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Coexistent xanthogranulomatous pyelonephritis and renal cell carcinoma
CT: THE JOURNAL
294
OF COMPUTED
TOMOGRAPHY
1987;
11:294-296
COEXISTENT XANTHOGRANULOMATOUS PYELONEPHRITIS AND RENAL CELL CARCINOMA D. RANDALL RADIN, MD, AND PARAKRAMA CHANDRASOMA,
A patient who presented with coexistent xanthogranulomatous pyelonephritis and renal cell carcinoma is reported. Nine previously reported cases are reviewed and the possible significance of this association is discussed. KEY WORDS:
Kidney, computed tomography, infection, neoplasm; Nephritis
Xanthogranulomatous pyelonephritis (XGP) is an uncommon, pathologically distinct inflammatory process in which renal parenchyma is replaced by sheets of lipid-laden macrophages (1, 2). Although its histogenesis is uncertain, XGP is usually associated with chronic urinary tract obstruction and recurrent infection. A patient with XGP and coexistent renal cell carcinoma (RCC) is described, and nine previously reported cases are reviewed.
CASE
REPORT
A 69-year-old woman was admitted with lower abdominal pain. Diverticulitis of the sigmoid colon was diagnosed 11 years earlier. Sixteen years before admission she was evaluated for recurrent urinary tract infections and underwent excretory urography and renal arteriography. A small, nonfunctioning
From the Departments of Radiology and Surgical Pathology, University of Southern California School of Medicine, Los Angeles, California. Address reprint requests to: D. Randall Radin, MD, Department of Radiology, Los Angeles County-USC Medical Center, 1200 North State Street, Los Angeles, California 90633. Received August 1986. 0 1987 by Elsevier Science Publishing Co., Inc. 52 Vanderbilt Avenue, New York, NY 10017 0149-936X/87/$3.50
MD
left kidney was ascribed to chronic atrophic pyelonephritis. Barium enema examination revealed complete obstruction of the sigmoid colon. Transverse loop colostomy was performed. Sigmoid diverticulitis was diagnosed by colonoscopy and barium enema. Computed tomography (CT) revealed an enlarged, nonfunctioning left kidney with marked hydronephrosis and hydroureter (Figure 1A). Several small calcifications were seen in dilated calices. An 8 cm soft tissue mass was present in the lateral aspect of the pars media. The site of left ureteral obstruction was in the pelvis, where a small irregular low-density collection was seen, corresponding to the location of the sigmoid diverticulitis. Two months later the patient developed left flank pain, fever, leukocytosis, and pyuria. Following an unsuccessful attempt to place a left ureteral stent, a left nephrectomy was performed. Pathological examination showed acute suppurative pyelonephritis as well as chronic and xanthogranulomatous pyelonephritis (Figure 1B). The solid mass seen on CT was an oncocytic renal cell carcinoma without capsular or venous invasion (Figure 1C). DISCUSSION Clinical features of the 10 reported patients with coexistent XGP and RCC (3-5) are similar to those of patients with XGP alone (1, 2). The patients ranged in age from 48 to 78 years and were evenly divided by gender. Patients with XGP are typically middle-aged and show a 2 :1 female predominance. Almost all of the patients with concurrent XGP and RCC presented with recurrent urinary tract infections. Gross hematuria was noted in only one patient. Serum creatinine level was normal in all but two patients. Excretory urography demonstrated a nonfunctioning or poorly functioning kidney in all
JULY
1987
XGP AND RENAL CELL CA
295
FIGURE 1. (A) Postcontrast CT scan of kidneys. Enlarged
left kidney contains dilated calices (C) and pelvis [P) that are displaced by soft-tissue mass I&f). Colostomy is present on right. (B) Photomicrograph of area of xanthogranulomatous pyelonephritis showing macrophages with abundant foamy, vacuolated cytoplasm and scattered lymphocytes. H and E, x 160.(C) Photomicrograph. Renal adenocarcinoma, oncocytic type, showing nuclear pleomorphism and abundant granular cytoplasm that characterize this neoplasm. H and E, x 160.
