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Dermatofibrosarcoma Protuberans (DFSP): An Uncommon Skin Cancer

Received: 7 August 2021    Accepted: 21 August 2021    Published: 5 November 2021
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Abstract

Dermatofibrosarcoma Protuberans (DFSP) is a very uncommon type of skin cancer that begins in the connective tissues of skin and extend to nearby structures in the form of lump. The cause of cutaneous fibroids is unknown, although a suggested risk factor includes skin lesions at the affected site. DFSP tends to affect people between the age of 20 and 50, but it has been diagnosed in people of all ages. This case report describes a highly recurrent DFSP disease condition of 25 years old female, who got admitted in a tertiary care cancer centre with complaints of painless swelling in the back, fever, and loss of weight since last 1 month. Surgical history revealed three past surgical excisions of the mass over the same location. CT scan and tissue biopsy revealed recurrent Dermatofibrosarcoma protuberans. Initially, the patient was on imatinib 600 mg/PO/OD, after which there was a subjective decrease in size of mass. But as the patient was unable to tolerate high doses of imatinib, she then underwent wide local excision of the mass with split skin graft taken from right thigh under general anesthesia. Surgery was uneventful and she was discharged thereafter. As local recurrence is common, patients required close clinical follow-up after completing treatment.

Published in American Journal of Nursing and Health Sciences (Volume 2, Issue 4)
DOI 10.11648/j.ajnhs.20210204.11
Page(s) 87-89
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2021. Published by Science Publishing Group

Keywords

Dermatofibrosarcoma Protuberans, Surgical Excisions, Soft Tissue Sarcoma, Rare Cancers

References
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[2] Dermatofibrosarcoma protuberans: MedlinePlus Genetics [Internet]. [cited 2021 May 15]. Available from: https://medlineplus.gov/genetics/condition/dermatofibrosarcoma-protuberans/.
[3] Dermatofibrosarcoma protuberans | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program [Internet]. [cited 2021 May 15]. Available from: https://rarediseases.info.nih.gov/diseases/9569/dermatofibrosarcoma-protuberans.
[4] Kamar FG, Kairouz VF, Sabri AN. Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report. Clinical Sarcoma Research. 2013 Apr 4; 3 (1): 5.
[5] Al Barwani AS, Taif S, Al Mazrouai RA, Al Muzahmi KS, Alrawi A. Dermatofibrosarcoma Protuberans: Insights into a Rare Soft Tissue Tumor. 6: 16.
[6] Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans [Internet]. StatPearls [Internet]. StatPearls Publishing; 2021 [cited 2021 May 15]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513305/.
[7] Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1992 Jun; 18 (3): 241–8.
[8] MD KO MD; Jonathan Courtney, MD; Richard Gorlick, MD and David Geller. Dermatofibrosarcoma Protuberans: Diagnosis, Treatment, Prognosis & Support [Internet]. Liddy Shriver Sarcoma Initiative. [cited 2021 May 15]. Available from: http://sarcomahelp.org/dermatofibrosarcoma-protuberans.html.
[9] Wiesmueller F, Agaimy A, Perrakis A, Arkudas A, Horch RE, Grützmann R, et al. Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor. World Journal of Surgical Oncology. 2019 May 28; 17 (1): 90.
[10] Asiri M, Moghazy KM, Alsaif HS, Al-Qahtani MS. Dermatofibrosarcoma Protuberance: A Case Report and Review of Literature. Biomedical Research [Internet]. 2008 [cited 2021 May 15]; 19 (2). Available from: https://www.alliedacademies.org/abstract/dermatofibrosarcoma-protuberance-a-case-report-and-review-of-literature-811.html.
[11] Tateishi U, Gladish GW, Kusumoto M, Hasegawa T, Yokoyama R, Tsuchiya R, et al. Chest wall tumors: radiologic findings and pathologic correlation: part 2. Malignant tumors. Radiographics. 2003 Dec; 23 (6): 1491–508.
[12] Chan IL, Carneiro S, Menezes M, Ramos-E-Silva S, Magalhães T, Cuzzi T, et al. Dermatofibrosarcoma protuberans: a case report. Case Rep Dermatol. 2014 May 9; 6 (2): 134–9.
[13] Bhambri S, Desai A, Del Rosso J, Mobini N. Dermatofibrosarcoma protuberans: A case report and review of the literature. The Journal of clinical and aesthetic dermatology. 2008 May 1; 1: 34–6.
[14] Veronese F, Boggio P, Tiberio R, Gattoni M, Fava P, Caliendo V, et al. Wide local excision vs. Mohs Tübingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review. Journal of the European Academy of Dermatology and Venereology. 2017 Dec 1; 31 (12): 2069–76.
[15] Saiag P, Grob J-J, Lebbe C, Malvehy J, del Marmol V, Pehamberger H, et al. Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline. European Journal of Cancer. 2015 Nov 1; 51 (17): 2604–8.
[16] Lowe GC, Onajin O, Baum CL, Otley CC, Arpey CJ, Roenigk RK, et al. A Comparison of Mohs Micrographic Surgery and Wide Local Excision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience. Dermatologic Surgery [Internet]. 2017; 43 (1). Available from: https://journals.lww.com/dermatologicsurgery/Fulltext/2017/01000/A_Comparison_of_Mohs_Micrographic_Surgery_and_Wide.14.aspx.
[17] Miller SJ, Alam M, Andersen JS, Berg D, Bichakjian CK, Bowen GM, et al. Dermatofibrosarcoma Protuberans. Journal of the National Comprehensive Cancer Network. 2012 Mar 1; 10 (3): 312–8.
[18] RESERVED IU-AR. Orphanet: Dermatofibrosarcoma protuberans [Internet]. [cited 2021 May 16]. Available from: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=31112.
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  • APA Style

