Bongiovanni Alberto, Riva Nada, Ricci Marianna, Mercatali Laura, Liverani Chiara, La Manna Federico, De Vita Alessandro, Cavaliere Davide, Pieri Federica, Oboldi Devil, Verdecchia Giorgio Maria, Amadori Dino, Ibrahim Toni
Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST) IRCCS, Meldola, Italy.
Unit of Surgery and Advanced Oncologic Therapies, Morgagni-Pierantoni Hospital, Forlì, Italy.
BMC Cancer. 2015 Dec 22;15:998. doi: 10.1186/s12885-015-2038-7.
Leiomyosarcoma (LMS) is an aggressive soft tissue sarcoma derived from smooth muscle cells typically of uterine, gastrointestinal or soft tissue origin. The prognosis for this tumor is poor, with survival rates among the lowest of all soft tissue sarcomas. Surgery is the best approach for localized disease. The principal role of chemotherapy is prevalently in the treatment of metastatic disease. Trabectedin, a promising new DNA-damaging agent with a mechanism of action that differs from that of traditional alkylating agents, has been approved in Europe for the treatment of patients with advanced soft tissue sarcoma after failure of anthracyclines and ifosfamide,
We report the case of a 53-year-old woman with metastatic well differentiated uterine leiomyosarcoma refractory to multiple treatments who underwent 22 cycles of trabectedin over 30 months, obtaining a partial response according to RECIST (Response Evaluation Criteria in Solid Tumors) criteria, with good tolerability, and maintaining the response for 10 months after trebectedin withdrawal.
This very prolonged response, which persisted after drug discontinuation, suggests that trabectedin exerts an oncostatic effect rather than the cytotoxic one produced by other chemotherapeutic agents. Our experience also raises the question of the best way to evaluate trabectedin efficacy.
平滑肌肉瘤(LMS)是一种侵袭性软组织肉瘤,起源于平滑肌细胞,通常来源于子宫、胃肠道或软组织。该肿瘤预后较差,在所有软组织肉瘤中生存率处于最低水平。手术是局限性疾病的最佳治疗方法。化疗的主要作用普遍在于转移性疾病的治疗。曲贝替定是一种有前景的新型DNA损伤剂,其作用机制不同于传统的烷化剂,在欧洲已被批准用于治疗蒽环类药物和异环磷酰胺治疗失败后的晚期软组织肉瘤患者。
我们报告了一例53岁女性,患有转移性高分化子宫平滑肌肉瘤,对多种治疗均耐药,在30个月内接受了22个周期的曲贝替定治疗,根据实体瘤疗效评价标准(RECIST)达到部分缓解,耐受性良好,在停用曲贝替定后缓解持续了10个月。
这种在停药后仍持续很长时间的缓解表明,曲贝替定发挥的是肿瘤抑制作用,而非其他化疗药物产生的细胞毒性作用。我们的经验还提出了评估曲贝替定疗效的最佳方法这一问题。