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，由于肿瘤体积大、边界不清、解剖结构限制以及覆盖腹膜较薄，腹膜后软组织肉瘤通常无法进行广泛切除，因此其局部复发率远高于四肢软组织肉瘤。局部复发是低级别腹膜后软组织肉瘤的主要死亡原因，而高级别肿瘤则易发生远处转移。近几十年来，为改善原发性局限性腹膜后软组织肉瘤的肿瘤学结局，新兴治疗策略（如更广泛的手术和（新）辅助治疗）的作用已得到广泛研究。本综述全面讨论了原发性局限性腹膜后软组织肉瘤多学科综合治疗的最新数据。腹膜后软组织肉瘤具有异质性，其组织学亚型和生物学行为各不相同，因此标准的“一刀切”治疗方法并不适用，应根据组织学类型和恶性程度调整治疗方案。有充分证据表明，对于低级别肉瘤和脂肪肉瘤，一线扩大手术（包括间室切除，即切除所有同侧腹膜后脂肪，并对邻近器官和结构进行广泛整块切除，即使它们未宏观受累）可提高局部肿瘤控制率，但对于平滑肌肉瘤，宏观完全切除似乎已足够。此外，术前放疗并非适用于所有腹膜后软组织肉瘤，但似乎对高分化脂肪肉瘤和I/II级去分化脂肪肉瘤有益，可能也对孤立性纤维瘤有益。新辅助化疗对高级别腹膜后软组织肉瘤是否有益，回顾性数据尚不明确，这是正在进行的随机STRASS 2试验的研究主题，其结果备受期待。个性化、基于组织学类型的多模式治疗前景广阔，随着我们对腹膜后软组织肉瘤分子和遗传特征认识的加深，该领域很可能进一步发展。
Retroperitoneal Soft Tissue Sarcoma: Emerging Therapeutic Strategies
肿瘤(癌症)患者之家