
Badhey AK, Tikhtman R, Tang AL
Curr Opin Otolaryngol Head Neck Surg 2021 Aug 1;29(4):278-282. doi: 10.1097/MOO.0000000000000721. PMID: 33993132

Recent clinical studies

Etiology

Myxoinflammatory Fibroblastic Sarcoma.
Wu H, Laskin WB
Surg Pathol Clin 2024 Mar;17(1):83-96. Epub 2023 Aug 8 doi: 10.1016/j.path.2023.07.003. PMID: 38278610
Dermatofibrosarcoma Protuberans in Children.
Sleiwah A, Wright TC, Chapman T, Dangoor A, Maggiani F, Clancy R
Curr Treat Options Oncol 2022 Jun;23(6):843-854. Epub 2022 Apr 8 doi: 10.1007/s11864-022-00979-9. PMID: 35394606
Dermatofibrosarcoma Protuberans.
Allen A, Ahn C, Sangüeza OP
Dermatol Clin 2019 Oct;37(4):483-488. doi: 10.1016/j.det.2019.05.006. PMID: 31466588
Non-rhabdomyosarcoma.
Dasgupta R, Rodeberg D
Semin Pediatr Surg 2016 Oct;25(5):284-289. Epub 2016 Sep 22 doi: 10.1053/j.sempedsurg.2016.09.012. PMID: 27955731
Dermatofibrosarcoma protuberans.
Mendenhall WM, Zlotecki RA, Scarborough MT
Cancer 2004 Dec 1;101(11):2503-8. doi: 10.1002/cncr.20678. PMID: 15503305

Diagnosis

Dermatofibrosarcoma Protuberans: What Is This?
Vitiello GA, Lee AY, Berman RS
Surg Clin North Am 2022 Aug;102(4):657-665. doi: 10.1016/j.suc.2022.05.004. PMID: 35952694
Management of dermatofibrosarcoma protuberans.
Badhey AK, Tikhtman R, Tang AL
Curr Opin Otolaryngol Head Neck Surg 2021 Aug 1;29(4):278-282. doi: 10.1097/MOO.0000000000000721. PMID: 33993132
Myxofibrosarcoma.
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
Myofibroblastic malignancies.
Fisher C
Adv Anat Pathol 2004 Jul;11(4):190-201. doi: 10.1097/01.pap.0000131773.16130.aa. PMID: 15220822
Myofibrosarcoma.
Fisher C
Virchows Arch 2004 Sep;445(3):215-23. Epub 2004 Jun 2 doi: 10.1007/s00428-004-1038-9. PMID: 15173943

Therapy

Intratumoral combination therapy with poly(I:C) and resiquimod synergistically triggers tumor-associated macrophages for effective systemic antitumoral immunity.
Anfray C, Mainini F, Digifico E, Maeda A, Sironi M, Erreni M, Anselmo A, Ummarino A, Gandoy S, Expósito F, Redrado M, Serrano D, Calvo A, Martens M, Bravo S, Mantovani A, Allavena P, Andón FT
J Immunother Cancer 2021 Sep;9(9) doi: 10.1136/jitc-2021-002408. PMID: 34531246Free PMC Article
Dermatofibrosarcoma Protuberans.
Allen A, Ahn C, Sangüeza OP
Dermatol Clin 2019 Oct;37(4):483-488. doi: 10.1016/j.det.2019.05.006. PMID: 31466588
Larotrectinib for paediatric solid tumours harbouring NTRK gene fusions: phase 1 results from a multicentre, open-label, phase 1/2 study.
Laetsch TW, DuBois SG, Mascarenhas L, Turpin B, Federman N, Albert CM, Nagasubramanian R, Davis JL, Rudzinski E, Feraco AM, Tuch BB, Ebata KT, Reynolds M, Smith S, Cruickshank S, Cox MC, Pappo AS, Hawkins DS
Lancet Oncol 2018 May;19(5):705-714. Epub 2018 Mar 29 doi: 10.1016/S1470-2045(18)30119-0. PMID: 29606586Free PMC Article
Dermatofibrosarcoma Protuberans.
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Non-rhabdomyosarcoma.
Dasgupta R, Rodeberg D
Semin Pediatr Surg 2016 Oct;25(5):284-289. Epub 2016 Sep 22 doi: 10.1053/j.sempedsurg.2016.09.012. PMID: 27955731

