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Dermatofibrosarcoma protuberans in children and adolescents: Primary and Relapsed disease—Experience of the Cooperative Weichteilsarkomstudiengruppe (CWS)
Author(s) -
Krewer Julia,
Rolle Udo,
Koscielniak Ewa,
Vokuhl Christian,
Mentzel Thomas,
Seitz Guido,
Feuchtgruber Simone,
Kalle Thekla,
Scheer Monika,
Münter Marc,
Bielack Stefan S.,
Fuchs Joerg,
Niggli Felix,
Hettmer Simone,
Klingebiel Thomas,
SparberSauer Monika
Publication year - 2020
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.25943
Subject(s) - dermatofibrosarcoma protuberans , medicine , surgery , dermatofibrosarcoma , pdgfb , resection , surgical resection , overall survival , young adult , pathology , platelet derived growth factor receptor , receptor , growth factor
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.