Since its first description in 1989 by Gerald and Rosai[1], desmoplastic small round cell tumour (DSRCT) is being increasingly identified, but its histogenesis remains uncertain. It is distinguished from the other small round cell tumours by a characteristic histologic appearance. This is marked by a nesting pattern of cellular growth within abundant desmoplastic stroma and a specific poly-phenotypic differentiation, with coexpression of epithelial, mesenchymal, and neural markers. A specific recurrent chromosomal translocation, t(11,22)(p13;q12),involving the EWS and the WT1 genes has been de-scribed. The tumour predominantly affects young males, usually in their second decade of life. DSRCT typically presents as a large abdominal mass already widely disseminated at the time of diagnosis, with extensive spread or the regional lymph nodes, peritoneal seeding, and distant metastases to the liver, lungs, and bones. Other, less frequent primary sites are the paratesticular region and the thoracic cavity, sometimes with extensive involvement of the pleura. This last supports a relationship between DSRCT and the mesothelial or sub mesothelial structures. Intracranial origin has also been described. Because of its rarity, the standard treatment for this tumour is still far from being established. Worldwide no consensus for treatment approach and molecular study are shared with the aim to better understand this tumor and its behaviour in an international collaborative manner; to date no new clinical trial (including new drug) or cooperative protocol are started. I strongly believe that an expert panel should be created and supported by international organizations or groups, to draw the necessary guidelines to obtain homogenous data from a larger series of desmopalstic small round cell tumors.
Agree, Are you interested to collaborate and create a set of guidelines?
Yes! I'm collaborating also with a panel of expert conducted by dr Whelan UK trying to do that, but I would happy to join you and perhaps we could prepare together a review for publishing? I could share some minutes from our meeting with you if you would.
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