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. 2020 Aug;122(2):263-272.
doi: 10.1002/jso.25943. Epub 2020 May 19.

Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease-Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

Julia Krewer  1 , Udo Rolle  1 , Ewa Koscielniak  2   3 , Christian Vokuhl  4 , Thomas Mentzel  5 , Guido Seitz  6 , Simone Feuchtgruber  2 , Thekla von Kalle  7 , Monika Scheer  2 , Marc Münter  8 , Stefan S Bielack  2   9 , Joerg Fuchs  10 , Felix Niggli  11 , Simone Hettmer  12 , Thomas Klingebiel  13 , Monika Sparber-Sauer  2 ; CWS Study Group
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Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease-Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)

Julia Krewer et al. J Surg Oncol. 2020 Aug.

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.

Keywords: CWS Group; children and adolescents; dermatofibrosarcoma protuberans; fibrosarcomatous transformation; surgery.

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References

REFERENCES

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