Palassini Elena, Frezza Anna Maria, Mariani Luigi, Lalli Luca, Colombo Chiara, Fiore Marco, Messina Antonella, Casale Alessandra, Morosi Carlo, Collini Paola, Stacchiotti Silvia, Casali Paolo Giovanni, Gronchi Alessandro
From the Departments of *Medical Oncology, †Statistics, and ‡Surgery, IRCCS Fondazione Istituto Nazionale Tumori; §Department of Medical Oncology, Humanitas Cancer Center, IRCCS; and Departments of ∥Radiology and ¶Pathology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy.
Cancer J. 2017 Mar/Apr;23(2):86-91. doi: 10.1097/PPO.0000000000000254.
Today, surgery and radiation therapy have a limited role in desmoid-type fibromatosis. Different systemic treatments were shown to be effective. Herein, we report on our institutional experience with low-dose methotrexate (MTX) + vinca alkaloids in this disease over the last 25 years.
We retrospectively reviewed data from all adult patients with sporadic desmoid-type fibromatosis treated with MTX and vinca alkaloids at our institution between 1989 and 2014.
We identified 75 patients treated with MTX + vinblastine (40%), MTX + vinorelbine (57%), and vinorelbine alone (3%). All patients had progressive disease before chemotherapy; 72%, 10%, and 48% of patients had received previous surgery, radiation therapy, and/or systemic treatments, respectively. Chemotherapy was administered for a median duration of 14 months and a median number of 37.5 cycles. Eight patients interrupted chemotherapy because of toxicity. According to RECIST (Response Evaluation Criteria in Solid Tumors) complete response, partial response, stable disease, and progressive disease were observed in 1%, 47%, 51%, and 1% of patients, respectively. Symptomatic relief was obtained in 80% of symptomatic cases. The median progression-free survival (PFS) was 75 months; it was 136 months in responding patients. Upon progression, after chemotherapy withdrawal, MTX plus vinblastine/vinorelbine was offered to 11 patients with partial response, stable disease, and progressive disease in 4, 6, and 1 cases, resulting in a median PFS of 53 months.
In this series, chemotherapy with MTX and vinca alkaloids is confirmed to be active and effective, with a remarkable PFS, higher in responding patients, and limited toxicity. Even progression can be successfully rechallenged.
如今，手术和放射治疗在硬纤维瘤病中的作用有限。已证明不同的全身治疗方法有效。在此，我们报告过去25年我们机构使用低剂量甲氨蝶呤（MTX）加长春花生物碱治疗该病的经验。
我们回顾性分析了1989年至2014年间在我们机构接受MTX和长春花生物碱治疗的所有成年散发性硬纤维瘤病患者的数据。
我们确定了75例接受MTX + 长春碱治疗的患者（40%）、MTX + 长春瑞滨治疗的患者（57%）以及单独使用长春瑞滨治疗的患者（3%）。所有患者在化疗前均患有进展性疾病；分别有72%、10%和48%的患者曾接受过手术、放射治疗和/或全身治疗。化疗的中位持续时间为14个月，中位周期数为37.5个。8例患者因毒性反应中断化疗。根据实体瘤疗效评价标准（RECIST），分别有1%、47%、51%和1%的患者出现完全缓解、部分缓解、病情稳定和病情进展。80%有症状的病例获得了症状缓解。中位无进展生存期（PFS）为75个月；缓解患者的PFS为136个月。病情进展后，化疗停药后，分别有4例部分缓解、6例病情稳定和1例病情进展的11例患者接受了MTX加长春碱/长春瑞滨治疗，中位PFS为53个月。
在本系列研究中，MTX和长春花生物碱化疗被证实具有活性且有效，PFS显著，缓解患者更高，毒性有限。即使病情进展也可成功再次挑战治疗。
