Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma

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Soft tissue sarcoma (STS) describes a rare group of malignancies of mesenchymal origin. There are approximately 60 subtypes of STS1 with wide variation in natural history and prognosis.
Approximately 15% of STS arise in the retroperitoneum, with an overall incidence of 0.5–1 per 100 000.2 The most common subtypes of retroperitoneal sarcoma (RPS) are liposarcoma (63%) and leiomyosarcoma (19%),3 and less common subtypes include synovial sarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour and undifferentiated pleomorphic sarcoma.3 Liposarcoma is further subdivided into well‐differentiated, dedifferentiated, myxoid, round cell and pleomorphic subtypes. The anatomical complexity of the retroperitoneum and proximity of the tumour to vital structures limit the ability to achieve a wide margin as is performed for patients with STS in the extremity. The large size, complex anatomical relationships and tumour growth patterns account for a significant risk of intra‐abdominal recurrence. The anatomical and pathological complexity of RPS requires individualized multidisciplinary management, which will be discussed below.

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