FOCAL XANTHOGRANULOMATOUS PYELONEPHRITIS ASSOCIATED WITH RENAL CELL CARCINOMA* THOMAS K. HUISMAN, JOHN P SANDS, M.D.
From the Department
M.D.
of Urology, Naval Hospital, San Diego, California
ABSTRACT-A case of focal xanthogranulomatous pyelonephritis (XGP) with an associated renal cell carcinoma is presented. The tumor was discovered incidentally during a general surgical procedure. Subsequent evaluation showed a ,3 cm mass in the lower pole of an otherwise normal kidney with no evidence of calculus or infection. Radical nephrectomy revealed classic findings of focal XGP with coexistent clear cell carcinoma. The literature describing the rare association between these conditions is reviewed.
Xanthogranulomatous pyelonephritis (XGP) has been aptly named “the great imitator” because of its close resemblance clinically, radiologically, angiographically, and pathologically to other renal disorders. Although its classic form has been well described, XGP is also believed t’o exist in a focal form with only partial involvement of renal parenchyma. Less extensive surgical treatment has been advocated for such lesions if the disease can be diagnosed preoperatively. ’ 5 We report the first case of renal cell carcinoma associated with this rare presentation of XGP and advise limited surgical approaches be tempered with the knowledge that these two pathologic entities do coexist.
tient denied previous history of gros,s hematuria, urinary tract infection, or calculus disease. Surgical history included endoscopic excision of a rectal villous adenoma six months prior and exploratory laparotomy for a perforated duodenal ulcer and appendectomy twentythree years prior. Abdominal exploration
Case Report A fifty-seven-year-old white man was admitted to tlhe general surgery service for treatment of a recurrent villous adenoma 6 cm from the anal verge. Medical history included peptic ulcer disease and alcoholic hepatitis thirteen years prior to admission, as well as an eighty pack/year history of cigarette smoking. The pa-
pyelogram FI(;~IKE 1. Intravenous right lower pole renal mass.
.shom
calcified
FIGURE2. (A) Abdominal CT Scan showing right lower pole mass (bottom 2 frames). (B) Close-up view of mass.
during an abdominoperineal resection (APR) of the rectosigmoid colon revealed a palpable 3 cm right lower-pole renal mass which was not further identified during that procedure. Recovery from the APR was uneventful. Postoperatively, the patient underwent an excretory urogram which revealed a calcified right lower-pole mass in an otherwise normally functioning right kidney (Fig. 1). Abdominal
282
CT scan confirmed the presence of a 3 cm mass in the inferior pole of the kidney (Fig. 2). The renal vein, perihilar lymph nodes, the Gerota fascia, and liver were not involved. The patient was admitted to the urology service six weeks after his APR. Findings on physical examination were normal with the exception of a functioning colostomy in the left lower quadrant. Laboratory studies revealed a hemoglobin of 13.9 g, a white blood cell count of 9,800, and serum creatinine of 1.2 mg/dL. Urinalysis showed 0 to 1 white blood cells and no red blood cellslhpf. Admission urine culture was sterile. Chest x-ray films and serum chemistry panels were normal. The patient underwent a right radical nephrectomy through a right flank approach. Gross examination of the specimen demonstrated a well-circumscribed 3 x 3 cm bright yellow mass involving the lower pole (Fig. 3). Microscopic examination revealed the tumor to be enclosed in a fibrous pseudocapsule. The
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yellowi.sh area contained the characteristic features of xanthogranulomatous pyelonephritis: collections of large foamy macrophages admixed with numbers of lymphocytes, plasma cells, and neutrophils (Fig. 4A, R). Throughout the mass, intermixed with the intense inflammatory infiltrate, was a tumor composed of cells forming papillary projections, glomeruloid bodies, and sheets of pleomorphic cells with scant eosinophilic cytoplasm (Fig. 4C, 11). The tumor did not penetrate the renal capsule. The surgical margins, renal vein, and associated lymph nodes were free of tumor. The patient’s postoperative course was unremarkable. He has been followed for one year and remains free of recurrent or metastatic disease. Comment Xanthogranulomatous pyelonephritis (XGP) is a chronic form of renal infection, characterized by the destruction and replacement of renal parenchyma bv solid sheets of lipid-laden macrophages. The -inflammation begins within the pelvis and calices, with subsequent mucosal destruction and extension into the adjacent renal parenchyma. In the majority of cases. renal
