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Dermatofibrosarcoma protuberans – the impact of radiation therapy: a single institution series

Published online by Cambridge University Press:  15 August 2018

Patricia Sebastian ,
Sharief K. Siddique ,
Sunitha Susan Varghese ,
Anne Jennifer Prabhu ,
V. T. K. Titus ,
Selvamani Backianathan  and
Sukriya Nayak
Patricia Sebastian*
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
Sharief K. Siddique
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
Sunitha Susan Varghese
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
Anne Jennifer Prabhu
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
V. T. K. Titus
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
Selvamani Backianathan
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
Sukriya Nayak
Affiliation:
Department of Radiotherapy, Christian Medical College, Vellore, India
*
Author for correspondence: Dr Patricia Sebastian, Department of Radiotherapy, Christian Medical College, Ida Scudder Road, Vellore, 632004, India. E-mail: drpat@cmcvellore.ac.in
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Abstract

Background

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive intermediate malignancy.

Objective

The purpose of this retrospective analysis is to determine the efficacy of radiation therapy (RT) in local control of DFSP.

Patients and methods

The recurrence-free survival (RFS) for 45 patients treated for DFSP at our institution was estimated and compared between surgery alone and postoperative RT groups.

Results

Age range of the patients were in the third and fourth decades; males:females=2:1; most common site: anterior abdominal wall; tumours >5 cm in size in 75%; low grade in 77·8%; margins positive in 31·8% and <5 mm margins in 45·5%. Two-thirds of patients had at least one recurrence before presentation to our institution. RT dose was >50 Gy in 88% of patients. The patients treated with postoperative RT had poorer prognostic factors compared with surgery alone: they were males (17 versus 13 patients), and presented with high-grade tumours (5 versus 1 patients), multiple recurrences prior to presentation (25 versus 20 patients) and positive or <5 mm margins (22 versus 12 patients). Median follow-up for surgery alone group was 17 (1–152) months and for postoperative RT group, this was 54 (5–121) months. RFS at 5 years was 77·1% for surgery alone and 87·9% for postoperative RT group but was not statistically significant. The median time to recurrence was 4 years.

Conclusion

RT delays the time to recurrence in DFSP. RT improves the outcome of DFSP for recurrent tumours and with positive margins.

Keywords

dermatofibrosarcoma protuberansradiotherapyrelapse-free survivaltime to recurrence
Type
Original Article
Information
Journal of Radiotherapy in Practice , Volume 17 , Issue 4 , December 2018 , pp. 390 - 395
Copyright
© Cambridge University Press 2018 

