纤维瘤样肿瘤初始治疗演变

Evolution of Initial Treatment for Desmoid Tumors

ANNALS OF SURGICAL ONCOLOGY

影响因子:3.5

分区:医学2区 / 外科2区肿瘤学3区

发表日期:2024 Nov

作者: Alex J Bartholomew, Kristen E Rhodin, Laura Noteware, Dimitrios Moris, Elishama Kanu, Sabran Masoud, T Clark Howell, Danielle Burner, Charles Y Kim, Daniel P Nussbaum, Sabino Zani, Michael E Lidsky, Peter J Allen, Richard F Riedel, Dan G Blazer

DOI: 10.1245/s10434-024-15938-x

摘要

纤维瘤样肿瘤（DT）是一类罕见的纤维母细胞增殖，具有局部侵袭性但无转移潜能。传统上，初始治疗主要采用切除术，但现代指南和专家小组逐渐倾向于优先考虑主动监测策略。此次单机构回顾性病例分析，纳入2007年至2020年期间任何部位诊断的原发性DT患者。主要观察指标为初始治疗策略随时间的变化。次要指标包括未治疗生存（TFS）和接受主动监测患者的治疗时间（TTT），以及接受切除术患者的无复发生存（RFS）和复发时间。共纳入103例患者，女性占68%，中位随访44个月（24-74）。肿瘤常见位置包括腹壁（27%）、腹腔内/系膜（25%）、胸壁（19%）和肢体（10%）。初始治疗包括切除（60%）、系统治疗（20%）、主动监测（18%）和冷冻消融（2%）。手术切除比例显著下降（p<0.001），从2018年前的69.6%降至2018年后的29.2%。采用切除术的患者，5年RFS为41.2%；采用主动监测的患者，2年TFS为66.7%，中位TTT为4个月（4-10）。本单机构队列跨越十余年，显示了向主动监测转变的趋势，并强调了在监测时代的重要指标。这一变化与专家小组和共识指南推荐的治疗策略一致。

Abstract

Desmoid tumors (DTs) are rare, fibroblastic cell proliferations that can exhibit locally aggressive behavior but lack metastatic potential. Initial management has traditionally involved upfront resection; however, contemporary guidelines and expert panels have increasingly advocated for prioritizing active surveillance strategies.A single-institution, retrospective chart review identified all patients diagnosed with a primary DT at any site from 2007 to 2020. The primary outcome was the initial management strategy over time. Secondary outcomes included treatment-free survival (TFS) and time to treatment (TTT) for those undergoing active surveillance, as well as recurrence-free survival (RFS) and time to recurrence for those undergoing resection.Overall, 103 patients were included, with 68% female and a median follow-up of 44 months [24-74]. The most common tumor locations included the abdominal wall (27%), intra-abdominal/mesenteric (25%), chest wall (19%), and extremity (10%). Initial management included resection (60%), systemic therapy (20%), active surveillance (18%), and cryoablation (2%). Rates of surgical resection significantly decreased (p < 0.001) over time, from 69.6% prior to 2018 to 29.2% after 2018. For those treated with upfront resection, 5-year RFS was 41.2%, and for patients undergoing initial active surveillance, TFS was 66.7% at 2 years, with a median TTT of 4 months [4-10].This single-institution cohort at a tertiary medical center spanning over a decade demonstrates the transition to active surveillance for initial management of DTs, and highlights salient metrics in the era of surveillance. This trend mirrors recommended treatment strategies by expert panels and consensus guidelines.