involvement is diffuse, and patients classically present with a nonfunctioning, chronically infected kidney associated with calculi in the collecting system .’ In up to 17 percent of cases, however, the kidney may show parti.al or focal involvement with XGP, with changes confined to the cortex without involvement of the collecting system. 2 Focal XGP has been described as a distinct entity which should be distinguished from the diffuse or segmental form of the disease. It has been postulated to arise as a result of altered host immunity and a failure of the body’s immune system to resolve a lowgrade infection within the renal parenchyma.3.4 It may also represent an early stage of the more diffuse form, with further destruction of the renal parenchyma possibly averted by ‘early local excision. 5 The clinical characteristics, laboratory findings, and bacteriologic studies in focal XGP are similar to those in patients with diffuse XGP The radiographic findings may include a nonfunctioning renal mass with or without calcification, and thereby may simulate renal cell carcinoma. As in diffuse XGP, the feature that may distinguish it from carcinoma is the presence of upper tract calculi or urinary tract infection. These findings are not always present,, however. In a large series of 29 cases of XGP Tolia et aL6 reported on 5 patients with the focal form of the disease, 2 of whom had negative urine cultures and 3 with no upper tract calculi. To date, there have been 7 cases noting the association of XGP and renal cell carc:inoma.4,7.R The first 2 cases reported were later discounted as being “unconvincing” due to a lack of supporting microscopic pathologic illustrations.s In 3 of the remaining 5 cases, the kidney had been completely replaced by tumor and the inflammatory process, and the patient died shortly after nephrectomy of diffuse metastatic disease.“,ifl In 2 of the reported cases altlhough the entire kidney was destroyed by the inflammatory process, the renal cell carcinoma was limited to focal areas within the kidney.*.” In all of the case reports accompanied by microscopic pathologic illustrations, the renal cell tumor was of the granular cell type.’ ” Our case report is the first to demonstrate a renal cell carcinoma in association with a focal XGP, although XGP has previously been noted to exist in association with transitional1 cell carcinoma of the pelvis. 5 A number of recent articles have advocated the use of either computerized tomography scanI or a combination of
Low power views demonstrating (A) interface between normal kidney (above) and tumor (beFIGURE 4. low); (B) area of central necrosis and cholesterol crystal surrounded by foamy macrophages, classic appearance of XGF! (C) C arcinoma forming glomeruloid bodies with adjacent clumps of foamy macrophages. (D) High power view of tumor cells demonstrating pleomorphic nuclei with nucleoli and scant eosinophilic cytoplasm. Also note adjacentfoamy macrophages and inflammatory cell infiltrate. (Hematoxylin and eosin, [A, B, C] x 50; [D] x 300.)
ultrasound and needle aspiration13 in the preoperative diagnosis of focal XGP, thereby possibly altering the operative approach to definitive treatment. Based on our finding of renal cell carcinoma in association with a focal lesion of XGP, we concur with the management plan as previously outlined by Schoborg and associatesg and advocate radical nephrectomy for the majority of such lesions. Partial nephrectomy should be limited to those cases in which maximal preservation of renal parenchyma is mandatory (as with solitary units) and only after appropriate vascular control is achieved and with the aid of liberal biopsy and frozen section examination. San Diego, California
921345000 (DR. SANDS)
References 1. Elder JS: Xanthogranulomatous pyclonephritis and gas forming infections of the urinary tract, AUA Update Series 3: 31 (1984). 2. Elder JS, Marshall FF: Focal aanthogranulomatous pyclonephritis in adulthood, Johns Hopkins Med J 146: 141 (1980).
