Objective To investigate the clinicopathologic features,immunohistochemical phenotypes,fluorescence in situ hybridization,differential diagnosis and prognosis of patients with TFE3-rearranged renal cell carcinoma(TFE3 rRCC).Methods Clinical data,pathomorphologic features,immunohistochemical phenotypes,and results of fluorescence in situ hybridization were collected and retrospectively analyzed in 16 cases of TFE3 rRCC that had been definitively diagnosed.Results Eleven of the patients were female and five were male,aged 3 to 53 years;the tumours ranged from 3 to 20 cm in diameter.Microscopically,all of them showed characteristic hyaline or eosinophilic tumour cells arranged in a papillary structure,accompanied by a varying proportion of lamellar,nesting or vesicular-like structures.All 16 cases showed nu-clear positivity for TFE3;TFE3 gene breakage signals were present in all 13 cases.The cases in this group were followed up for 2 to 67 months.As of January 2024,only one patient showed brain and new bone metastases,and one patient showed abdominal lymph node metastases,while the rest of the patients were disease-free and survived.Conclusion TFE3 rearranged renal cell carcinoma is a rare malignant tumour,immunohistochemical labelling of TFE3 and fluorescence in situ hybridisation assay are essential for diagnosis,surgery is the treatment of choice;venous cancer embolism,AJCC high stage and TFE3 are all key factors for tumour recurrence,and targeted and immunotherapy are still in clinical trials.
关键词
肾癌/TFE3重排肾细胞癌/免疫组化/荧光原位杂交/预后
Key words
Renal cancer/TFE3 rearrangement renal cell carcinoma/Immunohistochemistry/Fluorescence in situ hy-bridization/Prognosis
维普
万方数据