Diagnosis and treatment of primary soft tissue sarcomas: comprehensive review

Soft tissue sarcomas (STSs) comprise a group of rare malignancies with anatomic- and histologic-specific patterns of local and distant recurrence. Due to their rarity and histology-specific tumour behaviour, their natural history and the efficacy of various interventions may be challenging to assess. The aim of this review is thus to provide a comprehensive overview of diagnostic and treatment options for localized extremity and retroperitoneal STSs.

A literature search was conducted to identify articles related to the diagnosis and management of localized extremity and retroperitoneal STSs. English-language articles published until June 2025 were identified using Medical Subject Heading terms on PubMed. Results were reported using a narrative approach. Topics highlighted in this review include diagnosis, institutional volumes, treatment options, and multimodal management of STSs based on location.

Accurate diagnosis of STS relies on carefully planned preoperative biopsies and in selected cases is supplemented by advanced molecular diagnostic tools. Surgery remains the cornerstone of curative-intent treatment for localized STSs; however, resectability criteria for retroperitoneal STSs vary by institution. Institutional case volumes are prognostic of outcome, with 10-20 retroperitoneal sarcoma cases per year considered by experts to be indicative of high-volume sarcoma centres. The role of adjunctive therapies, including chemotherapy, radiation, and/or other locoregional treatments, is dictated by histological and molecular characteristics associated with local and distant recurrence rates.

The management of localized STSs is multidisciplinary in nature, requiring consideration of tumour and patient characteristics, and treatment factors. The rarity of STSs and the variable biological behaviour of the various histologic subtypes have impacted research in this field. Ongoing international collaborations and innovative study designs are essential for advancing the understanding of tumour behaviour and in optimizing treatment approaches.