284
3. Hartman DS: Radiologic pathologic correlation of the infectious granulomatous disease of the kidney, Monogr Ural 6: 2 (1985). 4. Mering JH, Kaplan CW, and McLaughlin HP III: Xanthogranulomatous pyelonephritis: unusual clinical presentations, Urology 1: 338 (1973). 5. Tolia BM, ct 01: Xanthogranulomatous pyeloncphritis; segmental or generalized disease? J Urol 124: 122 (1986). 6. Tolia BM, et al: Xanthogranulomatous pyelonephritis: detailed analysis of 29 cases and a brief discussion of atypical presentations, J Urol 126: 437 (1981). 7. Elliott CR, Johnson HW, and Balfour JA: Xanthogranulomatous pyelonephritis and perirenal xanthogranuloma, Br J Urol 40: 548 (1968). 8. Piscioli F, and Luciani I,: Association of xanthogranlllonratous pyclonephritis with small renal cell carcinoma: cast’ report and review of the literature, Eur Urol 10: 62 (1984). 9. Schoborg TW, Saffos HD, Urdancta I,, and Lewis CV2’: Xanthogranulomatous pyelonephritis associated with renal carcinoma, J Ural 124: 125 (1980). 10. Lorentzen M. and Overgaard NII: Xanthogrannlorrlatorls pyelonephritis, Stand J Urol Nephrol 14: 193 (1980). 11. Coulding FJ, and Moser A: Xanthogranulomatous pyclonephritis with associated renal cell carcinoma, Urology 23: 38.5 (1984). 12. Claes H, Vereecken R, Oyen R, and Van Darnme B: Xanthogranulomatous pyeloncphritis with emphasis on computerized tomography scan: retrospective study of 20 cases and Iitcraturc review. Uroloa 29: 389 (19X7). 13. Sees WE, Elyaderani MK, and Bclis JA: Ultrasonograph) and needle aspiration in diagnosis of xanthogrannlomatorrs pyelonephritis, Urology 29: 2.31 (1987).
UHOI,OCY
: MARCII
1992
:
V01,UME
XXXIN. NUMBER
3
From the Department
M.D.
of Urology, Naval Hospital, San Diego, California
ABSTRACT-A case of focal xanthogranulomatous pyelonephritis (XGP) with an associated renal cell carcinoma is presented. The tumor was discovered incidentally during a general surgical procedure. Subsequent evaluation showed a ,3 cm mass in the lower pole of an otherwise normal kidney with no evidence of calculus or infection. Radical nephrectomy revealed classic findings of focal XGP with coexistent clear cell carcinoma. The literature describing the rare association between these conditions is reviewed.
Xanthogranulomatous pyelonephritis (XGP) has been aptly named “the great imitator” because of its close resemblance clinically, radiologically, angiographically, and pathologically to other renal disorders. Although its classic form has been well described, XGP is also believed t’o exist in a focal form with only partial involvement of renal parenchyma. Less extensive surgical treatment has been advocated for such lesions if the disease can be diagnosed preoperatively. ’ 5 We report the first case of renal cell carcinoma associated with this rare presentation of XGP and advise limited surgical approaches be tempered with the knowledge that these two pathologic entities do coexist.
tient denied previous history of gros,s hematuria, urinary tract infection, or calculus disease. Surgical history included endoscopic excision of a rectal villous adenoma six months prior and exploratory laparotomy for a perforated duodenal ulcer and appendectomy twentythree years prior. Abdominal exploration
Case Report A fifty-seven-year-old white man was admitted to tlhe general surgery service for treatment of a recurrent villous adenoma 6 cm from the anal verge. Medical history included peptic ulcer disease and alcoholic hepatitis thirteen years prior to admission, as well as an eighty pack/year history of cigarette smoking. The pa-
pyelogram FI(;~IKE 1. Intravenous right lower pole renal mass.
