Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS). Issue 2 (19th May 2020)

• Record Type: Journal Article
• Authors: Krewer, Julia; Rolle, Udo; Koscielniak, Ewa; Vokuhl, Christian; Mentzel, Thomas; Seitz, Guido; Feuchtgruber, Simone; von Kalle, Thekla; Scheer, Monika; Münter, Marc; Bielack, Stefan S.; Fuchs, Joerg; Niggli, Felix; Hettmer, Simone; Klingebiel, Thomas; Sparber‐Sauer, Monika
• Is Part Of: Journal of surgical oncology. Volume 122:Issue 2(2020)
• Page Start: 263
• Page End: 272
• Publication Date: 2020-05-19
• Subjects: children and adolescents -- CWS Group -- dermatofibrosarcoma protuberans -- fibrosarcomatous transformation -- surgery; Cancer -- Surgery -- Periodicals; Neoplasms -- Periodicals; 616
• DOI: 10.1002/jso.25943
• Languages: English
• ISSNs: 0022-4790

Abstract

Abstract: Background: Dermatofibrosarcoma protuberans (DFSP) is a rare low‐grade tumor. Little is known about best treatment of primary and relapsed disease (RD). Methods: Treatment and outcome of 40 patients with DFSP prospectively registered within the CWS‐96 and ‐2002P trials and the registry SoTiSaR (1996‐2016) were analysed. Results: Median age was 8 years (range, 0.64‐17.77). Fluorescence in situ hybridization analysis to detect COL1A1‐PDGFB fusion genes was positive in 86% (12/14) of evaluated patients. Primary resection was performed in all patients. Patients had IRS group I ( n = 28), II ( n = 9), and III ( n = 2); not available ( n = 1). To achieve complete remission (CR), a secondary resection was performed in 18 patients resulting in microscopically complete (R0, n = 34/40) and microscopically incomplete (R1, n = 5/40) resection. All patients achieved CR. The 5‐year event‐free survival (EFS) and overall survival was 86% (±12; CI, 95%) and 100% (±0; CI, 95%), respectively. R0 resection/IRS I was significantly favorable for the 5‐year EFS. Local relapse occurred after a median time of 1.1 years (range, 0.04‐5.1) in 15% (6/40) after CR. All patients with RD underwent resection and achieved CR. Three patients had fibrosarcomatous DFSP, two were alive after R0 resection. Conclusion: Complete surgical resection is mandatory to prevent relapse of DFSP.