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Multicenter Study
. 2024 Jun;129(7):1354-1363.
doi: 10.1002/jso.27620. Epub 2024 Apr 1.

Natural history of undifferentiated pleomorphic sarcoma: Experience from the US Sarcoma Collaborative

Eleftherios A Makris  1 , Thuy B Tran  1 , Daniel J Delitto  1 , Byrne Lee  1 , Cecilia G Ethun  2 , Valerie Grignol  3 , J Harrison Howard  3 , Meena Bedi  4 , T Clark Gamblin  4 , Jennifer Tseng  5 , Kevin K Roggin  5 , Konstantinos Chouliaras  6 , Konstantinos Votanopoulos  6 , Darren Cullinan  7 , Ryan C Fields  7 , Kenneth Cardona  2 , George Poultsides  1 , Amanda Kirane  1
Affiliations
Multicenter Study

Natural history of undifferentiated pleomorphic sarcoma: Experience from the US Sarcoma Collaborative

Eleftherios A Makris et al. J Surg Oncol. 2024 Jun.

Abstract

Background: Undifferentiated pleomorphic sarcoma (UPS) is a relatively rare but aggressive neoplasm. We sought to utilize a multi-institutional US cohort of sarcoma patients to examine predictors of survival and recurrence patterns after resection of UPS.

Methods: From 2000 to 2016, patients with primary UPS undergoing curative-intent surgical resection at seven academic institutions were retrospectively reviewed. Epidemiologic and clinicopathologic factors were reviewed by site of origin. Overall survival (OS), recurrence-free survival (RFS), time-to-locoregional (TTLR), time-to-distant recurrence (TTDR), and patterns of recurrence were analyzed.

Results: Of the 534 UPS patients identified, 53% were female, with a median age of 60 and median tumor size of 8.5 cm. The median OS, RFS, TTLR, and TTDR for the entire cohort were 109, 49, 86, and 46 months, respectively. There were no differences in these survival outcomes between extremity and truncal UPS. Compared with truncal, extremity UPS were more commonly amenable to R0 resection (87% vs. 75%, p = 0.017) and less commonly associated with lymph node metastasis (1% vs. 6%, p = 0.031). R0 resection and radiation treatment, but not site of origin (extremity vs. trunk) were independent predictors of OS and RFS. TTLR recurrence was shorter for UPS resected with a positive margin and for tumors not treated with radiation.

Conclusion: For patients with resected extremity and truncal UPS, tumor size >5 cm and positive resection margin are associated with worse survival OS and RFS, irrespectively the site of origin. R0 surgical resection and radiation treatment may help improve these survival outcomes.

Keywords: sarcoma; soft tissue sarcoma; undifferentiated pleomorphic sarcoma.

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