後腹腔纖維化:多因素病因的疾病

李彥霓、鄒凱亦、董劭偉、魏汶玲、胡書維、高偉棠、江怡德、

劉家宏、陳冠州、崔克宏、吳佳璋

台北醫學大學-部立雙和醫院泌尿科

Retroperitoneal fibrosis: A disease of multifactorial etiologies

An Ni Lee, Kai-Yi Tzou, Shao-Wei Dong, Wen Ling Wu, Su-Wei Hu, Wei-Tang Kao, Yi-Te Chiang, Chia-Hung Liu, Kuan-Chou Chen, Ke-Hung Tsui, Chia-Chang Wu

Department of Urology, Taipei Medical University-Shuang Ho Hospital, New Taipei City, Taiwan

Abstract:

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of fibro-inflammatory tissue in the retroperitoneum that causes compression of the ureters leading to obstructive uropathy. It can be classified into two groups based on different etiologies: idiopathic retroperitoneal fibrosis, which comprises approximately 70% of cases mainly autoimmune related; and secondary RPF, which is more commonly associated with malignant cases, drugs or radiation therapy induced.

We herein report on two cases of retroperitoneal fibrosis with different etiologies, both of which presented initially with renal function deterioration, bilateral hydronephrosis and hydroureter, and a segmental soft-tissue mass lesion on imaging. In both cases, procedures were performed to relieve urinary obstruction, tissue sampling was obtained, and laparoscopic surgery was done. The first case is a 70-year-old man presented with nausea and vomiting for a week. He was diagnosed with idiopathic IgG4-related disease with RPF, achieved resolution of hydronephrosis after receiving steroid therapy and ureterolysis. The second case is a 73-year-old man presented with abdominal pain and nausea for 2 days. He was diagnosed with secondary RPF related to stage IV urothelial carcinoma after laparoscopic surgery. However, due to worsening of his general condition and failure to be extubated, his family decided to opt for hospice care.

Our cases will show the multifactorial etiologies of RPF, emphasize discussions on the management and treatment efficacy of both idiopathic and secondary RPF based on relevant literature reviews and our experience. We will suggest a possible algorithm for future disease diagnosis and management.