The retroperitoneum contains the kidneys, adrenal glands, ureters, aorta, inferior vena cava, vessel tributaries, lymph nodes, nerve plexuses and autonomic ganglia. Due to its inaccessible anatomical location tumours can attain a considerable size before clinical presentation with vague symptomatology or because of pressure effects on adjacent structures, e.g. ureter. Investigation is by CT scan supplemented by ultrasound and MRI as appropriate. Arteriography may be used if resection of a large tumour is planned. Tissue diagnosis is by percutaneous CT-guided needle core biopsy or FNA. The commonest malignancy by far is periaortic lym-phadenopathy due to nodal malignant lymphoma (diffuse large B-cell or follicular lymphoma) or metastatic disease (testicular germ cell tumour, gut, prostate, renal, pancreatic or gynaecological cancer). The need for a tissue diagnosis is determined by the availability of previous data, the nature and stage of the disease process at which the lymphadenopathy has arisen and any further planned therapeutic management, e.g. frozen section as a prequel to radical gynaecological resection, needle core biopsy to establish a diagnosis of malignant lymphoma, or imaging with serum tumour markers to indicate surgery or radio-/chemotherapy in a patient with known previous testicular germ cell tumour. Kidney, germ cell and adrenal gland tumours are dealt with in their respective sections but primary tumours include:
Liposarcoma: especially well-differentiated and sclerosing subtypes. May contain a dedifferentiated element. Leiomyosarcoma: arising from the wall of the inferior vena cava or its tributaries and prone to cystic change when large. Tumour necrosis and size >10 cm are strong pointers to malignancy even if of low mitotic rate.
Malignant fibrous histiocytoma: exclude this pattern as part of other sarcomas and also sarcomatoid renal carcinoma. Peripheral nerve tumours: neurilemmoma, neurofibroma, malignant peripheral nerve sheath tumours, paraganglioma, ganglioneuroma, neuroblastoma.
Ewing's sarcoma/PNET, rhabdomyosarcoma, desmoplastic small round cell tumour: usually in children and young adults and initial chemotherapy is more appropriate, but may be supplemented by surgery which is the main modality in the other sarcoma types.
Resection specimens can be large and complex with structures such as kidney enveloped by tumour. For TNM staging purposes retroperitoneum is a deep structure. Due to the late presentation and difficulties in obtaining complete excision, prognosis for soft tissue sarcomas is poor with an overall 5-year survival of about 25%.
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