Head and Neck Tumors: Management of Primary Undifferentiated Pleomorphic Sarcoma

Iran J Otorhinolaryngol. 2019 Nov;31(107):335-342. doi: 10.22038/ijorl.2019.30195.1990.

Abstract

Introduction: Sarcomas account for 1% of all tumors originated in the head and neck regions in adults. They constitute a heterogeneous group of tumors of mesenchymal origin with multiple histological variants. The undifferentiated pleomorphic sarcoma (UPS) subtype is the most frequent variant with an incidence range of 2.7-38%.

Materials and methods: This retrospective case series was conducted on 11 patients who were diagnosed with UPS and treated in our institution in the last 11 years.

Results: According to the results, 26.8% of the sarcomas corresponded to UPS variant. Out of 11 patients, 5 cases were female. The median age of the participants was 58 years (range: 18-74 years). Seven and four patients were T1b and T2b, respectively. Ten patients had no metastasis at the moment of diagnosis. Six patients were categorized as intermediate-grade, and the remaining (n=5) were classified as high-grade. The most frequent location was the scalp (n=3). In the patients undergoing surgery, the mean follow-up duration was 52 months. Disease-free survival (DFS) rates were obtained at 70%, 50%, and 50% in the 1st, 3rd, and 5th years, respectively, with the mean DFS of 36 months. In addition, the overall survival rates in the 1st, 3rd, and 5th years were 100%, 100%, and 83%, respectively.

Conclusion: The UPS represented the most common histological subtype in our series. They tend to be intermediate or high-grade tumors. An acceptable global survival rate justifies surgical treatment as the main therapeutic tool.

Keywords: Head; Neck; Neoplasms; Sarcomas; Undifferentiated pleomorphic sarcomas.