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Case profile: Retroperitoneal fibrosis profile
CASE PROFILE:
RETROPERITONEAL
This case represents a twenty-two-year follow-up of a Caucasian male first seen in 1953 with right flank pain and radiographic changes compatible with right hydroureteronephrosis (Fig. 1A). During exploration of the right side a dense, encased mass of scar tissue was found involving nearly the entire extent of the right retroperitoneum. Because of the extent of the disease and the technical aspects of dealing with the sclerotic tissue, which was thought to represent a malignant process, nephrectomy was performed. Pathologically, the tissue revealed a fibrous, sclerotic process with no identifiable malignancy. When similar symptoms of flank pain developed on the opposite side several months later, a ureteral catheter was passed with ease to the left renal pelvis, and the patient’s pain was relieved. At subsequent exploration a similar fibrous process involving a 4 to 6-cm. area
FIBROSIS
of the left ureter was found, and the ureter was dissected free and placed intraperitoneally. Clinically, the patient has been well and a representative pyelogram obtained twenty-two years later is seen in Figure 1B. There is deviation in the course of this otherwise normal-appearing ureter. Comment Retroperitoneal fibrosis was first described by Ormond in 1948, and by 1960 he had reported 95 known cases. Since that time it has become an increasingly recognized entity. Its cause remains unknown but has been attributed to processes of infection, trauma, urinary extravasation, and vasculitis, among others. The most common presenting symptom is flank pain, but there are no other consistent diagnostic present-
FIGURE 1. (A) Delayed hydronephrosis ureter involved
436
pyelogram obtained in 1953 demonstrating and hydroureter to level of midureter. (B) Pyelogram in retroperitoneal process.
UROLOGY
retention obtained
of contrast twenty-two
/ APRIL 1976
!
material and right years after lysis of
VOLU51E VII.
NUMBER
4
ing signs or symptoms. A hydroureteronephrosis is generally demonstrated and, in fact, a definite point of obstruction may be localized radiographically. A retrograde catheter is usually passed with ease beyond the point of obstruction with relief of pain, but return of pain and decreased urine output are generally noted after removal of the catheter. The most accurate diagnosis remains with exploration and biopsy. Grossly, the lesion represents a dense mass of fibrous tissue often lying in “dense collagen bundles.” Microscopically, various stages of a subacute, nonspecific inflammatory reaction are noted in a fibrofatty background. Cellular elements may comprise polymorphonuclear leukocytes, eosinophils, plasma cells, or pure connective tissue. The most accepted treatment is mobilization of the involved structures away from the retro-
peritoneal fibrous process as long as permanent damage does not necessitate nephroureterectomy. Treated appropriately in this manner, the disease process affecting the urinary tract usually does not recur. In 1965 Suby et al. * related a similar process to an antiserotonin drug, methysergide. The process is reversible after stopping the drug. Radiation treatment, antibiotics, or other chemotherapeutic agents have generally been unsuccessful in alleviating the process.
University
*Suby, H. I., et cd.: Retroperitoneal fibrosis: link in the chain, J. Urol. 93: 144 (1965).
Programs of interest to our readers are welcome from tributions, including an abbreviated history and legendfw thefilms, 34. D., feature editor.
NOTE
Leo A. bClilleman, M.D. David A. Culp, M.D. Department of Urology of Iowa Hospitals and Clinics Iowa City. Iowa 52242
a lnissing
urologists and radiologists. Conare to be sent to Arthur N. Tessler,
437
RETROPERITONEAL
This case represents a twenty-two-year follow-up of a Caucasian male first seen in 1953 with right flank pain and radiographic changes compatible with right hydroureteronephrosis (Fig. 1A). During exploration of the right side a dense, encased mass of scar tissue was found involving nearly the entire extent of the right retroperitoneum. Because of the extent of the disease and the technical aspects of dealing with the sclerotic tissue, which was thought to represent a malignant process, nephrectomy was performed. Pathologically, the tissue revealed a fibrous, sclerotic process with no identifiable malignancy. When similar symptoms of flank pain developed on the opposite side several months later, a ureteral catheter was passed with ease to the left renal pelvis, and the patient’s pain was relieved. At subsequent exploration a similar fibrous process involving a 4 to 6-cm. area
FIBROSIS
of the left ureter was found, and the ureter was dissected free and placed intraperitoneally. Clinically, the patient has been well and a representative pyelogram obtained twenty-two years later is seen in Figure 1B. There is deviation in the course of this otherwise normal-appearing ureter. Comment Retroperitoneal fibrosis was first described by Ormond in 1948, and by 1960 he had reported 95 known cases. Since that time it has become an increasingly recognized entity. Its cause remains unknown but has been attributed to processes of infection, trauma, urinary extravasation, and vasculitis, among others. The most common presenting symptom is flank pain, but there are no other consistent diagnostic present-
FIGURE 1. (A) Delayed hydronephrosis ureter involved
436
pyelogram obtained in 1953 demonstrating and hydroureter to level of midureter. (B) Pyelogram in retroperitoneal process.
UROLOGY
retention obtained
of contrast twenty-two
/ APRIL 1976
!
material and right years after lysis of
VOLU51E VII.
NUMBER
4
ing signs or symptoms. A hydroureteronephrosis is generally demonstrated and, in fact, a definite point of obstruction may be localized radiographically. A retrograde catheter is usually passed with ease beyond the point of obstruction with relief of pain, but return of pain and decreased urine output are generally noted after removal of the catheter. The most accurate diagnosis remains with exploration and biopsy. Grossly, the lesion represents a dense mass of fibrous tissue often lying in “dense collagen bundles.” Microscopically, various stages of a subacute, nonspecific inflammatory reaction are noted in a fibrofatty background. Cellular elements may comprise polymorphonuclear leukocytes, eosinophils, plasma cells, or pure connective tissue. The most accepted treatment is mobilization of the involved structures away from the retro-
peritoneal fibrous process as long as permanent damage does not necessitate nephroureterectomy. Treated appropriately in this manner, the disease process affecting the urinary tract usually does not recur. In 1965 Suby et al. * related a similar process to an antiserotonin drug, methysergide. The process is reversible after stopping the drug. Radiation treatment, antibiotics, or other chemotherapeutic agents have generally been unsuccessful in alleviating the process.
University
*Suby, H. I., et cd.: Retroperitoneal fibrosis: link in the chain, J. Urol. 93: 144 (1965).
Programs of interest to our readers are welcome from tributions, including an abbreviated history and legendfw thefilms, 34. D., feature editor.
NOTE
Leo A. bClilleman, M.D. David A. Culp, M.D. Department of Urology of Iowa Hospitals and Clinics Iowa City. Iowa 52242
a lnissing
urologists and radiologists. Conare to be sent to Arthur N. Tessler,
437