cases and obstructing calculi in most; the right and left kidneys were involved with equal frequency. RCC occurring in patients with XGP is indistinguishable from that which develops in the general population (3-5). Sporadic RCC is seen most commonly in patients in the fifth to seventh decade with a 3 : 1 male predilection. Histologically, RCC in patients with XGP has been of the clear cell type in 50 percent of cases and of the granular cell or oncocytic type in 50 percent. All stages of RCC are represented in patients with coexistent XGP. Most of the patients expired with local recurrence or distant metastases. The significance of the association of XGP and RCC is unknown. It is possible that their concurrence in the 10 reported patients is coincidental. Since little is known about the causes of XGP and RCC, a single factor or some combination of factors
might be responsible for producing both diseases in these patients. More intriguing, however, is the possibility that the chronic inflammation associated with XGP predisposes to the development of RCC. There are numerous instances of association of chronic inflammation and malignancy: chronic cholecystitis and gallbladder carcinoma, ulcerative colitis and colon carcinoma, Crohn’s disease and carcinoma of the colon and small bowel, and cystitis glandularis and bladder carcinoma, to name a few. Although the vast majority of cases of RCC arise de novo, some cases are seen in patients with von Hippel-Lindau disease (6), polycystic kidney disease (7), and acquired uremic cystic disease of the kidney (8). Perhaps XGP should be added to the list of diseases which are associated with an increased risk for the development of RCC.
CT: THE JOURNAL OF COMPUTED TOMOGRAPHY VOL. 11 NO. 3
296
REFERENCES 1. Malek RS, Elder JS: Xanthogranulomatous pyelonephritis: A
critical analysis of 26 cases and of the literature. J Urol 1978;119:589-93.
2. Tolia BM, Iloreta A, Freed SZ, et al: Xanthogranulomatous py-
elonephritis: detailed analysis of 29 cases and a brief discussion of atypical presentations. J Urol 1981;126:437-42.
3. Piscioli F, Luciani L: Association of xanthogranulomatous py-
elonephritis with small renal cell carcinoma: Case report and review of the literature. Eur Urol 1984;10:62-6.
4. Wadsworth D, Balfe DM: Renal mass, obstructing calculus,
and urinary tract infection (clinical pathologic conference). Ural Radio1 1982;4:266-8.
5. Cowley JP, Connolly CE, Hehir M, O’Beirn SF: Renal carci-
noma with staghorn calculus, perinephric abscess, and xanthogranulomatous pyelonephritis in same kidney. Urology 1983;21:635-8.
6. Levine E, Collins DL, Horton WA, Schimke RN: CT screening
of the abdomen 1982:139:505-10.
in
von
Hippel-Lindau
disease.
AJR
7. Tegtmeyer CJ, Gail W, Wyker AW Jr., Gillenwater JY: Angio-
graphic diagnosis of renal tumors associated with polycystir disease. Radiology 1978;126:105-9.
8. Dunnill MS, Millard PR, Oliver D: Acquired cystic disease of
the kidneys: A hazard of long-term intermittent maintenance haemodialysis. J Clin Path01 1977;30:868-77.
CONTINUING MEDICAL EDUCATION QUESTIONS
the association of xanthogranulomatous pyelonephritis (XGP) and renal cell carcinoma (RCC), the following statement is true a. Their coexistence is coincidental.
1. Concerning
b. The chronic inflammation of XGP predisposes to the development of RCC. c. Both XGP and RCC result from chronic urinary tract obstruction and recurrent infection. d. The significance of the association of XGP and RCC is not known. 2. Which one of the following diseases is not known to be associated with an increased incidence of renal cell carcinoma? a. von Hippel-Lindau disease b. Polycystic kidney disease c. Xanthogranulomatous pyelonephritis d. Acquired uremic cystic disease of the kidney 3. Which one a. Patients women. b. Patients c. Patients younger
of the following statements is false? with XGP are typically middle-aged with RCC are typically middle-aged men. with coexistent XGP and RCC tend to be than patients with sporadic RCC.
4. Which one of the following statements is true? a. In patients with coexistent XGP and RCC, the renal malignancy is usually clinically silent. b. Patients with coexistent XGP and RCC have a more favorable prognosis than patients with sporadic RCC. c. Most patients with coexistent XGP and RCC have gross hematuria.
