RESUMO
Desmoplastic Small Round Cell Tumour (DSRCT) is an uncommon malignant type of Small Round Blue Cell tumours with less than 200 cases reported in literature. Notorious for its aggressiveness, its commonest site of affectation is the abdomen, with a predilection for peritoneal cavity and prone to spread to multiple organs. We report a case of a 26-year old male undergraduate who presented with a 6-month history of progressive abdominal swelling associated with abdominal pain, weight loss, and change in bowel habits. He was cachectic with dyspnoea, bilateral pitting pedal oedema, a grossly distended and a palpable huge firm mass measuring 20 x 15cm. Immunohistochemisttry confirmed a diagnosis of DSRCT. An abdominopelvic Computed Tomography (CT) scan showed extensive abdominal and pelvic disease with pulmonary and liver metastases. He could only tolerate one course of chemotherapy due to deteriorating renal function. It ran an aggressive course of 9 months from onset of symptoms to eventual demise of the patient. CONCLUSION: Desmoplastic Small Round Blue Cell Tumour is an uncommon and fatal disease, with no significant improvement in survival despite aggressive multimodality therapy. A high index of suspicion and Immunohistochemistry for accurate diagnosis as well as prompt and effective treatment will improve outcome.