Recurrent retroperitoneal sarcomas: Clinical outcomes of surgical treatment and prognostic factors.

BACKGROUND AND PURPOSE: Locoregional recurrence after resection of primary retroperitoneal sarcoma (RPS) is a challenging therapeutic issue. The objective of this study was to identify clinicopathological factors predictive of overall survival (OS) and disease specific survival (DSS) after reoperation for recurrent RPS. PATIENTS AND METHODS: We retrospectively collected data from the medical records of 800 patients who underwent resection for sarcoma at our Institution, from 1983 to 2015. Among these patients, 120 were treated for retroperitoneal sarcoma and 55 had a locoregional recurrence (LR). Four of them did not undergo surgery and thus were excluded from this study leaving 51 cases available for data analysis. Univariate and multivariate survival analyses were performed to identify prognostic factors. RESULTS: Median overall survival was 33 months. The 1-year, 3-year and 5-year OS rates were 75.5%, 47.1% and 31.6% respectively. Multivariate Cox regression analysis suggested that extension of surgery (P = 0.026), surgical margin status (P = 0.015) and histological grade of recurrent tumor (P = 0.047) were independent prognostic factors for OS. Median DSS was 48 months. The 1-year, 3-year and 5-year DSS rates were 79.2%, 53.1% and 40.9%, respectively. At multivariate analysis, predictors of DSS were extension of surgery (P = 0.004), margin status (P = 0.011), histological grade of recurrent tumor (P = 0.008), and disease free interval (DFI) (P = 0.020). As regards histological subtype of recurrent RPS, at univariate analysis, well-differentiated liposarcoma (WDLS) was associated with better OS and DSS (P = 0.052 and P = 0.016 respectively) compared to dedifferentiated liposarcoma (DDLS). CONCLUSIONS: According to our findings, surgery is more beneficial in patients with low-grade sarcoma, WDLS and long DFI. The achievement of clear resection margins, rather than performing a multivisceral resection, appears to be a key factor to improve OS and DSS.

Myoepithelioma of the soft tissue: A systematic review of clinical reports.

BACKGROUND: Myoepithelioma of the soft tissue (MES) is a rare neoplasm, and available literature includes few studies with small sample sizes, thus limiting the comprehension of this disease. Hence, a systematic review was performed to summarize the available information on MES patients evaluated for surgical treatment. METHODS: The MEDLINE/PubMed, EMBASE and SCOPUS databases were reviewed for eligible studies in January 2019. Inclusion criteria were: patients with MES; indication for wide resection or amputation; observational studies; articles published from 1997 to 2018; English language; reporting of outcome measures such as overall survival (OS), disease-free survival (DFS), recurrence, metastases. Studies not including humans were excluded. Quality appraisal was performed using the Methodological Index for Non-Randomized Studies (MINORS) tool. A narrative synthesis of included studies was conducted, a formal meta-analysis being unfeasible. RESULTS: Overall, 10 eligible studies including 233 MES patients were identified. The most frequent tumor sites were the limbs and trunk. Most patients underwent surgical treatment, with R0 resection margin rates ranging from 24% to 78%. During follow-up, local recurrence rates ranged from 17% to 50, and distant metastasis rates varied 8%-48%. At 5 years, OS rates ranged from 8% to 94% and DFS rates from 11% to 40%. The average MINORS score was 9.1 (range: 6-11). CONCLUSIONS: Surgical resection was often sub-optimal. The long-term prognosis was poor, with a non-negligible rate of disease recurrence and metastasis. On average, the quality of available information is moderate. The centralization of patient information in large international registries is warranted to provide a better understanding of MES biology and ultimately improve patient outcomes.