Long-term results with combined treatment in adult localized extraskeletal Ewing's sarcoma (ES): A retrospective analysis of 51 patients (pts) from a single referral center

Abstract

10548 Background: After the first description in 1975, only small series of adult extraskeletal ES have been reported. In general, adulthood is claimed to be an adverse prognostic factor, while the meaning of extraskeletal origin is less established. Methods: We retrospectively reviewed adult pts with extraskeletal, localized ES, undergoing medical therapy between 1988 and 2004, who were included in the dataset of the current Adult Sarcoma Medical Unit at Istituto Nazionale Tumori, Milano, Italy, which serves as a reference center for adult soft tissue sarcoma. Fifty-one pts were treated with a combined approach, including chemotherapy, surgery when appropriate, and radiation therapy. Overall, 27/51 pts were male, and age ranged from 16 to 63 years. Site of primary lesions was: thorax 14 (27%), limbs 18 (35%), paravertebral region 5 (10%), pelvis 4 (8%), head&neck 3 (6%), superficial trunk 6 (12%), other 1 (2%). Seven pts presented with locally advanced disease. Chemotherapy regimens included: vincristine (V), ifosfamide (I), epirubicin (E)/adriamycin (A), dacarbazine (D), actinomycinD (ActD), cisplatin (C), etoposide (Et). 35/51 pts (68%) underwent preoperative chemotherapy: 30 pts received IVE × 4–7 cycles; 5 pts received VAI alternating with IEt × 9 cycles. 42/51 pts underwent surgery; 38/51 pts received radiation therapy. Results: Response rate to induction chemotherapy including antracicline, ifosfamide + etoposide was 83%. Of these pts, 9 showed a complete response (CR), 17 a partial response (PR), 3 a minor response (MR), 3 stable disease (SD), and 3 progressive disease (PD). Overall, DFS at 10 years (y) was 55%. Pts with limb, head&neck, or superficial trunk lesions had a better outcome compared to those with other primary sites (DFS at 10y is 71% vs 33%). Conclusions: In this series of adult pts with extraskeletal ES, response rate and DFS were comparable to those of ES of bone in conventional series. The primary site may identify a subset of pts with worse outcome and prognosis, for whom more intensive treatments could be investigated. No significant financial relationships to disclose.