Retroperitoneal sarcoma - the continued challenge for surgery and oncology

Retroperitoneal sarcomas (RS) are rare malignant tumours with an incidence rate of about 1-2 cases per million per year. Therefore only a few centres are able to acquire more experience in this field. Tumours are usually of l arge size, due to slow growth and uncommon symptoms. Different histologic t ypes, grades and rare incidence make any comparison difficult. Radical exci sion including adjacent organs, called "en-block" resection, is the treatme nt of choice, however it is very often difficult to obtain adequate free ma rgins around the tumour. Complete tumour excision remains a challenge even for an experienced surgeon. In the published series, resectability ranges f rom 38 to 100% with radicality rate between 8 and 95%. Local recurrence is very common (33-86%), with rare distant metastases (max. 33%), so local fai lure is usually the cause of death. It is well known that histological grad ing and completeness of surgery determine the chance of survival. Five-year survival rates after radical excision ranged from 62-92% in well-different iated tumours, compared with 16-48% in nondifferentiated sarcomas. There is no evidence that adjuvant or neoadjuvant treatment affects the prognosis. Only the development of an international registry of retroperitoneal sarcom a and co-operative intergroup studies can help in evaluating treatment and in applying innovative multimodal therapies to these neoplasms. (C) 1999 El sevier Science Ltd. All rights reserved.