Soft‐tissue Sarcoma in Adults

Soft-tissue Sarcomas (STS) are a rare and heterogeneous group of tumors, arising in connective tissues embryologically derived from the mesenchyme. There are a dozens of subtypes arising from cartilage, muscle, blood vessels, nerves, and fat.¹ Sarcomas make up <1% of all neoplasms, which often results in a delay in diagnosis.² Sarcomas are best managed at specialist referral centers, and suspected cases are recommended to be referred promptly, allowing for further investigation and review by a Multidisciplinary Team (MDT). Surgery remains the mainstay of treatment, supported by advancing multimodal therapies including chemotherapy and radiotherapy. This review discusses the diagnosis and management of sarcomas, along with the clinical challenges faced in orthopedic oncology.

Pathology of Soft-tissue sarcoma in adults

According to the fourth edition of the World Health Organization (WHO) Classification of Tumours of Soft Tissue and Bone, there are more than 100 different histologic subtypes of soft‐tissue tumors, the majority of which are STS, each with unique prognostic, clinical, and therapeutic features.When considering all adult STS, the most common histiotypes include liposarcoma, leiomyosarcoma, and Undifferentiated Pleomorphic Sarcoma (UPS). The usual approach to soft‐tissue tumor classification is by presumed cell lineage and is based on morphologic, immunohistochemical, and genetic features. Currently, tumors are categorized as adipocytic, fibroblastic or myofibroblastic, so‐called fibrohistiocytic, smooth muscle, pericytic, skeletal muscle, vascular, chondro‐osseous, and an “uncertain differentiation” category. Aside from tumor site and grade, the histologic subtype of STS is a major prognostic indicator.¹³ Because of the rarity of each histiotype, however, histology‐specific analyses have been difficult to perform in both retrospective and prospective studies, and much of the available data regarding optimal therapeutic strategies have used mixed histiotypes.

Truncal and Extremity Soft-tissue Sarcoma in Adults

Approximately 40% to 50% of STS occur in the extremities, and approximately 13% occur in the trunk. Recent series indicate that the most common histiotypes in the trunk or extremity include UPS, leiomyosarcoma, lip sarcoma, and synovial sarcoma. However, any of the nearly 100 histiotypes can develop within the trunk or extremity. The prognostic factors for DSS in patients who have truncal and extremity STS include tumour grade, size, and histologic subtype. Some studies have also found that deep location, positive margins, and lower extremity site are significantly associated with long‐term outcomes.Depending on the exact histiotype and its respective malignant potential, there is variation in the natural history of the STS, ranging from an increased risk of locoregional recurrence to a substantial risk of distant failure. However, with high‐grade tumour accounting for nearly 75% of truncal and extremity STS, the main pattern of failure is usually distant metastases because local recurrences can be managed with limb‐sparing reresection or, in rare circumstances, amputation.

Regards

Amalia Azzariti

Managing Editor

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Journal of Clinical Oncology and Cancer Research