Desmoplastic small round cell tumour: a review of literature and treatment options.

INTRODUCTION: Desmoplastic Small Round Cell Tumour (DSRCT) is a rare but aggressive malignancy with poor outcome.

AIMS: To review the clinico-pathological features and radiological, histological and tumour markers of the disease and to evaluate the evidence for treatment options available.

METHODS: We report a clinical case from our centre and have conducted a review of the literature from Medline (Pubmed) database from 1989 to 2007.

RESULTS: DSRCT typically presents with advanced disease and is prevalent in young males. Lack of staging criteria and small numbers of patients make comparison of evidence for its treatment difficult.

CONCLUSION: Surgical excision is only recommended for non-metastatic disease with combination chemo-radiotherapy as an adjunct. These modalities used in isolation may have less impact. Furthermore, the side effect profile from radiotherapy may outweigh any survival benefit. For advanced disease, symptom control is most important as these modalities impact survival minimally and palliation of secondary symptoms is paramount. Multi-disciplinary team and specialist centre review for histology and oncology are essential in managing this disease process and will enable greater numbers of patients to be enrolled into therapeutic trials and future evolving therapies.