Midlands Abdominal and Retroperitoneal Sarcoma Unit, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham, UK.
Department of Medical Statistics, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
J Surg Oncol. 2021 Mar;123(4):1057-1066. doi: 10.1002/jso.26353. Epub 2020 Dec 23.
Primary mesenteric soft tissue sarcomas (STS) are rare and limited evidence is available to inform management. Surgical resection is challenging due to the proximity of vital structures and a need to preserve enteric function.
To determine the overall survival (OS) and recurrence-free survival (RFS) for patients undergoing primary resection for mesenteric STS.
The Trans-Atlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG) is an intercontinental collaborative comprising specialist sarcoma centers. Data were collected retrospectively for all patients with mesenteric STS undergoing primary resection between 2000 and 2019.
Fifty-six cases from 15 institutions were included. The spectrum of pathology was similar to the retroperitoneum, although of a higher grade. R0/R1 resection was achieved in 87%. Median OS was 56 months. OS was significantly shorter in higher-grade tumors (p = .018) and extensive resection (p < .001). No significant association between OS and resection margin or tumor size was detected. Rates of local recurrence (LR) and distant metastases (DM) at 5 years were 60% and 41%, respectively. Liver metastases were common (60%), reflecting portal drainage of the mesentery.
Primary mesenteric sarcoma is rare, with a modest survival rate. LR and DM are frequent events. Liver metastases are common, highlighting the need for surveillance imaging.
原发性肠系膜软组织肉瘤（STS）较为罕见，目前可用于指导治疗的证据有限。由于毗邻重要结构且需要保留肠功能，因此手术切除具有挑战性。
评估接受原发性肠系膜 STS 切除术患者的总体生存率（OS）和无复发生存率（RFS）。
跨大西洋澳大拉西亚腹膜后肉瘤工作组（TARPSWG）是一个由专业肉瘤中心组成的洲际协作组织。该研究回顾性收集了 2000 年至 2019 年间所有接受原发性肠系膜 STS 切除术的患者数据。
15 个机构的 56 例患者纳入研究。其病理学谱与腹膜后相似，但分级更高。87%的患者达到了 R0/R1 切除。中位 OS 为 56 个月。高级别肿瘤（p=0.018）和广泛切除（p<0.001）患者的 OS 明显更短。OS 与切缘或肿瘤大小之间无显著相关性。5 年局部复发（LR）和远处转移（DM）的发生率分别为 60%和 41%。肝转移较为常见（60%），反映了肠系膜的门静脉引流。
原发性肠系膜肉瘤较为罕见，生存率适中。LR 和 DM 是常见事件。肝转移常见，强调了监测影像学的必要性。