.shom
calcified
FIGURE2. (A) Abdominal CT Scan showing right lower pole mass (bottom 2 frames). (B) Close-up view of mass.
during an abdominoperineal resection (APR) of the rectosigmoid colon revealed a palpable 3 cm right lower-pole renal mass which was not further identified during that procedure. Recovery from the APR was uneventful. Postoperatively, the patient underwent an excretory urogram which revealed a calcified right lower-pole mass in an otherwise normally functioning right kidney (Fig. 1). Abdominal
282
CT scan confirmed the presence of a 3 cm mass in the inferior pole of the kidney (Fig. 2). The renal vein, perihilar lymph nodes, the Gerota fascia, and liver were not involved. The patient was admitted to the urology service six weeks after his APR. Findings on physical examination were normal with the exception of a functioning colostomy in the left lower quadrant. Laboratory studies revealed a hemoglobin of 13.9 g, a white blood cell count of 9,800, and serum creatinine of 1.2 mg/dL. Urinalysis showed 0 to 1 white blood cells and no red blood cellslhpf. Admission urine culture was sterile. Chest x-ray films and serum chemistry panels were normal. The patient underwent a right radical nephrectomy through a right flank approach. Gross examination of the specimen demonstrated a well-circumscribed 3 x 3 cm bright yellow mass involving the lower pole (Fig. 3). Microscopic examination revealed the tumor to be enclosed in a fibrous pseudocapsule. The
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/
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1992
/
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yellowi.sh area contained the characteristic features of xanthogranulomatous pyelonephritis: collections of large foamy macrophages admixed with numbers of lymphocytes, plasma cells, and neutrophils (Fig. 4A, R). Throughout the mass, intermixed with the intense inflammatory infiltrate, was a tumor composed of cells forming papillary projections, glomeruloid bodies, and sheets of pleomorphic cells with scant eosinophilic cytoplasm (Fig. 4C, 11). The tumor did not penetrate the renal capsule. The surgical margins, renal vein, and associated lymph nodes were free of tumor. The patient’s postoperative course was unremarkable. He has been followed for one year and remains free of recurrent or metastatic disease. Comment Xanthogranulomatous pyelonephritis (XGP) is a chronic form of renal infection, characterized by the destruction and replacement of renal parenchyma bv solid sheets of lipid-laden macrophages. The -inflammation begins within the pelvis and calices, with subsequent mucosal destruction and extension into the adjacent renal parenchyma. In the majority of cases. renal
involvement is diffuse, and patients classically present with a nonfunctioning, chronically infected kidney associated with calculi in the collecting system .’ In up to 17 percent of cases, however, the kidney may show parti.al or focal involvement with XGP, with changes confined to the cortex without involvement of the collecting system. 2 Focal XGP has been described as a distinct entity which should be distinguished from the diffuse or segmental form of the disease. It has been postulated to arise as a result of altered host immunity and a failure of the body’s immune system to resolve a lowgrade infection within the renal parenchyma.3.4 It may also represent an early stage of the more diffuse form, with further destruction of the renal parenchyma possibly averted by ‘early local excision. 5 The clinical characteristics, laboratory findings, and bacteriologic studies in focal XGP are similar to those in patients with diffuse XGP The radiographic findings may include a nonfunctioning renal mass with or without calcification, and thereby may simulate renal cell carcinoma. As in diffuse XGP, the feature that may distinguish it from carcinoma is the presence of upper tract calculi or urinary tract infection. These findings are not always present,, however. In a large series of 29 cases of XGP Tolia et aL6 reported on 5 patients with the focal form of the disease, 2 of whom had negative urine cultures and 3 with no upper tract calculi. To date, there have been 7 cases noting the association of XGP and renal cell carc:inoma.4,7.R The first 2 cases reported were later discounted as being “unconvincing” due to a lack of supporting microscopic pathologic illustrations.s In 3 of the remaining 5 cases, the kidney had been completely replaced by tumor and the inflammatory process, and the patient died shortly after nephrectomy of diffuse metastatic disease.“,ifl In 2 of the reported cases altlhough the entire kidney was destroyed by the inflammatory process, the renal cell carcinoma was limited to focal areas within the kidney.*.” In all of the case reports accompanied by microscopic pathologic illustrations, the renal cell tumor was of the granular cell type.’ ” Our case report is the first to demonstrate a renal cell carcinoma in association with a focal XGP, although XGP has previously been noted to exist in association with transitional1 cell carcinoma of the pelvis. 5 A number of recent articles have advocated the use of either computerized tomography scanI or a combination of
Low power views demonstrating (A) interface between normal kidney (above) and tumor (beFIGURE 4. low); (B) area of central necrosis and cholesterol crystal surrounded by foamy macrophages, classic appearance of XGF! (C) C arcinoma forming glomeruloid bodies with adjacent clumps of foamy macrophages. (D) High power view of tumor cells demonstrating pleomorphic nuclei with nucleoli and scant eosinophilic cytoplasm. Also note adjacentfoamy macrophages and inflammatory cell infiltrate. (Hematoxylin and eosin, [A, B, C] x 50; [D] x 300.)
