[Our experience in the treatment of retroperitoneal fibrosis]

Abstract

To discuss retroperitoneal fibrosis (RPF) etiopathogenesis and to report on our experience in the treatment of the disease. From 1977 to 1998 26 RPF patients, 15 idiopathic (I group) and 11 secondary (II group), entered our clinic. Vascular risk factors of the I group were cigarette smoking (73.3%) and arterial hypertension (46.6%). Etiologic factors of the II group were aorta abdominal aneurysm (four cases), radiation therapy for female genital tract cancer (four case), aorto-bifemoral bypass for aorta aneurysm (two cases), retroperitoneal non-Hodgkin lymphoma (one case). Treatment performed for idiopathic disease was medical in eight cases (alone in two, with endourologic measures in six) using corticosteroids in five and tamoxifen in three; only endourological in three and surgical in four (nephrectomy in two patients, ureterolysis and ureteroneocystostomy in one, pyeloureterolysis in one) with perioperative corticosteroid treatment in three cases. Treatments performed in patients with secondary disease were endourologic in five surgical in three (ureteroureterostomy, ureteroneocystostomy, pyeloureterolysis and pyeloreduction), medical with corticosteroids in two; one patient affected by perianeurysmatic fibrosis did not require any treatment because of disease's spontaneous recovery. Medical treatment induced symptom remission and plaque reduction in all patients. Surgery determined complete recovery in all patients except for one in whom the disease relapsed with controlateral urinary tract involvement. Medical RPF treatment for idiopathic or secondary disease is effective in the first stage. Disease management with tamoxifen is easy to perform, safe and effective. In the steady state of RPF the best results are obtained by surgery and perioperative glucocorticoid therapy reduced significantly fibrosis' relapse.