DERMATOFIBROSARCOMA PROTUBERANCE; A CASE REPORT
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Abstract
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow-progressing tumor originating from the skin's dermal layer (1). Initially identified by Darier and Ferrand in 1924, DFSP was more precisely characterized by Hoffman in 1925 (2). This neoplasm typically appears as a painless, skin-colored plaque that can exhibit dark red or blue tints(3). Over time, DFSP can grow larger, often becoming raised or ulcerated (2). While it is locally invasive (4) and can penetrate nearby tissues including the subcutaneous layer, muscles, tendons, and even bones (5), it rarely metastasizes (4).
References
1. Wu, T. , Wu, H. , Tsai, Y. , Luo, T. and Lee, C. (2021) Dermatofibrosarcoma Protuberans of the Neck: A Case Report. International Journal of Otolaryngology and Head & Neck Surgery, 10, 85-91. doi: 10.4236/ijohns.2021.102009.
2. Elafram R, Romdhane MB, Khessairi N, Sghaier M, Annabi H. Dermatofibrosarcoma protuberans of the hallux: A case report with review of the literature. Int J Surg Case Rep. 2022 Jul;96:107325. doi: 10.1016/j.ijscr.2022.107325. Epub 2022 Jun 22. PMID: 35810681; PMCID: PMC9284047.
3. Menon G, Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans. [Updated 2024 Apr 18]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513305/
4. AbuMoussa S, Roshan MP, Souza FF, Daley D, Rosenberg A, Pretell J, Fullerton N, Subhawong T. Soft Tissue Masses of the Hand: A Review of Clinical Presentation and Imaging Features. Curr Oncol. 2023 Feb 7;30(2):2032-2048. doi: 10.3390/curroncol30020158. PMID: 36826119; PMCID: PMC9955517.
5. Lyu A, Wang Q. Dermatofibrosarcoma protuberans: A clinical analysis. Oncol Lett. 2018 Aug;16(2):1855-1862. doi: 10.3892/ol.2018.8802. Epub 2018 May 24. PMID: 30008876; PMCID: PMC6036409.
6. Al Laham O, Atia F, Shaheen J, Alkhoury L, Ibrahim D, Alchikh Omar M. A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report. Int J Surg Case Rep. 2022 Jul;96:107334. doi: 10.1016/j.ijscr.2022.107334. Epub 2022 Jun 22. PMID: 35777334; PMCID: PMC9284050.
7. Paramythiotis D, Stavrou G, Panagiotou D, Petrakis G, Michalopoulos A. Dermatofibrosarcoma protuberans: a case report and review of the literature. Hippokratia. 2016 Jan-Mar;20(1):80-83. PMID: 27895450; PMCID: PMC5074405.
8. Kesserwan C, Sokolic R, Cowen EW, Garabedian E, Heselmeyer-Haddad K, Lee CC, Pittaluga S, Ortiz C, Baird K, Lopez-Terrada D, Bridge J, Wayne AS, Candotti F. Multicentric dermatofibrosarcoma protuberans in patients with adenosine deaminase-deficient severe combined immune deficiency. J Allergy Clin Immunol. 2012 Mar;129(3):762-769.e1. doi: 10.1016/j.jaci.2011.10.028. Epub 2011 Dec 6. PMID: 22153773; PMCID: PMC3294021.
9. Allen A, Ahn C, Sangüeza OP. Dermatofibrosarcoma Protuberans. Dermatol Clin. 2019 Oct;37(4):483-488
10. Thway K, Noujaim J, Jones RL, Fisher C. Dermatofibrosarcoma protuberans: pathology, genetics, and potential therapeutic strategies. Ann Diagn Pathol. 2016 Dec;25:64-71
11. Effiom OA, Olojede ACO, Akinde OR, Olawuyi AB, Amoo AT, Arotiba GT. Dermatofibrosarcoma protuberans: clinicopathologic presentation in Nigerians. Pan Afr Med J. 2018 Sep 12;31:25. doi: 10.11604/pamj.2018.31.25.13665. PMID: 30918552; PMCID: PMC6430858.
