Dermatofibrosarcoma protuberans: Under the mask of scar
- Authors: Vertieva E.Y.1, Kayumova L.N.1, Bobkova A.E.1
-
Affiliations:
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
- Issue: Vol 26, No 6 (2023)
- Pages: 545-552
- Section: DERMATO-ONCOLOGY
- URL: https://journals.rcsi.science/1560-9588/article/view/254766
- DOI: https://doi.org/10.17816/dv568542
- ID: 254766
Cite item
Abstract
Dermatofibrosarcoma protuberans (Darier–Ferrand) is a rare mesenchymal tumor of fibrohistiocytic genesis with moderate malignancy and low incidence of metastasis, the pathogenesis and etiology of which remain unclear. The diagnosis is based on the clinical picture of a reddish-brown dense plaque or papule that gradually becomes a painful node with ulceration. Histological examination reveals the infiltrate with a moire pattern in the dermis in the form of fusiform cells with diffuse CD34+ staining during immunohistochemical analysis. Treatment of dermatofibrosarcoma protuberans (Darier–Ferrand) is a serious problem due to the high tendency for recurrence after removal. The methods described in the literature include extensive excision, Mohs surgery, chemotherapy, radiation therapy and targeted treatment with an anticancer protein-tyrosine kinase inhibitor.
The authors present their own clinical observation of a patient with dermatofibrosarcoma protuberans (Darier–Ferrand) in whom a diagnostic error by only visual method and an incorrect treatment led to the worsening of skin process. The interest of this clinical observation lies in the difficulty of diagnosis during the initial visit, in the complexity of differential and morphological diagnosis.
Keywords
Full Text
##article.viewOnOriginalSite##About the authors
Ekaterina Yu. Vertieva
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Email: ivertieva@gmail.com
ORCID iD: 0000-0002-1088-2911
SPIN-code: 3712-8453
MD, Cand. Sci. (Med.)
Russian Federation, 8/2 Trubetskaya street, 119992 MoscowLyailya N. Kayumova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Author for correspondence.
Email: avestohka2005@inbox.ru
ORCID iD: 0000-0003-0301-737X
SPIN-code: 4391-9553
MD, Cand. Sci. (Med.), Assistant Lecturer
Russian Federation, 8/2 Trubetskaya street, 119992 MoscowAnna E. Bobkova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Email: anya_bobkova98@mail.ru
ORCID iD: 0000-0003-3611-0917
SPIN-code: 5345-5746
Russian Federation, 8/2 Trubetskaya street, 119992 Moscow
References
- Clinical recommendations [Internet]. Soft tissue sarcomas. 2022-2023-2024 (25.07.2022). Approved by the Ministry of Health of the Russian Federation. Available from: http://disuria.ru/_ld/12/1283_kr22C49MZ.pdf. Accessed: 15.10.2023. (In Russ).
- Vashchenko LN, Dashkova IR, Salatov RN, et al. To the problem of surgical treatment soft tissue sarcomas. Int J Applied Fundamental Res. 2015;(8):234–238. (In Russ).
- Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023.
- Chuprov IN, Sydikov AA, Zaslavsky DV, Nasyrov RA. Dermatooncopathology: An illustrated guide for physicians. Ed. by I.N. Chuprov, A.A. Sydikov. Moscow: GEOTAR-Media; 2021. 528 p. (In Russ).
- Cabral R, Wilford M, Ramdass MJ, et al. Dermatofibrosarcoma protuberans associated with trauma: A case report. Mol Clin Oncol. 2020;13(5):51. doi: 10.3892/mco.2020.2121
- De Antoni E, Brambullo T, Pescarini E, et al. Dermatofibrosarcoma protuberans on tattooed skin: A case report. Adv Skin Wound Care. 2020;33(2):104–108. doi: 10.1097/01.ASW.0000613548.11947.b4
- Hayakawa K, Matsumoto S, Ae K, et al. Risk factors for distant metastasis of dermatofibrosarcoma protuberans. J Orthop Traumatol. 2016;17(3):261–266. doi: 10.1007/s10195-016-0415-x
- Abbott JJ, Oliveira AM, Nascimento AG. The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol. 2006;30(4):436–443. doi: 10.1097/00000478-200604000-00002
- Kibbi N, Wang D, Wang WL, et al. Dermatofibrosarcoma protuberans in pregnancy: A case series and review of the literature. Int J Dermatol. 2021;60(9):1114–1119. doi: 10.1111/ijd.15497
- Parlette LE, Smith CK, Germain LM, et al. Accelerated growth of dermatofibrosarcoma protuberans during pregnancy. J Am Acad Dermatol. 1999;41(5 Pt 1):778–783. doi: 10.1016/s0190-9622(99)70023-x
- Ugurel S, Kortmann RD, Mohr P. S1 guidelines for dermatofibrosarcoma protuberans (DFSP): Update 2018. J Dtsch Dermatol Ges. 2019;17(6):663–668. doi: 10.1111/ddg.13849
- Lyu A, Wang Q. Dermatofibrosarcoma protuberans: A clinical analysis. Oncol Lett. 2018;16(2):1855–1862. doi: 10.3892/ol.2018.8802
- Katina MA, Lesnichaya OV, Ryazanova NV. Dermatofibrosarcoma protuberans in dermatological practice. Case report. Consilium Medicum. 2022;24(8):523–528. (In Russ). doi: 10.26442/20751753.2022.8.201721
- Hao Х, Billings SD, Wu F, et al. Dermatofibrosarcoma protuberans: Update on the diagnosis and treatment. J Clin Med. 2020;9(1752):22. doi: 10.3390/jcm9061752
- Rutkowski P, Debiec-Rychter M. Current treatment options for dermatofibrosarcoma protuberans. Expert Rev Anticancer Ther. 2015;15(8):901–909. doi: 10.1586/14737140.2015.1052799
- An illustrated guide to dermatology. To prepare physicians for accreditation. Ed. by O.Yu. Olisova, N.P. Teplyuk. Moscow: GEOTAR-Media; 2023. 376 p. (In Russ).
- Asanov AY, Filippova MG. Neurofibromatosis: The current state of the problem. Russ J Skin Venereal Diseases. 2011;(5):14–20. (In Russ).
- Acosta AE, Vélez CS. Dermatofibrosarcoma protuberans. Curr Treat Options Oncol. 2017;18(9):56. doi: 10.1007/s11864-017-0498-5
- Mullen JT. Dermatofibrosarcoma protuberans: Wide local excision versus mohs micrographic surgery. Surg Oncol Clin N Am. 2016;25(4):827–839. doi: 10.1016/j.soc.2016.05.011
- Vitiello GA, Lee AY, Berman RS. Dermatofibrosarcoma protuberans: What is this? Surg Clin North Am. 2022;102(4):657–665. doi: 10.1016/j.suc.2022.05.004
- Mareş T, Răducu L, Avino A, et al. Dermatofibrosarcoma protuberans: One centre experience. Chirurgia (Bucur). 2022;117(5):601–607. doi: 10.21614/chirurgia.2778
- Galchina YS, Gogia BS, Stepanova YA. Dermatofibrosarcoma protuberans: Literature review and case report. Med Visualization. 2016;(2):119–130. (In Russ).
- Kilian KJ, Ruzicka T, Flaig M, et al. Recurrent fibrosarcomatous dermatofibrosarcoma protuberans. Ultrasound imaging. Hautarzt. 2013;64(7):512–515. doi: 10.1007/s00105-013-2559-4
Supplementary files
![](/img/style/loading.gif)