Long-term prognosis of primary retroperitoneal soft tissue sarcoma,☆☆

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Abstract

Aims

To evaluate the result of treatment and long-term outcome of a population-based cohort of patients with retroperitoneal soft tissue sarcoma (RSTS).

Methods

Between 1 January 1989 and 1 January 1994, 143 patients diagnosed as having primary RSTS were selected from a national pathology database (PALGA) in the Netherlands. In this population-based group of patients, the result of surgery, overall survival (OS) and disease-free survival (DFS) were analysed as well as factors affecting OS and DFS. Median follow-up was 10.2 years.

Results

Operative treatment resulted in a complete tumour resection in 55% of the patients (n = 78), low- and intermediate-grade tumours were more often completely resected than high-grade tumours (P = 0.016). Five- and 10-year cumulative OS was 39% and 21%, respectively, while DFS was 22% and 17%, respectively. In a multivariate analysis low malignancy grade (P = 0.017) and a complete tumour resection (P < 0.001) were associated with better OS.

Conclusions

Complete tumour resection and low malignancy grade were independent favourable prognosticators. However, these factors were related too, since surgical success was influenced by malignancy grade.

Introduction

Treatment of retroperitoneal soft tissue sarcomas (RSTS) is difficult. Adequate tumour-free margins are not easily obtained and locoregional recurrence is common following surgery for retroperitoneal soft tissue sarcomas (RSTS).1 To improve locoregional control, various authors advocate extensive surgery,2, 3 or suggest the (neo-) adjuvant use of chemo- and radiotherapy.4, 5, 6

Data on outcome of patients with RSTS and the efficacy of the proposed aggressive treatment strategies are difficult to interpret. RSTS are rare tumours, controlled data are scarce and mainly come from tertiary referral centres, and follow-up is usually limited.

In a population-based study in the Netherlands we aimed to elucidate the “natural” course of the disease by evaluating the long-term effect of various clinicopathological factors on the result of surgery and outcome.

Section snippets

Data collection

Information about patients was obtained with help of the Dutch Network and National Database for Pathology (PALGA). All patients that were registered in the Netherlands between 1989 and 1994 as having a newly diagnosed primary RSTS were selected by this registry (n = 301). In 1995, a questionnaire about the histopathology of the tumour (histological type, malignancy grade) was sent to the involved pathology laboratories (n = 58). The questionnaire was accompanied by a request for the original

Clinicopathological data

There were 79 females (55%) and 64 males, with a median age of 60 (range 18–88) years. The distribution of the clinicopathological factors is shown in Table 1. Liposarcomas and leiomyosarcomas prevailed and together comprised two-thirds of all tumours. Other histomorphological sarcoma types were sarcoma no otherwise specified (n = 18), malignant fibrous histiocytoma (n = 10), malignant peripheral nerve sheat tumour (n = 5), fibrosarcoma (n = 4). The remaining 10 patients had other sarcomas, and four of

Discussion

In the present population-based study in patients with retroperitoneal soft tissue sarcoma overall survival declined throughout the 10 year observation period. After 10 years, only 20% of the patients were alive and one fifth of the latter patients had recurrent disease. Malignancy grade had an impact on overall survival as well as on the success of surgical treatment.

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Presented to a meeting of the British Association of Surgical Oncology, London, UK, November 2004 and published in abstract form as Eur J Surg Oncol 2004;30:1016.

☆☆

No financial support was received for this study.

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