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Dermatofibrosarcoma Protuberans

MedGen UID:
95938
•Concept ID:
C0392784
•
Neoplastic Process
Synonyms: Dermatofibrosarcoma; Dermatofibrosarcoma protuberans; DFSP - dermatofibrosarcoma protuberans
SNOMED CT: Dermatofibrosarcoma (76594008); Dermatofibrosarcoma protuberans (76594008); Dermatofibrosarcoma (276799004); DFSP - dermatofibrosarcoma protuberans (276799004); Dermatofibrosarcoma protuberans (276799004)

Definition

A low grade fibroblastic neoplasm presenting as a nodular cutaneous mass, most often on the trunk and the proximal extremities. The tumor diffusely infiltrates the dermis and the subcutaneous tissues. It is considered a locally aggressive neoplasm, which often recurs but rarely metastasizes. [from NCI]

Professional guidelines

PubMed

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
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article

Recent clinical studies

Etiology

Cutaneous Sarcomas.
Tillman BN, Liu JC
Otolaryngol Clin North Am 2021 Apr;54(2):369-378. Epub 2021 Feb 16 doi: 10.1016/j.otc.2020.11.010. PMID: 33602520
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
Fibrohistiocytic Tumors.
Romano RC, Fritchie KJ
Clin Lab Med 2017 Sep;37(3):603-631. Epub 2017 Jun 15 doi: 10.1016/j.cll.2017.05.007. PMID: 28802503
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Kutane Sarkome.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446

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
Fibrohistiocytic Tumors.
Romano RC, Fritchie KJ
Clin Lab Med 2017 Sep;37(3):603-631. Epub 2017 Jun 15 doi: 10.1016/j.cll.2017.05.007. PMID: 28802503
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
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Kutane Sarkome.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446

Therapy

A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free 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
Dermatofibrosarcoma protuberans and gastrointestinal stromal tumor as models for targeted therapy in soft tissue sarcomas.
Koseła-Paterczyk H, Rutkowski P
Expert Rev Anticancer Ther 2017 Dec;17(12):1107-1116. Epub 2017 Oct 13 doi: 10.1080/14737140.2017.1390431. PMID: 28988501
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
Sarcoma.
Skubitz KM, D'Adamo DR
Mayo Clin Proc 2007 Nov;82(11):1409-32. doi: 10.4065/82.11.1409. PMID: 17976362

Prognosis

The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Cutaneous Sarcomas.
Bhatt MD, Nambudiri VE
Hematol Oncol Clin North Am 2019 Feb;33(1):87-101. doi: 10.1016/j.hoc.2018.08.007. PMID: 30497679
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
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Kutane Sarkome.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446

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
Pigmented neurofibroma with hypertrichosis.
Godínez-Chaparro JA, Valencia-Herrera AM, Mena-Cedillos CA, Toussaint-Caire S, Duarte-Abdala MR, Loza-Escutia O, Toledo-Bahena ME
Bol Med Hosp Infant Mex 2023;80(1):57-62. doi: 10.24875/BMHIM.21000197. PMID: 36867567
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article
Microarray analysis of sarcomas.
Nielsen TO
Adv Anat Pathol 2006 Jul;13(4):166-73. doi: 10.1097/00125480-200607000-00003. PMID: 16858150

Recent systematic reviews

Comparing Mohs micrographic surgery and wide local excision in the management of head and neck dermatofibrosarcoma protuberans: a scoping review.
Sanabria A, Pinillos P, Chiesa-Estomba C, Guntinas-Lichius O, Kowalski LP, Mäkitie AA, Rao KN, Ferlito A
J Dermatolog Treat 2024 Dec;35(1):2295816. Epub 2023 Dec 26 doi: 10.1080/09546634.2023.2295816. PMID: 38146660
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
Tyrosine kinase inhibitors versus radiation therapy in unresectable dermatofibrosarcoma protuberans (DFSP): A narrative systematic review.
Henry OS, Platoff R, Cerniglia KS, Batchu S, Goodwin BJ, Sandilos G, Adams A, Hong YK
Am J Surg 2023 Feb;225(2):268-274. Epub 2022 Sep 26 doi: 10.1016/j.amjsurg.2022.09.040. PMID: 36184329
Dermatofibrosarcoma Protuberans Recurrence After Wide Local Excision Versus Mohs Micrographic Surgery: A Systematic Review and Meta-Analysis.
Martin ECS, Vyas KS, Batbold S, Erwin PJ, Brewer JD
Dermatol Surg 2022 May 1;48(5):479-485. Epub 2022 Mar 30 doi: 10.1097/DSS.0000000000003411. PMID: 35353755
A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change.
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. PMID: 24755121

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