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Desmoplastic small round cell tumor (DSRCT) is an aggressive malignant neoplasm that occurs in adolescents and young adults. This tumor can co-express epithelial, neuronal, and mesenchymal markers. It is usually described a distinct type of small round blue cell tumor with a predilection for serosal surfaces such as the peritoneum and the tunica vaginalis.
Causes
There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer.
Typical Symptoms
DSRCT presents as an abdominal mass with peritoneal and omental implants. Associated symptoms may include crampy abdominal pain, weight loss and constipation. The most common presentation is bulky abdominal disease present in a young adult, often males. Other reported sites of disease include pleura, ethmoid sinuses, scalp, hand, posterior cranial fossa, pancreas, ovary, paratesticular and kidney.
Therapeutic Approach
DSRCT is a very aggressive neoplasm with a 5-year survival of less than 15%. Treatment options include surgery, radiotherapy, chemotherapy with or without stem cell transplantation, and recently introduced molecularly targeted therapies. Unfortunately there is no standard therapeutic regimen described since no modality is clearly superior to any other. Surgery is usually extensive and often includes excision of the omentum, splenectomy and lymph node resections.