Sarcoma Unit

Location: Royal Marsden

Section: NHS Clinical Research Programme

Head of Unit: Professor I R Judson MD FRCP

Introduction

Sarcomas are rare malignant tumours of the connective tissues or bones, accounting for less than 1% of all malignancies. The Sarcoma Unit provides a multidisciplinary service covering all aspects of diagnosis and therapy. Clinical trials are conducted both in-house and in collaboration with the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG). UK trials will be coordinated by the National Cancer Research Institute (NCRI) Sarcoma Clinical Studies Group. With increasing knowledge of the molecular basis of cancer we are entering a new era in sarcoma care. Individual disease subtypes can now be treated in a much more specific fashion, as exemplified by the ongoing experience with gastrointestinal stromal tumours (GIST) described below.

Sarcoma Treatment – a Specialised Service

Owing to their rarity we believe that the outcome of patients with sarcoma is improved by treatment in centres specialising in the care of these tumours. Such centres are capable of achieving superior results of local control, low amputation rates, reduced morbidity and probably improved survival. Centralisation of services for rare cancers is likely to have an increasing impact on referrals to this Unit. NICE published Improving Outcomes Guidance for people with sarcoma in 2006. This sets out the criteria for sarcoma treatment centres with the emphasis on surgical expertise. This is already having an impact on our practice and discussions are ongoing with Cancer Networks concerning arrangements to ensure that all sarcoma patients are discussed by a sarcoma multidisciplinary team. There are plans to incorporate a diagnostic service for patients with suspected sarcoma into a new Rapid Diagnostic Unit at the Royal Marsden NHS Foundation Trust.

Recent Highlights

• We have completed a first line Phase II study of the chemotherapy combination gemcitabine + docetaxel in metastatic leiomyosarcoma, in collaboration with University College Hospital. This is the first trial to study this combination in the first-line setting.
• New agents studied have included two receptor tyrosine kinase inhibitors, both of which produced significant responses, one was a study primarily in refractory GIST using AZD2171, performed at the Royal Marsden and Christie Hospitals. The other agent pazopanib was studied in a multicentre trial conducted by the EORTC STBSG. The minor groove binder brostallicin is in Phase II clinical trial
• Intensity Modulated Radiotherapy (IMRT) is being investigated as means of optimising the delivery of adjuvant radiotherapy for sarcoma patients
• Publications in 2007 included papers on trabectedin in myxoid liposarcoma, treatment of paediatric GIST, isolated limb perfusion, retroperitoneal sarcoma, and clear cell sarcoma
• We have a European Union Network of Excellence grant to facilitate cooperative work on the molecular biology of sarcoma

Future Aims

The Unit will continue to use its database of nearly 5,000 sarcoma patients to research rare sarcoma subtypes. The database is a valuable resource that is maintained prospectively and, not only generates research and audit information locally, but will also contribute to the national data on sarcomas once the NHS database is operational. Imatinib refractory GIST continues to be a focus of study and future trials will include a randomised study with the HSP90 from Infinity. The VORTEX radiotherapy study being conducted by the NCRI Sarcoma Clinical Studies Group will investigate the optimum volume for post-operative adjuvant radiotherapy. There is a growing interest in studying novel anticancer agents in the treatment of sarcomas other than GIST, which it is hoped will ultimately improve the outlook of patients with advanced disease in a similar fashion to the impact of imatinib. New agents to be studied include an antibody targeting a death receptor and an antibody targeting the receptor IGF1-R, which will principally focus on Ewing’s family tumours.