腎臟原發性惡性單發性纖維瘤合併肺部轉移-案例報告及文獻回顧
鄭百諭、李宏耕、吳維哲、曾一俢、鍾旭東
亞東紀念醫院 外科部 泌尿科
Malignant solitary fibrous tumor in kidney with lung metastasis–a case report and review of literature
Pai-Yu Cheng, Hung-Keng Li, Wei-Che Wu, Yu-Shiou Tseng, Shiu-Dong Chung
Divisions of Urology, Department of Surgery, Far Eastern Memorial Hospital, New Taipei City, Taiwan
Introduction: Malignant solitary fibrous tumor(SFT) accounts 10-15% in solitary fibrous tumor. However, kidney origined malignant SFT is very rare, with only few case reports were published. No treatment consensus was described in local recurrent or unresectable metastatic disease.
Case report: A 70-year-old woman presented with intermittent fever and chills. Obstructive uropathy and urosepsis were found during fever workup. Chest to abdomen CT discovered a 9 cm heterogenous and hypervascular tumor, with equivocal lung nodules. Renal cell carcinoma was impressed clinically. Nephrectomy was performed, and malignant SFT was proved by pathology and immunohistochemistry. Oncologist suggested active surveillance without chemotherapy or targeted therapy. Monthly chest X ray showed stationary lung nodules, and no local recurrence was found from sonography.
Discussion: Surgery remains the gold standard in treating malignant SFT. Higher mitotic figure, renal capsule penetrate in pathology seems to have association with local recurrence and poor outcome. For unresectable metastatic disease, chemotherapy with Doxorubicin-based regimen was published, but no clinical response benefit was observed. Systemic targeted therapy is popular nowadays, with several clinical trials ongoing. Combination therapy with Bevacizumab and Temozolomide, Sunitinib, Combined Figitumumab plus Everolimus, and Pazopanib seem to stabilize tumor growth.
Conclusion: We presented a case with particular clinical presentation. Malignant SFT was proved by histology and immunohistochemistry. Surgical excision of visible tumor lesion remains the mainstream treatment option, and close surveillance should be warranted. For local recurrence or metastatic disease, systemic targeted therapy may be considered.