294
OF COMPUTED
TOMOGRAPHY
1987;
11:294-296
COEXISTENT XANTHOGRANULOMATOUS PYELONEPHRITIS AND RENAL CELL CARCINOMA D. RANDALL RADIN, MD, AND PARAKRAMA CHANDRASOMA,
A patient who presented with coexistent xanthogranulomatous pyelonephritis and renal cell carcinoma is reported. Nine previously reported cases are reviewed and the possible significance of this association is discussed. KEY WORDS:
Kidney, computed tomography, infection, neoplasm; Nephritis
Xanthogranulomatous pyelonephritis (XGP) is an uncommon, pathologically distinct inflammatory process in which renal parenchyma is replaced by sheets of lipid-laden macrophages (1, 2). Although its histogenesis is uncertain, XGP is usually associated with chronic urinary tract obstruction and recurrent infection. A patient with XGP and coexistent renal cell carcinoma (RCC) is described, and nine previously reported cases are reviewed.
CASE
REPORT
A 69-year-old woman was admitted with lower abdominal pain. Diverticulitis of the sigmoid colon was diagnosed 11 years earlier. Sixteen years before admission she was evaluated for recurrent urinary tract infections and underwent excretory urography and renal arteriography. A small, nonfunctioning
From the Departments of Radiology and Surgical Pathology, University of Southern California School of Medicine, Los Angeles, California. Address reprint requests to: D. Randall Radin, MD, Department of Radiology, Los Angeles County-USC Medical Center, 1200 North State Street, Los Angeles, California 90633. Received August 1986. 0 1987 by Elsevier Science Publishing Co., Inc. 52 Vanderbilt Avenue, New York, NY 10017 0149-936X/87/$3.50
MD
left kidney was ascribed to chronic atrophic pyelonephritis. Barium enema examination revealed complete obstruction of the sigmoid colon. Transverse loop colostomy was performed. Sigmoid diverticulitis was diagnosed by colonoscopy and barium enema. Computed tomography (CT) revealed an enlarged, nonfunctioning left kidney with marked hydronephrosis and hydroureter (Figure 1A). Several small calcifications were seen in dilated calices. An 8 cm soft tissue mass was present in the lateral aspect of the pars media. The site of left ureteral obstruction was in the pelvis, where a small irregular low-density collection was seen, corresponding to the location of the sigmoid diverticulitis. Two months later the patient developed left flank pain, fever, leukocytosis, and pyuria. Following an unsuccessful attempt to place a left ureteral stent, a left nephrectomy was performed. Pathological examination showed acute suppurative pyelonephritis as well as chronic and xanthogranulomatous pyelonephritis (Figure 1B). The solid mass seen on CT was an oncocytic renal cell carcinoma without capsular or venous invasion (Figure 1C). DISCUSSION Clinical features of the 10 reported patients with coexistent XGP and RCC (3-5) are similar to those of patients with XGP alone (1, 2). The patients ranged in age from 48 to 78 years and were evenly divided by gender. Patients with XGP are typically middle-aged and show a 2 :1 female predominance. Almost all of the patients with concurrent XGP and RCC presented with recurrent urinary tract infections. Gross hematuria was noted in only one patient. Serum creatinine level was normal in all but two patients. Excretory urography demonstrated a nonfunctioning or poorly functioning kidney in all
JULY
1987
XGP AND RENAL CELL CA
295
FIGURE 1. (A) Postcontrast CT scan of kidneys. Enlarged
left kidney contains dilated calices (C) and pelvis [P) that are displaced by soft-tissue mass I&f). Colostomy is present on right. (B) Photomicrograph of area of xanthogranulomatous pyelonephritis showing macrophages with abundant foamy, vacuolated cytoplasm and scattered lymphocytes. H and E, x 160.(C) Photomicrograph. Renal adenocarcinoma, oncocytic type, showing nuclear pleomorphism and abundant granular cytoplasm that characterize this neoplasm. H and E, x 160.