Prognosis

Dermatofibrosarcoma Protuberans.
Acosta AE, Vélez CS
Curr Treat Options Oncol 2017 Aug 10;18(9):56. doi: 10.1007/s11864-017-0498-5. PMID: 28795284
Dermatofibrosarcoma protuberans.
Mendenhall WM, Zlotecki RA, Scarborough MT
Cancer 2004 Dec 1;101(11):2503-8. doi: 10.1002/cncr.20678. PMID: 15503305
Pediatric neurofibrosarcoma.
Neville H, Corpron C, Blakely ML, Andrassy R
J Pediatr Surg 2003 Mar;38(3):343-6; discussion 343-6. doi: 10.1053/jpsu.2003.50105. PMID: 12632346
Malignant fibrous tumors.
Stadler FJ, Scott GA, Brown MD
Semin Cutan Med Surg 1998 Jun;17(2):141-52. doi: 10.1016/s1085-5629(98)80007-2. PMID: 9669607
Dermatofibrosarcoma protuberans.
Gloster HM Jr
J Am Acad Dermatol 1996 Sep;35(3 Pt 1):355-74; quiz 375-6. doi: 10.1016/s0190-9622(96)90597-6. PMID: 8784271

Clinical prediction guides

Fibroblastic and Myofibroblastic Soft-Tissue Tumors: Imaging Spectrum and Radiologic-Pathologic Correlation.
Haseli S, Mansoori B, Christensen D, Abadi A, Pooyan A, Shomal Zadeh F, Mau B, Khalili N, Murphey M, Chalian M
Radiographics 2023 Aug;43(8):e230005. doi: 10.1148/rg.230005. PMID: 37440448
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
The Management of Head and Neck Sarcoma.
Han S, Yin X, Xu W, Wang Y, Han W
J Craniofac Surg 2020 Mar/Apr;31(2):e189-e192. doi: 10.1097/SCS.0000000000006162. PMID: 31934973
Larotrectinib for paediatric solid tumours harbouring NTRK gene fusions: phase 1 results from a multicentre, open-label, phase 1/2 study.
Laetsch TW, DuBois SG, Mascarenhas L, Turpin B, Federman N, Albert CM, Nagasubramanian R, Davis JL, Rudzinski E, Feraco AM, Tuch BB, Ebata KT, Reynolds M, Smith S, Cruickshank S, Cox MC, Pappo AS, Hawkins DS
Lancet Oncol 2018 May;19(5):705-714. Epub 2018 Mar 29 doi: 10.1016/S1470-2045(18)30119-0. PMID: 29606586Free PMC Article
Fibromatosis of the oral and paraoral region.
Fowler CB, Hartman KS, Brannon RB
Oral Surg Oral Med Oral Pathol 1994 Apr;77(4):373-86. doi: 10.1016/0030-4220(94)90200-3. PMID: 8015802

Recent systematic reviews

Dermatofibrosarcoma protuberans of the scalp: Surgical management in a multicentric series of 11 cases and systematic review of the literature.
Kuhlmann C, Ehrl D, Taha S, Wachtel N, Schmid A, Bronsert P, Zeller J, Giunta RE, Eisenhardt SU, Braig D
Surgery 2023 Jun;173(6):1463-1475. Epub 2023 Apr 1 doi: 10.1016/j.surg.2023.02.026. PMID: 37012145
Primary central nervous system sarcomas in adults: A systematic review.
Haider AS, Palmisciano P, Sagoo NS, Bin Alamer O, El Ahmadieh TY, Pan E, Garzon-Muvdi T
Clin Neurol Neurosurg 2022 Mar;214:107127. Epub 2022 Jan 12 doi: 10.1016/j.clineuro.2022.107127. PMID: 35151057
Sarcomas of the sellar region: a systematic review.
Guerrero-Pérez F, Vidal N, López-Vázquez M, Sánchez-Barrera R, Sánchez-Fernández JJ, Torres-Díaz A, Vilarrasa N, Villabona C
Pituitary 2021 Feb;24(1):117-129. doi: 10.1007/s11102-020-01073-9. PMID: 32785833
Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis.
Cai Z, Tang X, Liang H, Yang R, Yan T, Guo W
World J Surg Oncol 2020 Sep 30;18(1):257. doi: 10.1186/s12957-020-02036-x. PMID: 32998743Free PMC Article
Primary Thyroid Sarcoma: A Systematic Review.
Surov A, Gottschling S, Wienke A, Meyer HJ, Spielmann RP, Dralle H
Anticancer Res 2015 Oct;35(10):5185-91. PMID: 26408676

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