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References

1. Malkud, S, Dyavannanavar, V. Dermatofibrosarcoma protuberans. Indian Dermatol Online J. 2017; 8 (6): 495–497.Google Scholar
2. Lal, P, Goel, A, Mandal, A K. Dermatofibrosarcoma protuberans of scalp with cervical lymph node metastasis. Sarcoma 2004; 8 (1): 43–45.Google Scholar
3. Cai, H, Wang, Y, Wu, J, Shi, Y. Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol 2012; 105 (2): 142–148.Google Scholar
4. Hiraki-Hotokebuchi, Y, Yamada, Y, Kohashi, K et al. Alteration of PDGFRβ-Akt-mTOR pathway signaling in fibrosarcomatous transformation of dermatofibrosarcoma protuberans. Hum Pathol 2017; 67: 60–68.Google Scholar
5. Acosta, A E, Vélez, C S. Dermatofibrosarcoma protuberans. Curr Treat Options Oncol 2017; 18 (9): 56.Google Scholar
6. Veronese, F, Boggio, P, Tiberio, R et al. Wide local excision vs. Mohs Tübingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review. J Eur Acad Dermatol Venereol 2017; 31 (12): 2069–2076.Google Scholar
7. Oh, E, Jeong, H M, Kwon, M J et al. Unforeseen clonal evolution of tumor cell population in recurrent and metastatic dermatofibrosarcoma protuberans. PloS One 2017; 12 (10): e0185826.Google Scholar
8. Rutkowski, P, Debiec-Rychter, M. Current treatment options for dermatofibrosarcoma protuberans. Expert Rev Anticancer Ther 2015; 15 (8): 901–909.Google Scholar
9. Ballo, M T, Zagars, G K, Pisters, P, Pollack, A. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys 1998; 40 (4): 823–827.Google Scholar
10. Suit, H, Spiro, I, Mankin, H J, Efird, J, Rosenberg, A E. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol 1996; 14 (8): 2365–2369.Google Scholar
11. Haas, R L, Keus, R B, Loftus, B M, Rutgers, E J, van Coevorden, F, Bartelink, H. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. Soft Tissue Tumours Working Group. Eur J Cancer 1997; 33 (7): 1055–1060.Google Scholar
12. Li, Y, Wang, C, Xiang, B, Chen, S, Li, L, Ji, Y. Clinical features, pathological findings and treatment of recurrent dermatofibrosarcoma protuberans. J Cancer 2017; 8 (7): 1319–1323.Google Scholar
13. Trofymenko, O, Bordeaux, J S, Zeitouni, N C. Survival in patients with primary dermatofibrosarcoma protuberans: National Cancer Database analysis. J Am Acad Dermatol 2018; 78 (6): 1125–1134.Google Scholar
14. Paolino, G, Didona, D, Bottoni, U et al. Dermatofibrosarcoma protuberans: when the age makes the difference. G Ital Dermatol E Venereol Organo Uff Soc Ital Dermatol E Sifilogr 2018; 153 (3): 344–348.Google Scholar
15. Kreicher, K L, Kurlander, D E, Gittleman, H R, Barnholtz-Sloan, J S, Bordeaux, J S. Incidence and survival of primary dermatofibrosarcoma protuberans in the United States. Dermatol Surg 2016; 42 (suppl 1): S24–S31.Google Scholar
16. Ugurel, S, Becker, J C. Imatinib in dermatofibrosarcoma: targeted therapy or immunotherapy? J Invest Dermatol 2017; 137 (2): 277–279.Google Scholar
17. Rutkowski, P, Klimczak, A, Ługowska, I et al. Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate – the impact of fibrosarcomatous transformation. Eur J Surg Oncol 2017; 43 (6): 1134–1141.Google Scholar
18. Fontecilla, N M, Kittler, N W, Geskin, L et al. Recurrent dermatofibrosarcoma protuberans treated with neoadjuvant imatinib mesylate followed by Mohs micrographic surgery. JAAD Case Rep 2017; 3 (6): 467–469.Google Scholar
19. Wang, C, Luo, Z, Chen, J et al. Target therapy of unresectable or metastatic dermatofibrosarcoma protuberans with imatinib mesylate: an analysis on 22 Chinese patients. Medicine (Baltimore) 2015; 94 (17): e773.Google Scholar
20. Dagan, R, Morris, C G, Zlotecki, R A, Scarborough, M T, Mendenhall, W M. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol 2005; 28 (6): 537–539.Google Scholar
21. Farma, J M, Ammori, J B, Zager, J S et al. Dermatofibrosarcoma protuberans: how wide should we resect? Ann Surg Oncol 2010; 17 (8): 2112–2118.Google Scholar
22. Chen, Y-T, Tu, W-T, Lee, W-R, Huang, Y-C. The efficacy of adjuvant radiotherapy in dermatofibrosarcoma protuberans: a systemic review and meta-analysis. J Eur Acad Dermatol Venereol 2016; 30 (7): 1107–1114.Google Scholar
23. Castle, K O, Guadagnolo, B A, Tsai, C J, Feig, B W, Zagars, G K. Dermatofibrosarcoma protuberans: long-term outcomes of 53 patients treated with conservative surgery and radiation therapy. Int J Radiat Oncol Biol Phys 2013; 86 (3): 585–590.Google Scholar
24. Uysal, B, Sager, O, Gamsiz, H et al. Evaluation of the role of radiotherapy in the management of dermatofibrosarcoma protuberans. J BUON 2013; 18 (1): 268–273.Google Scholar
25. Angouridakis, N, Kafas, P, Jerjes, W et al. Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck. Head Neck Oncol 2011; 3 (1): 5.Google Scholar
26. Sun, L M, Wang, C J, Huang, C C et al. Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiother Oncol 2000; 57 (2): 175–181.Google Scholar
27. Ugurel, S, Kortmann, R-D, Mohr, P, Mentzel, T, Garbe, C, Breuninger, H. Short German guidelines: dermatofibrosarcoma protuberans. J Dtsch Dermatol Ges 2008; 6 (suppl 1): S17–S18.Google Scholar
28. McPeak, C J, Cruz, T, Nicastri, A D. Dermatofibrosarcoma protuberans: an analysis of 86 cases – five with metastasis. Ann Surg 1967; 166 (5): 803–816.Google Scholar
29. Liang, C A, Jambusaria-Pahlajani, A, Karia, P S, Elenitsas, R, Zhang, P D, Schmults, C D. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol 2014; 71 (4): 781–786.Google Scholar