de Bree Eelco, Michelakis Dimosthenis, Heretis Ioannis, Kontopodis Nikolaos, Spanakis Konstantinos, Lagoudaki Eleni, Tolia Maria, Zografakis-Sfakianakis Michail, Ioannou Christos, Mavroudis Dimitrios
Department of Surgical Oncology, Medical School of Crete University Hospital, 71110 Heraklion, Greece.
Department of Urology, Medical School of Crete University Hospital, 71110 Heraklion, Greece.
Cancers (Basel). 2023 Nov 18;15(22):5469. doi: 10.3390/cancers15225469.
Retroperitoneal soft tissue sarcoma (RPS) is a rare and heterogenous disease for which surgery is the cornerstone of treatment. However, the local recurrence rate is much higher than in soft tissue sarcoma of the extremities since wide resection is usually unfeasible in RPS due to its large size, indistinct tumour borders, anatomical constraints and the thinness of the overlying peritoneum. Local recurrence is the leading cause of death for low-grade RPS, whereas high-grade tumours are prone to distant metastases. In recent decades, the role of emerging therapeutic strategies, such as more extended surgery and (neo)adjuvant treatments to improve oncological outcome in primary localised RPS, has been extensively investigated. In this review, the recent data on the evolving multidisciplinary management of primary localised RPS are comprehensively discussed. The heterogeneity of RPS, with their different histological subtypes and biological behaviour, renders a standard therapeutic 'one-size-fits-all' approach inappropriate, and treatment should be modified according to histological type and malignancy grade. There is sufficient evidence that frontline extended surgery with compartmental resection including all ipsilateral retroperitoneal fat and liberal en bloc resection of adjacent organs and structures, even if they are not macroscopically involved, increases local tumour control in low-grade sarcoma and liposarcoma, but not in leiomyosarcoma for which complete macroscopic resection seems sufficient. Additionally, preoperative radiotherapy is not indicated for all RPSs, but seems to be beneficial in well-differentiated liposarcoma and grade I/II dedifferentiated liposarcoma, and probably in solitary fibrous tumour. Whether neoadjuvant chemotherapy is of benefit in high-grade RPS remains unclear from retrospective data and is subject of the ongoing randomised STRASS 2 trial, from which the results are eagerly awaited. Personalised, histology-tailored multimodality treatment is promising and will likely further evolve as our understanding of the molecular and genetic characteristics within RPS improves.
腹膜后软组织肉瘤(RPS)是一种罕见且异质性的疾病,手术是其治疗的基石。然而,由于RPS体积大、肿瘤边界不清、解剖学限制以及覆盖腹膜较薄,广泛切除通常不可行,因此其局部复发率远高于四肢软组织肉瘤。局部复发是低级别RPS的主要死亡原因,而高级别肿瘤则易于发生远处转移。近几十年来,人们广泛研究了新兴治疗策略的作用,如更广泛的手术和(新)辅助治疗,以改善原发性局限性RPS的肿瘤学结局。在本综述中,全面讨论了原发性局限性RPS不断发展的多学科管理的最新数据。RPS的异质性及其不同的组织学亚型和生物学行为,使得标准的“一刀切”治疗方法不合适,治疗应根据组织学类型和恶性程度进行调整。有充分证据表明,一线扩大手术包括隔室切除,即切除所有同侧腹膜后脂肪以及对相邻器官和结构进行广泛整块切除,即使它们在肉眼下未受累,可提高低级别肉瘤和脂肪肉瘤的局部肿瘤控制率,但对平滑肌肉瘤似乎并不适用,因为完全肉眼切除似乎就足够了。此外,并非所有RPS都需要术前放疗,但术前放疗似乎对高分化脂肪肉瘤和I/II级去分化脂肪肉瘤有益,可能对孤立性纤维瘤也有益。回顾性数据尚不清楚新辅助化疗对高级别RPS是否有益,这也是正在进行的随机STRASS 2试验的研究内容,人们急切期待该试验的结果。随着我们对RPS分子和遗传特征的理解不断提高,个性化的、根据组织学定制的多模式治疗前景广阔,且可能会进一步发展。