Key result
Grade 3 myxofibrosarcoma linked to worse 5-year overall survival of ~73% compared to lower grades.
Why the study?
The study was conducted to understand the natural history of myxofibrosarcoma and determine whether prognosis is influenced by histologic grade.
Does histologic grade predict survival and local recurrence in adult patients with primary myxofibrosarcoma of the limbs?
Cohort (n=229)
Does histologic grade predict survival and local recurrence in adult patients with primary myxofibrosarcoma of the limbs?
Histologic grade 3 in myxofibrosarcoma of the extremities is associated with worse overall survival compared to lower grades, while grade 1 has the lowest risk of local recurrence.
Answers are grounded in this paper and cite their sources.
May inform prognostic counseling in limb myxofibrosarcoma; leaves open impact on treatment decisions.
BACKGROUND: The aim of this retrospective analysis is to understand the natural history of myxofibrosarcoma (MFS), in particular whether the prognosis can be influenced by histologic grade. METHODS: We reviewed 229 adult patients with primary MFS of the limbs. We analyzed disease-specific survival (overall survival [OS]) and local recurrence (LR). RESULTS: Median age was 70 years (range, 19-92). Sixteen (7.0%) were grade 1, 38 (16.6%) grade 2, and 175 (76.4%) grade 3. A worse OS was found in grade 3 MFS (73.1%) than in grade 2 and 1 MFS (91.9 and 100%, respectively) at 5 years (p = 0.031). Locally recurred MFS had a worse OS (p = 0.018). A better LR-free rate (100% at 5 years) was observed in grade 1 MFS; however, a similar rate was observed between grade 2 and 3 tumors (77.1 and 80.0% at 5 years, respectively, p = 0.412). CONCLUSIONS: Grade 3 MFS has the worst prognosis. Grade 1 MFS have the lowest risk of LR. These data could help identify a high-risk patient group, thus selecting a more careful follow-up for higher-risk patients. Since MFS mostly affects the elderly population, it might be useful to reserve adjuvant treatments (radiotherapy and chemotherapy) to higher-risk patients.
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Sambri et al. (2020) conducted a cohort in Primary myxofibrosarcoma (MFS) of the limbs (n=229). Histologic grade was evaluated on Disease-specific survival (overall survival [OS]) (p=0.031). Grade 3 myxofibrosarcoma was associated with worse 5-year overall survival (73.1%) compared to grade 2 (91.9%) and grade 1 (100%) tumors (p=0.031).
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