Li Zhi-Ke, Liu Jie, Chen Chen, Yang Ke-Yi, Deng Yao-Tiao, Jiang Yu
Department of Medical Oncology, Cancer Centre, West China Hospital, Sichuan University, Chengdu, China.
Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
J Int Med Res. 2021 Mar;49(3):300060521996940. doi: 10.1177/0300060521996940.
Preoperative diagnosis of solitary fibrous tumour (SFT) may not provide a complete tumour picture and may be inaccurate. There is no standard treatment for locally advanced or metastasised malignant SFT (MSFT). Here, the case of a 17-year-old male patient with final pathology diagnosis of MSFT is reported. Preoperative biopsy pathology results suggested an Ewing sarcoma that was positive for CD99 antigen, vimentin, friend leukaemia integration 1 transcription factor, apoptosis regulator Bcl-2, and synaptophysin; and negative for CD34 antigen, S-100 protein (S-100), smooth muscle antigen, cytokeratin, and Wilms tumour 1 associated protein. The Ki67 positive rate was 8%, so the patient initially received eight cycles of conversion chemotherapy (vincristine, etoposide, ifosfamide and pirarubicin for one cycle, and vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide for 7 cycles in total). The tumour shrunk significantly and was surgically removed. The final pathology diagnosis was MSFT that was positive for CD99 and signal transducer and activator of transcription 6, and negative for CD34, tumour protein 63, S-100, desmin, and epithelial membrane antigen. Fluorescence in situ hybridization showed no gene translocation in EWS RNA binding protein 1, SS18 subunit of BAF chromatin remodelling complex or FUS RNA binding protein. The patient finally accepted adjuvant radiotherapy of 5600 cGy. Disease-free survival has been > 1 year, with no recurrence or metastasis detected to date. MSFT is rare and treatment for locally advanced or metastatic MSFT remains controversial. The efficacy of the present therapeutic strategy requires further research.
孤立性纤维瘤（SFT）的术前诊断可能无法提供完整的肿瘤情况，且可能不准确。对于局部晚期或转移性恶性SFT（MSFT），尚无标准治疗方法。在此，报告一例17岁男性患者，最终病理诊断为MSFT。术前活检病理结果提示为尤因肉瘤，CD99抗原、波形蛋白、Friend白血病整合1转录因子、凋亡调节蛋白Bcl-2和突触素呈阳性；CD34抗原、S-100蛋白（S-100）、平滑肌抗原、细胞角蛋白和威尔姆斯瘤1相关蛋白呈阴性。Ki67阳性率为8%，因此患者最初接受了8个周期的转化化疗（长春新碱、依托泊苷、异环磷酰胺和吡柔比星一个周期，长春新碱、多柔比星以及环磷酰胺/异环磷酰胺和依托泊苷总共7个周期）。肿瘤明显缩小并接受了手术切除。最终病理诊断为MSFT，CD99和信号转导及转录激活因子6呈阳性，CD34、肿瘤蛋白63、S-100、结蛋白和上皮膜抗原呈阴性。荧光原位杂交显示在EWS RNA结合蛋白1、BAF染色质重塑复合物的SS18亚基或FUS RNA结合蛋白中无基因易位。患者最终接受了5600 cGy的辅助放疗。无病生存期已超过1年，迄今未检测到复发或转移。MSFT罕见，局部晚期或转移性MSFT的治疗仍存在争议。目前治疗策略的疗效需要进一步研究。
