Orthopaedic Oncology Department, Azienda Ospedaliero Universitaria Careggi, Florence, Italy.
Sarcoma Unit, Royal National Orthopaedic Hospital, Stanmore, UK.
BMC Cancer. 2021 Apr 20;21(1):437. doi: 10.1186/s12885-021-08189-6.
Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate.
QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation.
This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF.
Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3-85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group.
Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.
腹外型硬纤维瘤病（DTF）是一种罕见的局部侵袭性软组织肿瘤。对于这一患者群体，最佳的治疗方式仍存在争议。
问题/目的：本文旨在（1）比较不同治疗方式后 DTF 的结果，（2）评估手术切除后复发的预后因素，以及（3）评估观察期间进展的预后因素。
这是一项在欧洲肌肉骨骼肿瘤学会（EMSOS）赞助下进行的回顾性多中心研究。所有参与的七个中心均为软组织肿瘤的三级转诊中心。所有患者均收集了基线人口统计学数据，以及诊断、肿瘤特征、临床特征、治疗方式以及是否存在 DTF 诱发因素的数据。
本研究共纳入 388 例患者（240 例女性，140 例男性），平均年龄为 37.6（±18.8 标准差，范围：3-85）。257 例患者（66%）接受了 ADF 的手术切除，70 例患者（18%）未接受治疗而处于观察状态，其余患者接受了不同的保守治疗。不同治疗组之间，在肿瘤复发或进展方面无显著差异。手术后，年龄较小、复发性疾病和较大的肿瘤大小是复发的危险因素，而肩部周围的肿瘤和疼痛性病变在观察组中则有进展的风险。
手术后的局部复发率与观察期间的进展率相似。因此，考虑到在连续两次（3 个月和 6 个月）的影像学检查中出现体积进展或出现疼痛性病变时进行手术，以及对肩部周围的病变给予特别关注，观察对于 DTF 是合理的。
