Prognostic Factors and Metastatic Patterns in Primary Myxoid/Round-Cell Liposarcoma

J Haniball,V P Sumathi,S R Carter,R J Grimer,D Spooner,R M Tillman,L.-G Kindblom,D Peake,A Abudu,L Jeys

doi:10.1155/2011/538085

Abstract

Background. This study aimed to investigate prognostic factors for patients with myxoid/round-cell liposarcoma (MRCLS), in particular the significance of the round cell component, and to identify metastatic patterns as well as possibly suggest a suitable strategy for followup. Methods. Clinical, morphologic, and follow-up data from 160 patients with MRCLS was reviewed and statistically analysed. Results. Of 130 tumours with the round cell component evaluated, 61 had no round cell component, 27 had <5% round cell component, and 42 had >5%. All patients underwent surgical excision, 15 requiring amputation. 107 patients received adjuvant radiotherapy. Local recurrence occurred in 19 patients (12%), predominantly in patients with marginal or intralesional margins and a round cell component. Overall disease specific survival was 75% at 5 years and 56% at 10 years and was related to the proportion of round cell component. Of 52 patients who developed metastases, 38 (73%) had purely extrapulmonary metastases. We could not identify any factors predicting the site of metastases developing. Conclusions. The occurrence of any round cell component is the most important adverse prognostic factor for patients with MRCLS; patients with >5% round cell component are at higher risk of local recurrence, metastasis and tumour-related death and should be considered for adjuvant radiotherapy and possibly chemotherapy. The best method of monitoring extrapulmonary metastases remains to be established.

Highlights

Liposarcomas, which constitute a family of malignant mesenchymal neoplasms showing adipocytic differentiation, are the most common soft tissue sarcoma in adults [1]
The continuous morphologic spectrum, the shared clinical characteristics, and the common chromosome translocation t(12; 16), resulting in the fusion of the TLS (FUS) and CHOP (DDT3) genes seen in most of these tumours, clearly indicate that myxoid/round-cell liposarcoma (MRCLS) represents a distinct family among liposarcomas [3,4,5,6]
This study reports on the clinical behavior and outcome in terms of local control, metastatic disease, and disease-specific survival in 160 patients with primary myxoid/round-cell liposarcoma, diagnosed and surgically treated at a single centre over the last 20 years

Summary

Introduction

Liposarcomas, which constitute a family of malignant mesenchymal neoplasms showing adipocytic differentiation, are the most common soft tissue sarcoma in adults [1]. MRCLS carries an intermediate risk with approximately one-third of patients developing metastases and eventually dying of their tumours [7,8,9,10]. Another feature distinguishing MRCLS from the other types of liposarcoma and most other soft tissue sarcomas is its tendency to metastasize to other soft tissue sites, including the trunk and extremities, the retroperitoneum, the chest wall, the pleura, and the pericardium [11,12,13]. The occurrence of any round cell component is the most important adverse prognostic factor for patients with MRCLS; patients with >5% round cell component are at higher risk of local recurrence, metastasis and tumour-related death and should be considered for adjuvant radiotherapy and possibly chemotherapy. The best method of monitoring extrapulmonary metastases remains to be established

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Conclusion