盆腔腹膜后孤立性纤维性肿瘤2例并文献复习

Two Cases of Solitary Fibrous Tumors in the Retroperitoneum of the Pelvic Cavity and Literature Review

摘要: 目的：提高对孤立性纤维性肿瘤(solitary fibrous tumor, SFT)的认识。方法：对2例源自盆腔腹膜后的SFT病人的诊疗经过及随访进行分析并复习相关文献。结果：2例患者影像学提示盆腔巨大肿块，接受根治性手术切除，肿瘤检材镜下均符合SFT诊断，术后随访患者现一般情况均良好。结论：SFT是一种罕见疾病，其发生部位非常广泛，并且SFT的临床表现及影像学表现均无特异性，确诊主要依赖病理形态学特征及免疫表型。手术是SFT的首选治疗手段，因SFT肿瘤表面及周围血供通常都较为丰富，手术难度大，术前建议采取MDT诊疗模式，做好充分的术前准备。术中采取髂内动脉结扎(Iliac Internal Artery Ligation, IIAL)联合间断髂总动脉阻断(Intermittent Common Iliac Artery Occlusion, ICIAO)可降低术中出血量。通过手术达到完整切除、切缘阴性的SFT预后较好，但其复发及进展可能是缓慢的，术后仍需长期管理及随访。

Abstract: Objective: To improve the understanding of solitary fibrous tumor (SFT). Methods: The diagnosis and treatment processes and follow-up of 2 patients with SFT originating from retroperitoneum of the pelvic cavity were analyzed, and the relevant literature was reviewed. Results: Imaging findings of 2 patients indicated huge pelvic masses. Radical surgical resection was performed. The tumor samples were all in line with the diagnosis of SFT under the microscope. The general conditions of the patients were good during the postoperative follow-up. Conclusion: SFT is a rare disease with a very wide range of occurrence sites. Moreover, the clinical manifestations and imaging manifestations of SFT are not specific. The diagnosis mainly relies on pathological morphological characteristics and immunophenotypes. Surgery is the preferred treatment method for SFT. Since the blood supply on the surface and around the tumor of SFT is usually abundant, the surgery is difficult. It is recommended that the MDT diagnosis and treatment model be adopted before the operation and adequate preoperative preparations are made. Intraoperative Iliac Internal Artery Ligation (IIAL) combined with Intermittent Common Iliac Artery Occlusion (ICIAO) can reduce intraoperative blood loss. SFT that achieves complete resection through surgery and has a negative resection margin has a better prognosis. However, its recurrence and progression may be slow, and long-term management and follow-up are still required after the operation.