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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
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
Current treatment options for dermatofibrosarcoma protuberans.
Rutkowski P, Debiec-Rychter M
Expert Rev Anticancer Ther 2015;15(8):901-9. Epub 2015 May 31 doi: 10.1586/14737140.2015.1052799. PMID: 26027711

Recent clinical studies

Etiology

Intracranial Relapse in Pediatric Sarcoma.
Smith DE, Hamby T, Heym K, Mohamed A, Vallance KL, Ray A
J Pediatr Hematol Oncol 2023 Oct 1;45(7):e810-e816. Epub 2023 Jul 14 doi: 10.1097/MPH.0000000000002713. PMID: 37526369
Dermatofibrosarcoma Protuberans: One Centre Experience.
Mareş T, Răducu L, Avino A, Gheoca-Mutu DE, Teodoreanu RN, Jecan CR
Chirurgia (Bucur) 2022 Oct;117(5):601-607. doi: 10.21614/chirurgia.2778. PMID: 36318691
Dermatofibrosarcoma Protuberans: The Current State of Multidisciplinary Management.
Ramirez-Fort MK, Meier-Schiesser B, Niaz MJ, Niaz MO, Feily A, Fort M, Lange CS, Caba D
Skinmed 2020;18(5):288-293. Epub 2020 Oct 1 PMID: 33160438
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
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

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
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: The Current State of Multidisciplinary Management.
Ramirez-Fort MK, Meier-Schiesser B, Niaz MJ, Niaz MO, Feily A, Fort M, Lange CS, Caba D
Skinmed 2020;18(5):288-293. Epub 2020 Oct 1 PMID: 33160438
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

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
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
Current treatment options for dermatofibrosarcoma protuberans.
Rutkowski P, Debiec-Rychter M
Expert Rev Anticancer Ther 2015;15(8):901-9. Epub 2015 May 31 doi: 10.1586/14737140.2015.1052799. PMID: 26027711
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

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
Single-institution review of managing dermatofibrosarcoma protuberans.
Tan YG, Chia CS, Loh WL, Teo MC
ANZ J Surg 2016 May;86(5):372-6. Epub 2015 Sep 2 doi: 10.1111/ans.13276. PMID: 26334110
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

COL1A1::PDGFB fusion-associated uterine fibrosarcoma: A case report and review of the literature.
Rota S, Franza A, Fabbroni C, Paolini B, Greco FG, Alessi A, Padovano B, Casali P, Sanfilippo R
Cancer Rep (Hoboken) 2024 Feb;7(2):e1969. Epub 2024 Jan 26 doi: 10.1002/cnr2.1969. PMID: 38279510Free 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

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