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Treatment of low-grade fibromyxoid sarcoma
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
Context: Treatment of Fibroblastic and Myofibroblastic Tumors / Low-Grade Fibromyxoid Sarcoma
Treatment options for low-grade fibromyxoid sarcoma include the following:
Surgery.
Low-grade fibromyxoid sarcoma is not very chemosensitive, and the limited treatment information suggests that surgery is the treatment of choice.[109]
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Evidence (surgery):
The German Cooperative Weichteilsarkom Studiengruppe (CWS) reported study results for 31 patients younger than 21 years with low-grade fibromyxoid sarcoma.[104][Level of evidence C2]
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The German Cooperative Weichteilsarkom Studiengruppe (CWS) reported study results for 31 patients younger than 21 years with low-grade fibromyxoid sarcoma.[104][Level of evidence C2]
The 5-year EFS rate was 71% (95% CI, ±18.6%), the 5-year local relapse-free survival rate was 76% (95% CI, ±17.6%), and the 5-year OS rate was 100%.
The German Cooperative Weichteilsarkom Studiengruppe (CWS) reported study results for 31 patients younger than 21 years with low-grade fibromyxoid sarcoma.[104][Level of evidence C2]
Among 24 patients who had R0 resections (complete resection with negative microscopic margins), 5 patients (21%) experienced disease relapses (3 local, 1 metastatic, and 1 combined).
The German Cooperative Weichteilsarkom Studiengruppe (CWS) reported study results for 31 patients younger than 21 years with low-grade fibromyxoid sarcoma.[104][Level of evidence C2]
Among seven patients who had R1 resections (margins were microscopically positive), three patients (43%) experienced local disease relapses.
There are little data regarding the use of chemotherapy and/or radiation therapy in this disease. One report suggests that trabectedin may be effective in the treatment of low-grade fibromyxoid sarcoma.[112]
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Preserved source evidence · Not medical advice