U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Locally Advanced Fibrosarcoma

MedGen UID:
1729403
•Concept ID:
C5419080
•
Neoplastic Process

Definition

Fibrosarcoma that has spread from its original site of growth to nearby tissues or lymph nodes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocally Advanced 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
Imatinib Treatment for Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans: A Systematic Review.
Navarrete-Dechent C, Mori S, Barker CA, Dickson MA, Nehal KS
JAMA Dermatol 2019 Mar 1;155(3):361-369. doi: 10.1001/jamadermatol.2018.4940. PMID: 30601909Free PMC Article
Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans.
Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Través V, Sanmartín O
Actas Dermosifiliogr (Engl Ed) 2018 Dec;109(10):868-877. Epub 2018 Jul 4 doi: 10.1016/j.ad.2018.05.006. PMID: 30539729

Recent clinical studies

Etiology

Dermatofibrosarcoma Protuberans.
Reha J, Katz SC
Surg Clin North Am 2016 Oct;96(5):1031-46. doi: 10.1016/j.suc.2016.05.006. PMID: 27542641
The evolution of systemic therapy in sarcoma.
Constantinidou A, Pollack S, Loggers E, Rodler E, Jones RL
Expert Rev Anticancer Ther 2013 Feb;13(2):211-23. doi: 10.1586/era.12.161. PMID: 23406562
Advances in management of dermatofibrosarcoma protuberans.
Johnson-Jahangir H, Ratner D
Dermatol Clin 2011 Apr;29(2):191-200, viii. doi: 10.1016/j.det.2011.02.003. PMID: 21421145
Neoadjuvant imatinib therapy for dermatofibrosarcoma protuberans.
Han A, Chen EH, Niedt G, Sherman W, Ratner D
Arch Dermatol 2009 Jul;145(7):792-6. doi: 10.1001/archdermatol.2009.140. PMID: 19620561
Advances in the adjuvant treatment of infantile fibrosarcoma.
Ferguson WS
Expert Rev Anticancer Ther 2003 Apr;3(2):185-91. doi: 10.1586/14737140.3.2.185. PMID: 12722878

Diagnosis

What is new in fibroblastic/myofibroblastic tumors in children.
Al-Ibraheemi A, Zhou Y, Rullo E, Alaggio R
Virchows Arch 2025 Jan;486(1):127-141. Epub 2024 Nov 5 doi: 10.1007/s00428-024-03964-9. PMID: 39499317
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
The landscape of genetic aberrations in myxofibrosarcoma.
Takeuchi Y, Yoshida K, Halik A, Kunitz A, Suzuki H, Kakiuchi N, Shiozawa Y, Yokoyama A, Inoue Y, Hirano T, Yoshizato T, Aoki K, Fujii Y, Nannya Y, Makishima H, Pfitzner BM, Bullinger L, Hirata M, Jinnouchi K, Shiraishi Y, Chiba K, Tanaka H, Miyano S, Okamoto T, Haga H, Ogawa S, Damm F
Int J Cancer 2022 Aug 15;151(4):565-577. Epub 2022 May 13 doi: 10.1002/ijc.34051. PMID: 35484982
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.
Reha J, Katz SC
Surg Clin North Am 2016 Oct;96(5):1031-46. doi: 10.1016/j.suc.2016.05.006. PMID: 27542641

Therapy

Dermatofibrosarcoma Protuberans Of Trunk In A Male Patient.
Hashmi JZ, Ghafoor A, Rauf Khan Hiraj MU, Chaudhary WA
J Ayub Med Coll Abbottabad 2022 Apr-Jun;34(2):364-365. doi: 10.55519/JAMC-02-8960. PMID: 35576304
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
The evolution of systemic therapy in sarcoma.
Constantinidou A, Pollack S, Loggers E, Rodler E, Jones RL
Expert Rev Anticancer Ther 2013 Feb;13(2):211-23. doi: 10.1586/era.12.161. PMID: 23406562
Molecular targeting of dermatofibrosarcoma protuberans: a new approach to a surgical disease.
McArthur GA
J Natl Compr Canc Netw 2007 May;5(5):557-62. doi: 10.6004/jnccn.2007.0049. PMID: 17509258

Prognosis

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
A clinicopathologic review of a case series of dermatofibrosarcoma protuberans with fibrosarcomatous differentiation.
Kuzel P, Mahmood MN, Metelitsa AI, Salopek TG
J Cutan Med Surg 2015 Jan-Feb;19(1):28-34. Epub 2015 Jan 1 doi: 10.2310/7750.2014.13192. PMID: 25775660
Dermatofibrosarcoma protuberans: a review of the literature.
Bogucki B, Neuhaus I, Hurst EA
Dermatol Surg 2012 Apr;38(4):537-51. Epub 2012 Jan 30 doi: 10.1111/j.1524-4725.2011.02292.x. PMID: 22288484
Advances in management of dermatofibrosarcoma protuberans.
Johnson-Jahangir H, Ratner D
Dermatol Clin 2011 Apr;29(2):191-200, viii. doi: 10.1016/j.det.2011.02.003. PMID: 21421145

Clinical prediction guides

Therapeutic benefit of larotrectinib over the historical standard of care in patients with locally advanced or metastatic infantile fibrosarcoma (EPI VITRAKVI study).
Orbach D, Carton M, Khadir SK, Feuilly M, Kurtinecz M, Phil D, Vokuhl C, Koscielniak E, Pierron G, Lemelle L, Sparber-Sauer M
ESMO Open 2024 May;9(5):103006. Epub 2024 Apr 23 doi: 10.1016/j.esmoop.2024.103006. PMID: 38657345Free PMC Article
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
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
Neoadjuvant imatinib therapy for dermatofibrosarcoma protuberans.
Han A, Chen EH, Niedt G, Sherman W, Ratner D
Arch Dermatol 2009 Jul;145(7):792-6. doi: 10.1001/archdermatol.2009.140. PMID: 19620561
The role of cytogenetics in the classification of soft tissue tumours.
Dei Tos AP, Dal Cin P
Virchows Arch 1997 Aug;431(2):83-94. doi: 10.1007/s004280050073. PMID: 9293889

Recent systematic reviews

Disease-Specific Mortality of Dermatofibrosarcoma Protuberans After Mohs Surgery Versus Wide Local Excision: A Systematic Review and Meta-Analysis.
Crum OM, O'Hern K, Demer AM, Brewer JD
Dermatol Surg 2024 Apr 1;50(4):317-321. Epub 2024 Feb 8 doi: 10.1097/DSS.0000000000004088. PMID: 38335454
Impact of CT and MRI in the diagnostic workup of malignant triton tumour-a monocentric analysis and review of the literature.
Drews MA, Milosevic A, Hamacher R, Grüneisen JS, Haubold J, Opitz MK, Bauer S, Umutlu L, Forsting M, Schaarschmidt BM
Br J Radiol 2024 Feb 2;97(1154):430-438. doi: 10.1093/bjr/tqad035. PMID: 38308031
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
Imatinib Treatment for Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans: A Systematic Review.
Navarrete-Dechent C, Mori S, Barker CA, Dickson MA, Nehal KS
JAMA Dermatol 2019 Mar 1;155(3):361-369. doi: 10.1001/jamadermatol.2018.4940. PMID: 30601909Free PMC Article

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...