Desmoplastic Small Round Cell Tumor: I: a Histopathologic Study of 39 Cases with Emphasis on Unusual Histological Patterns

The clinical and histological features of 39 cases of desmoplastic small round cell tumor (DSRCT) diagnosed at M.D. Anderson Cancer Center are presented. Thirty-two of the patients were men and seven were women ranging in age from 10 to 41 years (mean, 25 years). Twenty-five of the 35 patients for whom follow-up information was available died of widespread metastases 8 to 50 months (mean, 25.2 months) from the time of their diagnosis and the remaining 10 were alive with disease. With the exception of two cases that occurred in the liver and in the scrotum, respectively, all of the cases originated within the abdominal and/or pelvic peritoneum. Eight tumors also involved the retroperitoneum. Although the characteristic histologic pattern of "small, blue cells" embedded in a dense fibrous stroma was seen in most cases, about one third of the tumors exhibited a wide range of morphologic features. The recognition of these uncommon morphologic variants of DSRCT is of paramount importance to avoid a misdiagnosis because these tumors could potentially be confused with other neoplastic conditions.