恶性肾孤立性纤维瘤合并胸椎转移临床特征分析(附1例报告并文献复习)

Clinical characteristics of malignant solitary fibrous tumors of the kidney with thoracic vertebral metastasis(A case report and literature review)

目的:探讨恶性肾孤立性纤维瘤(solitary fibrous tumor,SFT)合并胸椎转移的临床病理特征、诊断及治疗方法。方法:回顾性分析1例恶性肾SFT合并胸椎转移患者的疾病进展过程、诊断及治疗措施,影像学及组织病理学特征等,并进行文献回顾。结果:临床及影像学诊断为肾癌。病理检查提示肾中部见一肿物,大小5.5cm×4.0cm×4.0cm,切面灰白质偏硬,侵及肾被膜及肾盂黏膜,肿物与周围界限尚清。免疫组化显示肿瘤细胞:Bcl-2(灶状+)、CD34(++)、S-100(-)、Vimentin(++)、CD117(-)、CK(-),诊断为恶性SFT。于2010年4月22日在全麻下行后腹腔镜下右肾输尿管全长切除术,术后痊愈出院。术后3个月出现腰背部疼痛,确诊为胸椎转移瘤,于2010年7月28日在全麻下行胸椎管减压,椎管内肿瘤切除,胸10腰2椎体骨水泥灌注,椎弓根钉棒内固定术,术后病理提示椎管内组织见梭形细胞肿瘤组织浸润,结合形态及病史,考虑为恶性SFT复发转移。免疫组化染色显示肿瘤细胞:Bcl-2(-),CD34(-),S-100(-),SMA(+),Vimentin(+),CK(+)。术后2个月,患者出现全身多发转移,病情恶化,经医治无效去世。结论:恶性肾SFT是一种非常罕见的恶性肿瘤,其诊断主要依靠磁共振、组织病理学及免疫组织化学,并应与肾的其他梭形细胞肿瘤鉴别,治疗以手术切除为主。

Objective：To investigate clinical and pathological features, diagnosis and treatment of malignant sol- itary fibrous tumors of the kidney with thoracic vertebral metastasis. Method： A case of malignant solitary fibrous tumor of the kidney with thoracic vertebral metastasis was retrospectively analyzed. The progression, diagnosis and treatment,imaging and histopathological characteristics of the disease were investigated, and a literature review was conducted. Result： A 60-year-old male patient was diagnosed clinically and via imaging with renal carcinoma. Renal biopsy revealed a 5.5x4x4cm mass in the middle portion of the kidney,which has also invaded the renal capsule and mucosa of the renal pelvis. Immunohistoehemical examination revealed that the tumor cells were positive for Bcl-2 （focus-like ＋ ）, CD34 （ ＋ ＋ ）, and Vimentin（ ＋ ＋ ） and negative for S-100 （ - ）, CD117 （ - ）, and CK （ - ）. The pa- tient was diagnosed with a malignant solitary fibrous tumor of the kidney. The patient underwent right laparoscopic radical nephroureterectomy under general anesthesia. Three months following surgery, the patient was subsequent- ly diagnosed with thoracic vertebral metastasis. He underwent decompression of the thoracic spinal canal and resec- tion of the intracanal tumor under general anesthesia. Postoperative pathology suggested spindle-cell tumor tissue infiltration in the intracanal tissue. Recurrence and metastasis of the malignant solitary fibrous tumor was consid ered in combination with tumor morphology and medical history. Immunohistochemical staining revealed that tumor cells were negative for Bcl-2 （ - ）, CD34 （ - ）, and S- 100 （ - ）, and positive for SMA （ ＋ ）, Vimentin （ ＋ ）, and CK （＋）. The patient experienced multiple systemic metastases and disease progression 2 months after surgery, failed to respond to any medical treatment,and died. Conclusion ： Malignant solitary fibrous tumor of the kidney is a very rare malignant tumor,and its diagnosis primarily depends on histopathology and immunohistochemistry. It should be differentiated from other spindle cell tumors of the kidney,and surgical excision is the preferred treatment.