Pavlidis Efstathios T, Pavlidis Theodoros E
2 Propedeutic Department of Surgery, Hippocration Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece.
World J Clin Oncol. 2023 Feb 24;14(2):89-98. doi: 10.5306/wjco.v14.i2.89.
Soft tissue sarcoma (STS) accounts for 1% of all malignant neoplasms in adults. Their diagnosis and management constitute a challenging target. They originate from the mesenchyme, and 50 subtypes with various cytogenetic profiles concerning soft tissue and bones have been recognized. These tumors mainly affect middle-aged adults but may be present at any age. Half of the patients have metastatic disease at the time of diagnosis and require systemic therapy. Tumors above 3-5 cm in size must be suspected of potential malignancy. A thorough history, clinical examination and imaging that must precede biopsy are necessary. Modern imaging techniques include ultrasound, computed tomography (CT), new magnetic resonance imaging (MRI), and positron emission tomography/CT. MRI findings may distinguish low-grade from high-grade STS based on a diagnostic score (tumor heterogeneity, intratumoral and peritumoral enhancement). A score ≥ 2 indicates a high-grade lesion, and a score ≤ 1 indicates a low-grade lesion. For disease staging, abdominal imaging is recommended to detect early abdominal or retroperitoneal metastases. Liquid biopsy by detecting genomic material in serum is a novel diagnostic tool. A preoperative biopsy is necessary for diagnosis, prognosis and optimal planning of surgical intervention. Core needle biopsy is the most indicative and effective. Its correct performance influences surgical management. An unsuccessful biopsy means the dissemination of cancer cells into healthy anatomical structures that ultimately affect resectability and survival. Complete therapeutic excision (R0) with an acceptable resection margin of 1 cm is the method of choice. However, near significant structures, , vessels, nerves, an R2 resection (macroscopic margin involvement) preserving functionality but having a risk of local recurrence can be an acceptable choice, after informing the patient, to prevent an unavoidable amputation. For borderline resectability of the tumor, neoadjuvant chemo/radiotherapy has a place. Likewise, after surgical excision, adjuvant therapy is indicated, but chemotherapy in nonmetastatic disease is still debatable. The five-year survival rate reaches up to 55%. Reresection is considered after positive or uncertain resection margins. Current strategies are based on novel chemotherapeutic agents, improved radiotherapy applications to limit local side effects and targeted biological therapy or immunotherapy, including vaccines. Young age is a risk factor for distant metastasis within 6 mo following primary tumor resection. Neoadjuvant radiotherapy lasting 5-6 wk and surgical resection are indicated for high-grade STS (grade 2 or 3). Wide surgical excision alone may be acceptable for patients older than 70 years. However, locally advanced disease requires a multidisciplinary task of decision-making for amputation or limb salvage.
软组织肉瘤(STS)占成人所有恶性肿瘤的1%。其诊断和治疗是一项具有挑战性的目标。它们起源于间充质,已识别出50种涉及软组织和骨骼的具有不同细胞遗传学特征的亚型。这些肿瘤主要影响中年成年人,但可出现在任何年龄。一半的患者在诊断时已发生转移性疾病,需要进行全身治疗。大小超过3 - 5厘米的肿瘤必须怀疑有潜在恶性。在活检前必须进行全面的病史、临床检查和影像学检查。现代影像学技术包括超声、计算机断层扫描(CT)、新型磁共振成像(MRI)和正电子发射断层扫描/CT。MRI表现可根据诊断评分(肿瘤异质性、瘤内和瘤周强化)区分低级别和高级别STS。评分≥2表明为高级别病变,评分≤1表明为低级别病变。对于疾病分期,建议进行腹部影像学检查以检测早期腹部或腹膜后转移。通过检测血清中的基因组物质进行液体活检是一种新型诊断工具。术前活检对于诊断、预后评估和手术干预的最佳规划是必要的。粗针活检是最具指示性和有效性的。其正确操作会影响手术管理。活检不成功意味着癌细胞扩散到健康的解剖结构中,最终影响可切除性和生存率。以1厘米的可接受切缘进行完整的治疗性切除(R0)是首选方法。然而,在靠近重要结构,如血管、神经处,在告知患者后,保留功能但有局部复发风险的R2切除(肉眼可见切缘受累)可能是一种可接受的选择,以避免不可避免的截肢。对于肿瘤的临界可切除性,新辅助化疗/放疗有其应用价值。同样,手术切除后也需要进行辅助治疗,但非转移性疾病的化疗仍存在争议。五年生存率可达55%。切除边缘阳性或不确定时考虑再次切除。当前的策略基于新型化疗药物、改进的放疗应用以限制局部副作用以及靶向生物治疗或免疫治疗,包括疫苗。年轻是原发性肿瘤切除后6个月内发生远处转移的危险因素。对于高级别STS(2级或3级),建议进行持续5 - 6周的新辅助放疗和手术切除。对于70岁以上的患者,单独进行广泛手术切除可能是可接受的。然而,局部晚期疾病需要多学科团队进行截肢或保肢的决策。