Post by admin on Sept 15, 2005 20:57:01 GMT -5
From the University of Minnesota Cancer Center
ASCO 2001 Annual Meeting
PEDIATRIC ADVANCED THERAPIES
ASCO 2001 Annual Meeting
PEDIATRIC ADVANCED THERAPIES
New research from the Advanced Therapies Program presented at the American Society for Clinical Oncology Annual Meeting.
Outcome of Treatment of Desmoplastic Small Round Cell Tumor (DSRCT) of the Abdomen
Brenda Weigel, Jennifer Hirshfeld, Robert Ettinger, Thomas Loew, Joseph Neglia, John Perentesis
University of Minnesota Cancer Center, Minneapolis, MN; Gunderson Lutheran Medical Center, LaCrosse, WI; University of Iowa, Iowa City, IA.
Desmoplastic Small Round Cell Tumor (DSRCT), first reported in 1989, is an aggressive tumor in the Ewing's sarcoma family of tumors most often occurring in young males. It typically presents with widespread intraabdominal seeding, metastasizes to liver and lymph nodes and is associated with a dismal prognosis. We report 6 patients (5 males, 1 female) with DSRCT diagnosed at a median age of 14.3 years (range 8-30 years). Tumor cytogenetic analysis was performed on 4 patients, 2 had the characteristic t(11,22) translocation and 1 had a t(8,10)del(22)(q11). All patients received induction therapy with intensive alkylating agent/anthracycline based regimens: vincristine, doxorubicin and cyclophosphamide (VAdriaC) alternating with ifosfamide, carboplatin and etoposide (3 patients); VAdriaC alternating with ifosfamide and etoposide (2 patients); or VAdriaC + methotrexate alternating with cisplatin, bleomycin and vinblastine (1 patient). Following induction chemotherapy, 2 patients had major responses and underwent debulking surgery; 2 patients had stable disease (SD) but were not candidates for surgery, and 2 patients had progressive disease (PD) and died from disease after failing salvage therapies. The 2 patients with SD were additionally treated with irinotecan which resulted in a PR in 1 patient, permitting surgery; and SD for 8 months in the other patient. Intensification therapy with high dose alkylators (busulfan/melphalan/thiotepa) and autologous stem cell transplant (ASCT) was conducted in all 3 patients that underwent surgical resection of their tumor. After recovery from ASCT, patients received whole abdominal irradiation therapy (XRT). A second stem cell re-infusion was performed in 1 patient following XRT. The 3 patients who underwent ASCT/XRT are alive, without evidence of disease 1 - 45 months post ASCT (12-53 months post diagnosis). No toxic deaths occurred. DSRCT is an aggressive malignancy, though intensive treatment with sarcoma-based chemotherapy, surgery, ASCT, and whole abdominal irradiation is feasible and may provide the possibility of successful therapy.
New research from the Advanced Therapies Program presented at the American Society for Clinical Oncology Annual Meeting.
Outcome of Treatment of Desmoplastic Small Round Cell Tumor (DSRCT) of the Abdomen
Brenda Weigel, Jennifer Hirshfeld, Robert Ettinger, Thomas Loew, Joseph Neglia, John Perentesis
University of Minnesota Cancer Center, Minneapolis, MN; Gunderson Lutheran Medical Center, LaCrosse, WI; University of Iowa, Iowa City, IA.
Desmoplastic Small Round Cell Tumor (DSRCT), first reported in 1989, is an aggressive tumor in the Ewing's sarcoma family of tumors most often occurring in young males. It typically presents with widespread intraabdominal seeding, metastasizes to liver and lymph nodes and is associated with a dismal prognosis. We report 6 patients (5 males, 1 female) with DSRCT diagnosed at a median age of 14.3 years (range 8-30 years). Tumor cytogenetic analysis was performed on 4 patients, 2 had the characteristic t(11,22) translocation and 1 had a t(8,10)del(22)(q11). All patients received induction therapy with intensive alkylating agent/anthracycline based regimens: vincristine, doxorubicin and cyclophosphamide (VAdriaC) alternating with ifosfamide, carboplatin and etoposide (3 patients); VAdriaC alternating with ifosfamide and etoposide (2 patients); or VAdriaC + methotrexate alternating with cisplatin, bleomycin and vinblastine (1 patient). Following induction chemotherapy, 2 patients had major responses and underwent debulking surgery; 2 patients had stable disease (SD) but were not candidates for surgery, and 2 patients had progressive disease (PD) and died from disease after failing salvage therapies. The 2 patients with SD were additionally treated with irinotecan which resulted in a PR in 1 patient, permitting surgery; and SD for 8 months in the other patient. Intensification therapy with high dose alkylators (busulfan/melphalan/thiotepa) and autologous stem cell transplant (ASCT) was conducted in all 3 patients that underwent surgical resection of their tumor. After recovery from ASCT, patients received whole abdominal irradiation therapy (XRT). A second stem cell re-infusion was performed in 1 patient following XRT. The 3 patients who underwent ASCT/XRT are alive, without evidence of disease 1 - 45 months post ASCT (12-53 months post diagnosis). No toxic deaths occurred. DSRCT is an aggressive malignancy, though intensive treatment with sarcoma-based chemotherapy, surgery, ASCT, and whole abdominal irradiation is feasible and may provide the possibility of successful therapy.
PEDIATRIC ADVANCED THERAPIES