Retroperitoneal sarcoma

Retroperitoneal sarcoma

Retroperitoneal sarcomas are rare tumours accounting for only 1–2% of all solid malignancies (10–20% of all sarcomas are retroperitoneal). The peak incidence is in the fifth decade of life, although they can occur at almost any age. The most frequently encountered cell types are: /uni25CF liposarcoma; /uni25CF leiomyosarcoma; /uni25CF malignant fibrous histiocytoma. History and examination Patients with sarcomas present late because these tumours arise in the large potential spaces of the retroperitoneum and can grow to a considerable size without producing symptoms. Moreover, when symptoms do occur, they are non-specific, such as abdominal pain and fullness, and are easily dismissed as being caused by other less serious processes. Retroperitoneal sarcomas are therefore often very large at the time of presentation. Investigation Detailed multiplanar imaging (CT and MRI) with reconstruc tions is required not only for tumour detection, staging and surgical planning but also for guiding percutaneous or surgical biopsy of these tumours. Treatment The definitive treatment of primary retroperitoneal sarcomas is surgical resection. Chemotherapy and radiotherapy without surgical debulking have rarely been beneficial, when used alone or in combination. A multidisciplinary treatment approach with imaging review will be required when assessing operabil ity (based on adjacency or involvement of vital structures) and approach. Up to 75% of retroperitoneal sarcoma resections visceral organ (commonly large or small bowel or kidney). The most common types of v ascular involvement precluding resection are involvement of the proximal superior mesenteric vessels or involvement of bilateral renal vessels. Prognosis Survival rates are in general poor, even after complete resec - tion, being of the order of 35–50% (excluding low-grade liposarcomas, which may frequently be cured by resection).