Dermatofibrosarcoma: report of a case with complete response to imatinib and conservative surgery during the COVID-19 pandemic

ABSTRACT

Dermatofibrosarcoma protuberans (DFSP) is an extremely rare and potentially aggressive soft tissue tumor. Around 85 to 90% of DFSP cases are low-grade tumors, while the fibrosarcomatous variant (DFSP-FS) is associated with medium and high-grade neoplasms. DFSP affects more young adults, in their 30s; however, it can be present in all ages. It has a high potential for locoregional dissemination and recurrence, directly related to tumor size. In the present study, a case of dermatofibrosarcoma protuberans with local recurrence after surgical treatment will be reported, subsequently treated with imatinib and a new surgical approach, with no new recurrences so far. The effectiveness of imatinib is noted in a deep, infiltrative and relapsed DFSP, in which upfront surgery would cause significant functional and cosmetic damage. Imatinib allowed an important clinical improvement for the patient, with tumor reduction in the initial weeks of treatment.

Keywords:

Dermatofibrosarcoma - Imatinib mesylate - Complementary therapies

Publikationsverlauf

Eingereicht: 18. Juli 2021

Angenommen: 31. August 2021

Artikel online veröffentlicht:
09. März 2022

• REFERENCES

• 1 Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM. et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer 2000; Jun; 88 (12) 2711-2720
• 2 Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol 1992; Jun; 18 (03) 241-248
• 3 Bergin P, Rezaei S, Lau Q, Coucher J. Dermatofibrosarcoma protuberans, magnetic resonance imaging and pathological correlation. Australas Radiol 2007; 51 (spe): B64-6
• 4 Thornton SL, Reid J, Papay FA, Vidimos AT. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol 2005; Jul; 53 (01) 7683
• 5 Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O'Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. AJR Am J Roentgenol 2002; Apr; 178 (04) 989-993
• 6 Khatri VP, Galante JM, Bold RJ, Schneider PD, Ramsamooj R, Goodnight Junior JE. Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment. Ann Surg Oncol 2003; Nov; 10 (09) 1118-1122
• 7 Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol 2004; Apr; 30 (03) 341-345
• 8 Fiore M, Miceli R, Mussi C, Lo Vullo S, Mariani L, Lozza L. et al. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol 2005; Oct; 23 (30) 7669-7675
• 9 Stojadinovic A, Hoos A, Karpoff HM, Leung DHY, Antonescu C, Brennan MF. et al. Soft tissue tumors of the abdominal wall: analysis of disease patterns and treatment. Arch Surg 2001; Jan; 136 (01) 70-79
• 10 Behbahani R, Patenotre P, Capon N, Martinot-Duquennoy V, Kulik JF, Piette F. et al. To a margin reduction in the dermatofibrosarcoma protuberans? Retrospective study of 34 cases. Ann Chir Plast Esthet 2005; Jun; 50 (03) 179-185
• 11 Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, Rubin BP, Debiec-Rychter M. et al. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol 2010; Apr; 28 (10) 1772-1779
• 12 McArthur GA, Demetri GD, Van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL. et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with Imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol 2005; Feb; 23 (04) 866-873
• 13 Ugurel S, Mentzel T, Utikal J, Mohr P, Pföhler C, Schiller M. et al. Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up. Clin Cancer Res 2014; Jan; 20 (02) 499-510
• 14 Rutkowski P, Dębiec-Rychter M, Nowecki Z, Michej W, Symonides M, Ptaszynski K. et al. Treatment of advanced dermatofibrosarcoma protuberans with Imatinib mesylate with or without surgical resection. J Eur Acad Dermatol Venereol 2011; Mar; 25 (03) 264-270
• 15 Kérob D, Porcher R, Vérola O, Dalle S, Maubec E, Aubin F. et al. Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients. Clin Cancer Res 2010; Jun; 16 (12) 3288-3295
• 16 Suit H, Spiro I, Mankin HJ, Efird J, Rosenberg AE. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol 1996; Aug; 14 (08) 2365-2369