12. Pathak B, Maimoon S. Dermatofibrosarcoma Protuberans Over Right Lumbar Region: A Case Report. Cureus. 2023 Jan 1;15(1):e33208. doi: 10.7759/cureus.33208. PMID: 36733580; PMCID: PMC9887664.
13. Gong X, Li J, Ding A, Chen J, Tao X, Xiong P, Rao Y, Liu Y, Sun Q. Multimodal ultrasound for preoperative evaluation of dermatofibrosarcoma protuberans: a series of 40 cases. BMC Cancer. 2022 Nov 5;22(1):1137. doi: 10.1186/s12885-022-10211-4. PMID: 36335314; PMCID: PMC9637320.
14. Hao X, Billings SD, Wu F, Stultz TW, Procop GW, Mirkin G, Vidimos AT. Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment. J Clin Med. 2020 Jun 5;9(6):1752. doi: 10.3390/jcm9061752. PMID: 32516921; PMCID: PMC7355835.
15. Al Laham O, Atia F, Shaheen J, Alkhoury L, Ibrahim D, Alchikh Omar M. A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report. Int J Surg Case Rep. 2022 Jul;96:107334. doi: 10.1016/j.ijscr.2022.107334. Epub 2022 Jun 22. PMID: 35777334; PMCID: PMC9284050.
16. Silvia Stacchiotti, Annalisa Astolfi, Alessandro Gronchi, Andrea Fontana, Maria A. Pantaleo, Tiziana Negri, Monica Brenca, Marcella Tazzari, Milena Urbini, Valentina Indio, Chiara Colombo, Stefano Radaelli, Silvia Brich, Angelo P. Dei Tos, Paolo G. Casali, Chiara Castelli, Gian Paolo Dagrada, Silvana Pilotti, Roberta Maestro; Evolution of Dermatofibrosarcoma Protuberans to DFSP-Derived Fibrosarcoma: An Event Marked by Epithelial–Mesenchymal Transition–like Process and 22q Loss. Mol Cancer Res 1 September 2016; 14 (9): 820–829. https://doi.org/10.1158/1541-7786.MCR-16-0068
17. Hamid R, Hafeez A, Darzi AM, Zaroo I, Owais H, Akhter A. Dermatofibrosarcoma protuberans: Role of wide local excision. South Asian J Cancer. 2013 Oct;2(4):232-8. doi: 10.4103/2278-330X.119926. PMID: 24455646; PMCID: PMC3889049.
18. Sambri A, Caldari E, Fiore M, Zucchini R, Giannini C, Pirini MG, Spinnato P, Cappelli A, Donati DM, De Paolis M. Margin Assessment in Soft Tissue Sarcomas: Review of the Literature. Cancers (Basel). 2021 Apr 2;13(7):1687. doi: 10.3390/cancers13071687. PMID: 33918457; PMCID: PMC8038240.
19. Saporito F, Charalampoudis P, Kinney M, Knox S. Operative management of dermatofibrosarcoma protuberans of the breast. Proc (Bayl Univ Med Cent). 2017 Jan;30(1):128-129. doi: 10.1080/08998280.2017.11929558. PMID: 28154469; PMCID: PMC5287695.
20. Kong K, Rowlands CJ, Varma S, Perkins W, Leach IH, Koloydenko AA, Williams HC, Notingher I. Diagnosis of tumors during tissue-conserving surgery with integrated autofluorescence and Raman scattering microscopy. Proc Natl Acad Sci U S A. 2013 Sep 17;110(38):15189-94. doi: 10.1073/pnas.1311289110. Epub 2013 Sep 3. PMID: 24003124; PMCID: PMC3780864.
21. Muto P, Pastore F. Radiotherapy in the Adjuvant and Advanced Setting of CSCC. Dermatol Pract Concept. 2021 Oct 1;11(Suppl 2):e2021168S. doi: 10.5826/dpc.11S2a168S. PMID: 34877076; PMCID: PMC8609952.
22. Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71:781–786.
