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Surgery with or without radiation therapy
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Most patients with dermatofibrosarcoma tumors can be cured by complete surgical resection. Wide excision with negative margins or Mohs/modified-Mohs surgery will prevent most tumors from recurring.[44] Despite the locally aggressive behavior of the tumor, lymph node or visceral metastasis rarely occurs.
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Evidence (surgery):
The EpSSG prospective NRSTS 2005 (NCT00334854) trial identified 46 patients with dermatofibrosarcoma protuberans.[45] The median age at diagnosis was 6.9 years (range, 0.4–17.5 years). All patients had localized disease, 93% of patients had small tumors (<5 cm), and 76% of patients had Intergroup Rhabdomyosarcoma Study (IRS) group I tumors.
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The EpSSG prospective NRSTS 2005 (NCT00334854) trial identified 46 patients with dermatofibrosarcoma protuberans.[45] The median age at diagnosis was 6.9 years (range, 0.4–17.5 years). All patients had localized disease, 93% of patients had small tumors (<5 cm), and 76% of patients had Intergroup Rhabdomyosarcoma Study (IRS) group I tumors.
All patients underwent up-front surgery, and 32 patients required two procedures.
The EpSSG prospective NRSTS 2005 (NCT00334854) trial identified 46 patients with dermatofibrosarcoma protuberans.[45] The median age at diagnosis was 6.9 years (range, 0.4–17.5 years). All patients had localized disease, 93% of patients had small tumors (<5 cm), and 76% of patients had Intergroup Rhabdomyosarcoma Study (IRS) group I tumors.
There were 11 patients with IRS group II tumors, 2 of whom went on to have a local recurrence.
The EpSSG prospective NRSTS 2005 (NCT00334854) trial identified 46 patients with dermatofibrosarcoma protuberans.[45] The median age at diagnosis was 6.9 years (range, 0.4–17.5 years). All patients had localized disease, 93% of patients had small tumors (<5 cm), and 76% of patients had Intergroup Rhabdomyosarcoma Study (IRS) group I tumors.
After a median follow-up of 49 months (range, 4.2–130.9 months), all patients were alive at the time of this report.
The EpSSG prospective NRSTS 2005 (NCT00334854) trial identified 46 patients with dermatofibrosarcoma protuberans.[45] The median age at diagnosis was 6.9 years (range, 0.4–17.5 years). All patients had localized disease, 93% of patients had small tumors (<5 cm), and 76% of patients had Intergroup Rhabdomyosarcoma Study (IRS) group I tumors.
The 5-year EFS rate was 92.6% (95% CI, 78.8%–97.6%), and the OS rate was 100%.
In retrospective reviews, postoperative radiation therapy after incomplete excision may have decreased the likelihood of recurrence.[46,47] Metastatic disease is more likely after multiple recurrences, and radiation or other adjuvant therapy should be considered in patients with recurrences that cannot be managed surgically.[40,42]
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Preserved source evidence · Not medical advice