Lymphosarcoma and Periureteral Fibrosis

Abstract

Numerous terms have been applied to the condition resulting from fibrotic tissue surrounding, compressing, and occasionally occluding one or both ureters (3, 11, 14, 16, 20, 22). While the process usually involves the mid or upper portions of the ureters, involvement of the lower ureters, retroperitoneal vessels, kidneys, bladder, lungs, and mediastinum has been reported (1, 3, 5, 6, 18, 19). Multiple nonneoplastic etiologies have been surmised (3, 4, 6–8, 10, 11, 13, 15, 17, 22). Reticulum-cell sarcoma and Hodgkin's disease, however, have been implicated (9, 21). We wish to report 4 cases in which periureteric fibrosis was found in association with lymphosarcoma, 3 of which were without antecedent irradiation to the retroperi-toneum. Case I: In this 68-year-old white woman generalized adenopathy developed in 1960. Biopsy of a left axillary node disclosed lymphosarcoma (Fig. 1). Intravenous pyelography in November 1960 and again in January 1961 revealed bilateral hydronephrosis and proximal hydroureter (Fig. 2). Right hydronephrosis was known to have been present since 1952, but earlier films were not available for comparison. In February 1961 the right kidney and ureter were explored. The kidney appeared normal, but the renal pelvis was markedly dilated. The proximal portion of the ureter was dilated and encased in fibrous tissue. The ureter was freed of the fibrotic cover, and nephrostomy was performed. On biopsy, the tissue enveloping the ureter was originally interpreted as being fibrous, but was recognized on review as lymphosarcoma with desmoplastic reaction. Multiple intravenous pyelographic studies over the course of the next year showed some improvement. In April 1961, radiation therapy was administered to massively enlarged inguinal nodes. In July 1962, involvement of the right tibia developed, responding to irradiation. Right chylothorax has required several thoracenteses. Currently, the renal function appears adequate. Case II: A 56-year-old white man was hospitalized in March 1961, with left costovertebral angle pain of one month duration. Physical examination disclosed left costovertebral angle tenderness and a palpably enlarged left kidney. Blood urea nitrogen was 70 mg. per 100 ml. Retrograde pyelography disclosed bilateral hydronephrosis and proximal hydroureter with tapered segments of ureteral narrowing just proximal to the iliac crests (Figs. 3 and 4). A diagnosis of retroperitoneal fibrosis was made and confirmed at exploration a few days later. The ureter could not be identified because of the extensive fibrosis. Efforts at ureterolysis were abandoned and a right nephrostomy was performed. Biopsies of both renal and periureteral tissue disclosed lymphosarcoma with marked fibrosis of the latter (Fig. 5). Radiation therapy to the retroperitoneum was instituted, 2,500 r of rotational cobalt therapy being administered.