cases and obstructing calculi in most; the right and left kidneys were involved with equal frequency. RCC occurring in patients with XGP is indistinguishable from that which develops in the general population (3-5). Sporadic RCC is seen most commonly in patients in the fifth to seventh decade with a 3 : 1 male predilection. Histologically, RCC in patients with XGP has been of the clear cell type in 50 percent of cases and of the granular cell or oncocytic type in 50 percent. All stages of RCC are represented in patients with coexistent XGP. Most of the patients expired with local recurrence or distant metastases. The significance of the association of XGP and RCC is unknown. It is possible that their concurrence in the 10 reported patients is coincidental. Since little is known about the causes of XGP and RCC, a single factor or some combination of factors
might be responsible for producing both diseases in these patients. More intriguing, however, is the possibility that the chronic inflammation associated with XGP predisposes to the development of RCC. There are numerous instances of association of chronic inflammation and malignancy: chronic cholecystitis and gallbladder carcinoma, ulcerative colitis and colon carcinoma, Crohn’s disease and carcinoma of the colon and small bowel, and cystitis glandularis and bladder carcinoma, to name a few. Although the vast majority of cases of RCC arise de novo, some cases are seen in patients with von Hippel-Lindau disease (6), polycystic kidney disease (7), and acquired uremic cystic disease of the kidney (8). Perhaps XGP should be added to the list of diseases which are associated with an increased risk for the development of RCC.
CT: THE JOURNAL OF COMPUTED TOMOGRAPHY VOL. 11 NO. 3
296
REFERENCES 1. Malek RS, Elder JS: Xanthogranulomatous pyelonephritis: A
critical analysis of 26 cases and of the literature. J Urol 1978;119:589-93.
2. Tolia BM, Iloreta A, Freed SZ, et al: Xanthogranulomatous py-
elonephritis: detailed analysis of 29 cases and a brief discussion of atypical presentations. J Urol 1981;126:437-42.
3. Piscioli F, Luciani L: Association of xanthogranulomatous py-
elonephritis with small renal cell carcinoma: Case report and review of the literature. Eur Urol 1984;10:62-6.
4. Wadsworth D, Balfe DM: Renal mass, obstructing calculus,
and urinary tract infection (clinical pathologic conference). Ural Radio1 1982;4:266-8.
5. Cowley JP, Connolly CE, Hehir M, O’Beirn SF: Renal carci-
noma with staghorn calculus, perinephric abscess, and xanthogranulomatous pyelonephritis in same kidney. Urology 1983;21:635-8.
6. Levine E, Collins DL, Horton WA, Schimke RN: CT screening
of the abdomen 1982:139:505-10.
in
von
Hippel-Lindau
disease.
AJR
7. Tegtmeyer CJ, Gail W, Wyker AW Jr., Gillenwater JY: Angio-
graphic diagnosis of renal tumors associated with polycystir disease. Radiology 1978;126:105-9.
8. Dunnill MS, Millard PR, Oliver D: Acquired cystic disease of
the kidneys: A hazard of long-term intermittent maintenance haemodialysis. J Clin Path01 1977;30:868-77.
CONTINUING MEDICAL EDUCATION QUESTIONS
the association of xanthogranulomatous pyelonephritis (XGP) and renal cell carcinoma (RCC), the following statement is true a. Their coexistence is coincidental.
1. Concerning
b. The chronic inflammation of XGP predisposes to the development of RCC. c. Both XGP and RCC result from chronic urinary tract obstruction and recurrent infection. d. The significance of the association of XGP and RCC is not known. 2. Which one of the following diseases is not known to be associated with an increased incidence of renal cell carcinoma? a. von Hippel-Lindau disease b. Polycystic kidney disease c. Xanthogranulomatous pyelonephritis d. Acquired uremic cystic disease of the kidney 3. Which one a. Patients women. b. Patients c. Patients younger
of the following statements is false? with XGP are typically middle-aged with RCC are typically middle-aged men. with coexistent XGP and RCC tend to be than patients with sporadic RCC.
4. Which one of the following statements is true? a. In patients with coexistent XGP and RCC, the renal malignancy is usually clinically silent. b. Patients with coexistent XGP and RCC have a more favorable prognosis than patients with sporadic RCC. c. Most patients with coexistent XGP and RCC have gross hematuria.