2. Elafram R, Romdhane MB, Khessairi N, Sghaier M, Annabi H. Dermatofibrosarcoma protuberans of the hallux: A case report with review of the literature. Int J Surg Case Rep. 2022 Jul;96:107325. doi: 10.1016/j.ijscr.2022.107325. Epub 2022 Jun 22. PMID: 35810681; PMCID: PMC9284047.
3. Menon G, Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans. [Updated 2024 Apr 18]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK513305/
4. AbuMoussa S, Roshan MP, Souza FF, Daley D, Rosenberg A, Pretell J, Fullerton N, Subhawong T. Soft Tissue Masses of the Hand: A Review of Clinical Presentation and Imaging Features. Curr Oncol. 2023 Feb 7;30(2):2032-2048. doi: 10.3390/curroncol30020158. PMID: 36826119; PMCID: PMC9955517.
5. Lyu A, Wang Q. Dermatofibrosarcoma protuberans: A clinical analysis. Oncol Lett. 2018 Aug;16(2):1855-1862. doi: 10.3892/ol.2018.8802. Epub 2018 May 24. PMID: 30008876; PMCID: PMC6036409.
6. Al Laham O, Atia F, Shaheen J, Alkhoury L, Ibrahim D, Alchikh Omar M. A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report. Int J Surg Case Rep. 2022 Jul;96:107334. doi: 10.1016/j.ijscr.2022.107334. Epub 2022 Jun 22. PMID: 35777334; PMCID: PMC9284050.
7. Paramythiotis D, Stavrou G, Panagiotou D, Petrakis G, Michalopoulos A. Dermatofibrosarcoma protuberans: a case report and review of the literature. Hippokratia. 2016 Jan-Mar;20(1):80-83. PMID: 27895450; PMCID: PMC5074405.
8. Kesserwan C, Sokolic R, Cowen EW, Garabedian E, Heselmeyer-Haddad K, Lee CC, Pittaluga S, Ortiz C, Baird K, Lopez-Terrada D, Bridge J, Wayne AS, Candotti F. Multicentric dermatofibrosarcoma protuberans in patients with adenosine deaminase-deficient severe combined immune deficiency. J Allergy Clin Immunol. 2012 Mar;129(3):762-769.e1. doi: 10.1016/j.jaci.2011.10.028. Epub 2011 Dec 6. PMID: 22153773; PMCID: PMC3294021.
9. Allen A, Ahn C, Sangüeza OP. Dermatofibrosarcoma Protuberans. Dermatol Clin. 2019 Oct;37(4):483-488
10. Thway K, Noujaim J, Jones RL, Fisher C. Dermatofibrosarcoma protuberans: pathology, genetics, and potential therapeutic strategies. Ann Diagn Pathol. 2016 Dec;25:64-71
11. Effiom OA, Olojede ACO, Akinde OR, Olawuyi AB, Amoo AT, Arotiba GT. Dermatofibrosarcoma protuberans: clinicopathologic presentation in Nigerians. Pan Afr Med J. 2018 Sep 12;31:25. doi: 10.11604/pamj.2018.31.25.13665. PMID: 30918552; PMCID: PMC6430858.
12. Pathak B, Maimoon S. Dermatofibrosarcoma Protuberans Over Right Lumbar Region: A Case Report. Cureus. 2023 Jan 1;15(1):e33208. doi: 10.7759/cureus.33208. PMID: 36733580; PMCID: PMC9887664.
13. Gong X, Li J, Ding A, Chen J, Tao X, Xiong P, Rao Y, Liu Y, Sun Q. Multimodal ultrasound for preoperative evaluation of dermatofibrosarcoma protuberans: a series of 40 cases. BMC Cancer. 2022 Nov 5;22(1):1137. doi: 10.1186/s12885-022-10211-4. PMID: 36335314; PMCID: PMC9637320.
14. Hao X, Billings SD, Wu F, Stultz TW, Procop GW, Mirkin G, Vidimos AT. Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment. J Clin Med. 2020 Jun 5;9(6):1752. doi: 10.3390/jcm9061752. PMID: 32516921; PMCID: PMC7355835.
