Should High-grade XXXX Be Treated With Multimodality Therapy Like Other Soft Tissue Sarcomas?

@entity1 @entity632 @entity5 @entity79 @entity421 @entity1260 @entity204
BACKGROUND: @entity421 is rare, and the most appropriate therapy is unclear because there are few studies regarding its treatment. The effectiveness of radiation and chemotherapy remains uncertain owing to conflicting results in previous reports. QUESTIONS/PURPOSES: To review our experience with contemporary multimodality treatment, we asked: (1) What is the disease-specific survival and local relapse-free survival? (2) Does American Joint Commission on Cancer (AJCC) stage, @entity5 size, or location relate to disease outcome? (3) Does radiation therapy improve local control or survival? (4) Do @entity632 and @entity1260 improve local control or survival? METHODS: Between 1990 and 2012, we treated 40 @entity1 for localized, high-grade @entity421 . In this retrospective study, we could determine the status of 36 @entity1 (90%) either to @entity204 or for a minimum of 24 months of followup; four (10%) were lost to followup before 24 months. There were 11 @entity1 with AJCC Stage IIA and 25 with @entity5 . All @entity1 underwent wide surgical excision. Of the @entity1 with @entity5 , four received radiation and none received chemotherapy. Of the @entity1 with @entity5 , six received radiation, seven were treated with chemotherapy, and six received radiation and chemotherapy. During the study period, high-dose @entity632 and @entity1260 was the preferred chemotherapy regimen for @entity1 younger than 60 years with normal cardiac and renal function. Local relapse-free survival and disease-specific survival were determined by Kaplan-Meier analysis using a prospectively maintained institutional database supplemented by information from the institutional @entity5 registry. The Cox proportional hazard model was used to determine the effect of various factors on local recurrence and @entity1 survival. RESULTS: At 5 years, local relapse-free survival was 47% (95% CI, 27%-64%), and disease-specific survival was 53% (95% CI, 35%-68%). In multivariate analysis, AJCC stage, which depends on @entity5 size, was the strongest predictor of local relapse-free survival (hazard ratio [HR] = 0.17, p = 0.02), while @entity5 depth was the best predictor of disease-specific survival (HR = 5.6, p = 0.02). Radiation improved local relapse-free survival (HR = 0.30, p = 0.03) but not disease-specific survival in multivariate analysis. A regimen of @entity632 and @entity1260 was associated with better local relapse-free survival for @entity1 with @entity5 (HR = 0.16, p = 0.04) but not disease-specific survival (HR = 0.32, p = 0.08). CONCLUSIONS: With the limited number of @entity1 in our study, it appears that @entity421 behaves differently than @entity79 . Multimodality treatment that includes @entity632 and @entity1260 -based chemotherapy, radiation, and surgery may be a valid therapeutic strategy for @entity5 , but larger, prospective studies will be needed to verify our preliminary observations. LEVEL OF EVIDENCE: Level III, therapeutic study.
