Tumours of the retroperitoneum and management of retroperitoneal sarcomas

AbstractRetroperitoneal sarcomas (RPS) are rare tumours representing less than 0.3 per cent of all adult malignancies. Although rare, a knowledge of their presentation, workup and principles of management is important for primary care clinicians, radiologists and general surgeons, as early diagnosis and treatment is critical to the successful management of these complex and aggressive malignancies. Primary retroperitoneal tumours (RPT) arise separate from the organs of the retroperitoneum i.e. the kidneys, adrenals, pancreas and retroperitoneal colon. They may be benign or malignant and represent a wide range of pathologies with sarcomas accounting for one third of the total number. Retroperitoneal sarcomas (RPS) usually occur sporadically with few described risk factors. Histopathologically, these tumours arise from mesenchymal derived tissues with over 70 different histological subtypes though 50 per cent arise from the three commonest subtypes: liposarcoma, leiomyosarcoma, and undifferentiated pleomorphic sarcoma. The clinical presentation is usually with a large abdominal mass or as an incidental finding on radiology for other conditions. Diagnostic workup includes a contrast CT scan and often a core biopsy if a liposarcoma is not confidently diagnosed on radiological features. Multidisciplinary discussion is critical to the management of these rare tumours and, though there is a role for radiotherapy and chemotherapy, complete surgical resection is the key to successful management. However, many patients with RPS will present late with incurable disease, or recur either locally or systemically after surgery, and therefore good palliative care and support systems are important in providing holistic care for these patients.