Paratesticular liposarcoma: Unusual patterns of recurrence and importance of margins

摘要

Background: Paratesticular liposarcoma (LPS) is a rare entity for which optimal treatment has not been defined. We sought to determine recurrence patterns and prognostic factors. Methods: A total of 25 patients with localized paratesticular LPS between 1987 and 2009 were reviewed. Actuarial local-recurrence-free survival (LRFS), disease-free-survival (DFS), and overall survival (OS) were determined using the Kaplan-Meier method. Results: LPS histology was well differentiated for 10 patients (40 %), de-differentiated for 14 (56 %), and pleomorphic for 1 (4 %). Final margins were positive in 8 patients (32 %). Radiation therapy (RT) was given to 10 patients; fields included inguinal canal ± scrotum and low pelvis. LRFS rates at 3 and 5 years were 76 and 67 %. The 3-year LRFS rates were lower in patients with positive margins compared with those with negative margins (29 vs 100 %, p =.0005) and in patients with recurrent versus primary disease (38 vs 83 %, p =.04). Among patients who received surgery and RT, margins remained a significant predictor of local recurrence (p =.009). Interestingly, recurrences in 4 patients tracked along gonadal vessels, and only 1 patient had a distant recurrence. OS at 5 years was 100 %. Conclusions: For patients with localized paratesticular LPS, positive margins and presentation with recurrent disease are adverse prognostic factors for LRFS. LR for patients with positive margins is still high despite RT; thus aggressive surgery to attain negative margins should be attempted in all cases. The finding of regional recurrences along gonadal vessels should be validated, and imaging studies should be tailored to reflect potential patterns of disease at presentation and subsequent recurrence.