    Saumya Prakash Srivastava. (2021). Dermatofibrosarcoma Protuberans (DFSP): An Uncommon Skin Cancer. American Journal of Nursing and Health Sciences, 2(4), 87-89. https://doi.org/10.11648/j.ajnhs.20210204.11

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    ACS Style

    Saumya Prakash Srivastava. Dermatofibrosarcoma Protuberans (DFSP): An Uncommon Skin Cancer. Am. J. Nurs. Health Sci. 2021, 2(4), 87-89. doi: 10.11648/j.ajnhs.20210204.11

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    AMA Style

    Saumya Prakash Srivastava. Dermatofibrosarcoma Protuberans (DFSP): An Uncommon Skin Cancer. Am J Nurs Health Sci. 2021;2(4):87-89. doi: 10.11648/j.ajnhs.20210204.11

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  • @article{10.11648/j.ajnhs.20210204.11,
      author = {Saumya Prakash Srivastava},
      title = {Dermatofibrosarcoma Protuberans (DFSP): An Uncommon Skin Cancer},
      journal = {American Journal of Nursing and Health Sciences},
      volume = {2},
      number = {4},
      pages = {87-89},
      doi = {10.11648/j.ajnhs.20210204.11},
      url = {https://doi.org/10.11648/j.ajnhs.20210204.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajnhs.20210204.11},
      abstract = {Dermatofibrosarcoma Protuberans (DFSP) is a very uncommon type of skin cancer that begins in the connective tissues of skin and extend to nearby structures in the form of lump. The cause of cutaneous fibroids is unknown, although a suggested risk factor includes skin lesions at the affected site. DFSP tends to affect people between the age of 20 and 50, but it has been diagnosed in people of all ages. This case report describes a highly recurrent DFSP disease condition of 25 years old female, who got admitted in a tertiary care cancer centre with complaints of painless swelling in the back, fever, and loss of weight since last 1 month. Surgical history revealed three past surgical excisions of the mass over the same location. CT scan and tissue biopsy revealed recurrent Dermatofibrosarcoma protuberans. Initially, the patient was on imatinib 600 mg/PO/OD, after which there was a subjective decrease in size of mass. But as the patient was unable to tolerate high doses of imatinib, she then underwent wide local excision of the mass with split skin graft taken from right thigh under general anesthesia. Surgery was uneventful and she was discharged thereafter. As local recurrence is common, patients required close clinical follow-up after completing treatment.},
     year = {2021}
    }
    

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    T1  - Dermatofibrosarcoma Protuberans (DFSP): An Uncommon Skin Cancer
    AU  - Saumya Prakash Srivastava
    Y1  - 2021/11/05
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    DO  - 10.11648/j.ajnhs.20210204.11
    T2  - American Journal of Nursing and Health Sciences
    JF  - American Journal of Nursing and Health Sciences
    JO  - American Journal of Nursing and Health Sciences
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    AB  - Dermatofibrosarcoma Protuberans (DFSP) is a very uncommon type of skin cancer that begins in the connective tissues of skin and extend to nearby structures in the form of lump. The cause of cutaneous fibroids is unknown, although a suggested risk factor includes skin lesions at the affected site. DFSP tends to affect people between the age of 20 and 50, but it has been diagnosed in people of all ages. This case report describes a highly recurrent DFSP disease condition of 25 years old female, who got admitted in a tertiary care cancer centre with complaints of painless swelling in the back, fever, and loss of weight since last 1 month. Surgical history revealed three past surgical excisions of the mass over the same location. CT scan and tissue biopsy revealed recurrent Dermatofibrosarcoma protuberans. Initially, the patient was on imatinib 600 mg/PO/OD, after which there was a subjective decrease in size of mass. But as the patient was unable to tolerate high doses of imatinib, she then underwent wide local excision of the mass with split skin graft taken from right thigh under general anesthesia. Surgery was uneventful and she was discharged thereafter. As local recurrence is common, patients required close clinical follow-up after completing treatment.
    VL  - 2
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Author Information
  • Vivekanand Polyclinic College of Nursing, Lucknow, India

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