Desmoplastic Small Round Cell Tumour (DSRCT) is a rare malignancy that usually affects young males. Children, adolescents and young adults are most commonly affected and the median age of diagnosis is in the 20s. Most cases are advanced at presentation and cure is rare; 5-year survival even with treatment is in the order of 15%.
Basic Sciences
Diagnosis
Presentation
Patients, usually men, present with abdominal complaints in the vast majority of cases. Rarely patients present with disease of the tunica vaginalis or pleura in which case presenting symptoms are localised to these sites.
History
History should focus on the symptoms, any past history of malignancy, previous radiotherapy and any family history of malignancy. The patient's ability to tolerate the various treatments should be established.
Examination
Common findings include abdominal masses, distension or ascites. Patients may have obstruction of the gastrointestinal, hepatobiliary or renal systems due to widespread involvement of the peritoneum.
Investigations
Blood tests are useful to establish baseline organ function which may direct acute management (eg. stenting for renal or hepatic impairment). Tumour markers are not typically used.
The role of the various imaging modalities is not well established due to the rarity of the condition; CT Abdomen typically shows widespread involvement of the peritoneum.
Staging
There is no specific staging system for DSRCT. Most cases are considered stage IV due to the widespread disease and difficulty achieving cure.
