ET 743 is an effective agent against sarcoma

20516 Background: ET-743 has shown clinical activity in soft tissue sarcoma after failure of anthracyclines and ifosfamide. We propose to assess these results in our institution. Methods: We reviewed the patients treated (in phase II studies or on a compassionate basis) until June 2006 Results: Median age of the 99 patients was 53 years (18–82, with 20% leiomyosarcomas, 18% other spindle cell sarcomas, 12% liposarcomas, 9% osteosarcomas and 4% Ewing's sarcomas. The initial tumor was in the lower limbs in 31% of cases, and 70% had lung metastases. Patients had received anthracyclines (72%) or ifosfamide (61%), but 12% received ET-743 as their 1st chemotherapy. Most patients received ET-743 as a single agent, but 7 received it in combination with doxorubicin. After a median of 4 cycles (1–25), 50% of patients responded: 0 CR, 12 patients with PR, 4 MR (minor response), 34 SD and 49 progressed. Median time to progression for patients with a clinical benefit (PR+MR+SD) was 7 months (1.5 to 66 months). Among the 29 patients who didn't progress in the first six months, 10 had leiomyosarcomas and 8 spindle cell sarcomas. Grade3/4 toxicity, mainly hematological (42% neutropenia, 16% anemia, 11% thrombocytopenia) and hepatic (33%), was noted in 69 patients (28 needed a dose reduction). Main cause of treatment discontinuation was progression (70%) but 7 patients had to stop treatment because of toxicity. Conclusions: ET- 743 can benefit some patients with sarcomas, especially leiomyosarcomas, even pretreated. Some patients have long lasting responses. Grade ¾ toxicity is frequent but rarely leads to treatment discontinuation No significant financial relationships to disclose. </jats:p

Superficial primitive Ewing's sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases.

Superficial primitive Ewing's sarcomas are rare and have been reported to be of favorable prognosis compared to conventional deep-seated tumors. In the skin and subcutis, the diagnosis is often difficult, and performing molecular cytogenetic techniques may be helpful. We performed a retrospective analysis of 14 cases of superficial Ewing's sarcomas, all confirmed by molecular cytogenetics. Clinical, histological, immunohistochemical, molecular cytogenetic, therapeutic, and follow-up data are reported. There were 11 female and 3 male patients aged from 12 to 77 years (median: 17 years). Seven tumors occurred in the extremities, five in the trunk wall, and two in the head. Tumor size ranged from 1 to 5 cm (median, 3 cm). They were all small round-cell proliferations with a strong membranous positivity for CD99. Ewing's sarcoma translocations/fusion gene transcripts were detected in eight cases, both by FISH and reverse transcriptase (RT)-PCR. Four tumors were positive by RT-PCR alone (FISH not done in three cases and not interpretable in one case), and two cases were positive by FISH alone (RT-PCR not done). Surgical resection was performed in all patients. Chemotherapy was given in ten patients and radiotherapy in six. At last medical examination (median follow-up, 47 months), two patients who underwent surgical resection alone had died from the tumor. Our results confirm that superficial Ewing's sarcomas are of good prognosis. Given the difficulty of the diagnosis and the importance of an adapted treatment, a confirmation of the diagnosis by molecular or cytogenetic techniques is recommended when dealing with a superficial tumor