• 17 Terrier-Lacombe MJ, Guillou L, Maire G. et al. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data--a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol 2003; Jan; 27 (01) 27-39
• 18 Lemm D, Mügge LO, Mentzel T, Höffken K. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol 2009; May; 135 (05) 653-665
• 19 Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD. Fibrosarcomatous (“high-grade”) dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol 1998; May; 22 (05) 576-587
• 20 Arnaud EJ, Perrault M, Revol M, Servant JM, Banzet P. Surgical treatment of dermatofibrosarcoma protuberans. Plast Reconstr Surg 1997; Sep; 100 (04) 884-895
• 21 National Comprehensive Cancer Network (NCCN). NCCN clinical practice guidelines in oncology [Internet]. Plymouth Meeting: NCCN; 2021. ;[access in 2021 May 06]. Available from: https://www.nccn.org/professionals/physician_gls
• 22 Saiag P, Grob JJ, Lebbe C, Malvehy J, Del Marmol V, Pehamberger H. et al. Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline. Eur J Cancer 2015; Nov; 51 (17) 2604-2608
• 23 Gloster Junior HM, Harris KR, Roenigk RK. A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996; Jul; 35 (01) 82-87
• 24 DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK. Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer 2004; Mar; 100 (05) 1008-1016
• 25 Snow SN, Gordon EM, Larson PO, Bagheri MM, Bentz ML, Sable DB. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow- up and review of the literature. Cancer 2004; Jul; 101 (01) 28-38
• 26 Wacker J, Khan-Durani B, Hartschuh W. Modified Mohs micrographic surgery in the therapy of dermatofibrosarcoma protuberans: analysis of 22 patients. Ann Surg Oncol 2004; Apr; 11 (04) 438-444
• 27 Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg 2002; Nov; 28 (11) 1060-1064
• 28 Robinson JK. Dermatofibrosarcoma protuberans resected by Mohs' surgery (chemosurgery). A 5-year prospective study. J Am Acad Dermatol 1985; Jun; 12 (06) 1093-1098
• 29 Ratner D, Thomas CO, Johnson TM, Sondak VK, Hamilton TA, Nelson BR. et al. Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. Results of a multiinstitutional series with an analysis of the extent of microscopic spread. J Am Acad Dermatol 1997; Oct; 37 (04) 600-613
• 30 Haycox CL, Odland PB, Olbricht SM, Casey B. Dermatofibrosarcoma protuberans (DFSP): growth characteristics based on tumor modeling and a review of cases treated with Mohs micrographic surgery. Ann Plast Surg 1997; Mar; 38 (03) 246-251
• 31 Nelson RA, Arlette JP. Mohs micrographic surgery and dermatofibrosarcoma protuberans: a multidisciplinary approach in 44 patients. Ann Plast Surg 2008; Jun; 60 (06) 667-672
• 32 Foroozan M, Sei JF, Amini M, Beauchet A, Saiag P. Efficacy of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: systematic review. Arch Dermatol 2012; Sep; 148 (09) 1055-1063
• 33 Sarcoma Alliance for Research through Collaboration. Neoadjuvant imatinib in DFSP. ClinicalTrials.gov [Internet]. 2005 ;[access in 2012 Nov 01]. Available from: http://clinicaltrials.gov/ct2/show/NCT00243191?term=00243191&rank=1
• 34 A short course of neoadjuvant Gleevec (Imatinib mesylate) in DFSP (NCT 00176709). ClinicalTrials.gov [Internet]. ANO. ;[access in 2012 Nov 01]. Available from: http://clinicaltrials.gov/ct2/results?term=00176709
• 35 Merlo G, Cozzani E, Comandini D, Trave I, Centurioni MG, Franchelli S. et al. Neoadjuvant imatinib as treatment preceding surgery for vulvar dermatofibrosarcoma protuberan. Dermatol Ther 2021; Mar; 34 (02) e14860 https://doi.org/10.1111/dth.14860
• 36 Kamar FG, Kairouz VF, Sabri AN. Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report. Clin Sarcoma Res 2013; 3: 5
• 37 Ong HS, Ji T, Wang LZ, Yu ZW, Zhang CP. Dermatofibrosarcoma protuberans on the right neck with superior vena cava syndrome: case report and literature review. Int J Oral Maxillofac Surg 2013; 42 (06) 707-710
• 38 Delyon J, Porcher R, Battistella M, Meyer N, Adamski H, Bertucci F. et al. A multicenter phase II study of pazopanib in patients with unresectable dermatofibrosarcoma protuberans. J Invest Dermatol 2021; Sep; 141 (04) 761-9.e2