15. Al Laham O, Atia F, Shaheen J, Alkhoury L, Ibrahim D, Alchikh Omar M. A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report. Int J Surg Case Rep. 2022 Jul;96:107334. doi: 10.1016/j.ijscr.2022.107334. Epub 2022 Jun 22. PMID: 35777334; PMCID: PMC9284050.
16. Silvia Stacchiotti, Annalisa Astolfi, Alessandro Gronchi, Andrea Fontana, Maria A. Pantaleo, Tiziana Negri, Monica Brenca, Marcella Tazzari, Milena Urbini, Valentina Indio, Chiara Colombo, Stefano Radaelli, Silvia Brich, Angelo P. Dei Tos, Paolo G. Casali, Chiara Castelli, Gian Paolo Dagrada, Silvana Pilotti, Roberta Maestro; Evolution of Dermatofibrosarcoma Protuberans to DFSP-Derived Fibrosarcoma: An Event Marked by Epithelial–Mesenchymal Transition–like Process and 22q Loss. Mol Cancer Res 1 September 2016; 14 (9): 820–829. https://doi.org/10.1158/1541-7786.MCR-16-0068
17. Hamid R, Hafeez A, Darzi AM, Zaroo I, Owais H, Akhter A. Dermatofibrosarcoma protuberans: Role of wide local excision. South Asian J Cancer. 2013 Oct;2(4):232-8. doi: 10.4103/2278-330X.119926. PMID: 24455646; PMCID: PMC3889049.
18. Sambri A, Caldari E, Fiore M, Zucchini R, Giannini C, Pirini MG, Spinnato P, Cappelli A, Donati DM, De Paolis M. Margin Assessment in Soft Tissue Sarcomas: Review of the Literature. Cancers (Basel). 2021 Apr 2;13(7):1687. doi: 10.3390/cancers13071687. PMID: 33918457; PMCID: PMC8038240.
19. Saporito F, Charalampoudis P, Kinney M, Knox S. Operative management of dermatofibrosarcoma protuberans of the breast. Proc (Bayl Univ Med Cent). 2017 Jan;30(1):128-129. doi: 10.1080/08998280.2017.11929558. PMID: 28154469; PMCID: PMC5287695.
20. Kong K, Rowlands CJ, Varma S, Perkins W, Leach IH, Koloydenko AA, Williams HC, Notingher I. Diagnosis of tumors during tissue-conserving surgery with integrated autofluorescence and Raman scattering microscopy. Proc Natl Acad Sci U S A. 2013 Sep 17;110(38):15189-94. doi: 10.1073/pnas.1311289110. Epub 2013 Sep 3. PMID: 24003124; PMCID: PMC3780864.
21. Muto P, Pastore F. Radiotherapy in the Adjuvant and Advanced Setting of CSCC. Dermatol Pract Concept. 2021 Oct 1;11(Suppl 2):e2021168S. doi: 10.5826/dpc.11S2a168S. PMID: 34877076; PMCID: PMC8609952.
22. Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71:781–786.
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Published
Jul 9, 2024
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How to Cite
Sania Malik, Qudsiya Bano, Nimra Ehsan, Zeeshan Masood Mian, Taha Alam, & Muhammad Haider Ali. (2024). DERMATOFIBROSARCOMA PROTUBERANCE; A CASE REPORT. Journal of Population Therapeutics and Clinical Pharmacology, 31(7), 291–295. https://doi.org/10.53555/jptcp.v31i7.6948
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Author Biographies
Sania Malik
Resident Surgeon, Khyber Teaching Hospital Peshawar, Pakistan
Qudsiya Bano
Fellow Plastic Surgery, Shaikh Zayed Hospital Lahore, Pakistan
Nimra Ehsan
Medical Officer, MTI Hayatabad Medical Complex Peshawar, Pakistan
Zeeshan Masood Mian
Medical Officer, MTI Hayatabad Medical Complex Peshawar, Pakistan
Taha Alam
MBBS, Student, DOW University of Health Sciences
Muhammad Haider Ali
Resident Surgeon, MTI Hayatabad Medical Complex Peshawar, Pakistan