ultrasound and needle aspiration13 in the preoperative diagnosis of focal XGP, thereby possibly altering the operative approach to definitive treatment. Based on our finding of renal cell carcinoma in association with a focal lesion of XGP, we concur with the management plan as previously outlined by Schoborg and associatesg and advocate radical nephrectomy for the majority of such lesions. Partial nephrectomy should be limited to those cases in which maximal preservation of renal parenchyma is mandatory (as with solitary units) and only after appropriate vascular control is achieved and with the aid of liberal biopsy and frozen section examination. San Diego, California
921345000 (DR. SANDS)
References 1. Elder JS: Xanthogranulomatous pyclonephritis and gas forming infections of the urinary tract, AUA Update Series 3: 31 (1984). 2. Elder JS, Marshall FF: Focal aanthogranulomatous pyclonephritis in adulthood, Johns Hopkins Med J 146: 141 (1980).
284
3. Hartman DS: Radiologic pathologic correlation of the infectious granulomatous disease of the kidney, Monogr Ural 6: 2 (1985). 4. Mering JH, Kaplan CW, and McLaughlin HP III: Xanthogranulomatous pyelonephritis: unusual clinical presentations, Urology 1: 338 (1973). 5. Tolia BM, ct 01: Xanthogranulomatous pyeloncphritis; segmental or generalized disease? J Urol 124: 122 (1986). 6. Tolia BM, et al: Xanthogranulomatous pyelonephritis: detailed analysis of 29 cases and a brief discussion of atypical presentations, J Urol 126: 437 (1981). 7. Elliott CR, Johnson HW, and Balfour JA: Xanthogranulomatous pyelonephritis and perirenal xanthogranuloma, Br J Urol 40: 548 (1968). 8. Piscioli F, and Luciani I,: Association of xanthogranlllonratous pyclonephritis with small renal cell carcinoma: cast’ report and review of the literature, Eur Urol 10: 62 (1984). 9. Schoborg TW, Saffos HD, Urdancta I,, and Lewis CV2’: Xanthogranulomatous pyelonephritis associated with renal carcinoma, J Ural 124: 125 (1980). 10. Lorentzen M. and Overgaard NII: Xanthogrannlorrlatorls pyelonephritis, Stand J Urol Nephrol 14: 193 (1980). 11. Coulding FJ, and Moser A: Xanthogranulomatous pyclonephritis with associated renal cell carcinoma, Urology 23: 38.5 (1984). 12. Claes H, Vereecken R, Oyen R, and Van Darnme B: Xanthogranulomatous pyeloncphritis with emphasis on computerized tomography scan: retrospective study of 20 cases and Iitcraturc review. Uroloa 29: 389 (19X7). 13. Sees WE, Elyaderani MK, and Bclis JA: Ultrasonograph) and needle aspiration in diagnosis of xanthogrannlomatorrs pyelonephritis, Urology 29: 2.31 (1987).
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: MARCII
1992
:
V01,UME
XXXIN. NUMBER
3
