"Desmoplastic Small Round-Cell Tumor: Prolonged Progression-Free Survival with Aggressive Multimodality Therapy"

DSRCT is a recently recognized cancer which has distinctive hisologic, biologic, and clinical features. It may have epithelial, neural, and muscle markers. It also has a recurrent specific chromosomal abnormality t(11;22)(p13;q12). The break points involve two genes which are associated with other malignant tumors, specifically, the Ewing’s family of tumors and the Wilm’s tumor.

DSRCT primarily occurs in young males who usually develop obstructive symptoms via intra-abdominal or pelvic masses. there is usually widespread peritoneal involvement and metastases to the lung, liver, and lymph nodes.

It rarely spreads to bone or bone marrow. Treatment for this tumor has included single and/or multi-agent chemotherapy. This study has treated DSRCT with high dose alkylator based regimen which is labeled the P6 protocol.

In this study 10 patients previously untreated and 2 patients previously treated were assessed. male to female ratio was 11 to 1, median age 14. Obstructive problems from the tumor were common. The largest masses were abdomen/pelvis, seen in 9 patients, abdomen only in 2, or thorax, in 1. Of the 10 patients previously untreated, 7 had a partial response or very good response to the protocol, which included HD-CAV (high dose cyclophosphamide, doxorubicin, vincristine). Two patients had no assessable disease after tumor resection at diagnosis, and one patient could not be studied due to early death. After P6 chemotherapy and surgery, 7 patients were in complete remission and 2 were in partial remission. 5 out of 7 patients in complete remission remain event free survivors. The P6 protocol required intensive transfusional and antibiotic support.

This study suggests that, like the other small round cell tumors of young people, DSRCT is alkylator sensitive and dose responsive. The HD-CAV produced regression of DSRCT, and therefore may allow tumors to be resectable and eliminate microscopic disease.

|   home   |   back to Bibliography   |