Bilateral Solid Renal Masses in a Young Man

0022-5347 /86/1352-0327$02,00/Q ~/.ol. 135, February

THE JOURNh.L OF UROLOGY

Copyright© 1986 'Jy The VVillia1ns & \1/ilkins Co.

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Clinicopa BILATERAL SOLID RENAL MASSES IN A YOUNG MAN S. W. SIEGEL, R RISIUS, R. TUBBS, K. V. SHEPARD

AND

J. E. PONTES*

From the Cleveland Clinic Foundation, Cleveland, Ohio

The ureter was normal and the renal pelvis contained a shaggy brown mass that was consistent with old blood clot on biopsy. Cytology studies were abnormal, consisting of atypical cells. An abdominal computerized tomogram (CT) was performed. Doctor Risius. The CT scan without contrast medium demo~strated ~ curvilinear opacity in the mid portion of the right kidney, which could have been retained contrast material from the previous retrograde injection. A large mass involving the mid and upper portions of the right kidney also was identified. Following administration of contrast medium this mass was demonstrated to be of decreased density compared to the surrounding renal parenchyma. The mass was solid and inhomogeneous in nature, and projected beyond the expected confines of the normal renal pelvis. On the left side the-re was a small, well defined, soft tissue mass that was entirely intraparenchymal and did not involve the collecting system. The remainder of the study was normal, without evidence of retroperitoneal adenopathy or liver abnormalities (fig. 3). Doctor Siegel. Bilateral renal arteriograms then were done. Doctor Risius. The left renal arteriogram was normal. The intraparenchymal mass that was demonstrated on CT was not identified. The selective right renal arteriogram demonstrated draping and effacement of the mid and upper pole branches around an avascular mass (fig. 4). Doctor Siegel. At this point a percutaneous aspiration needle biopsy was obtained from the left posterior re-nal mass. The aspiration was positive for malignant cells of indeterminate type. The evaluation was completed with a head CT scan which was negative. The patient then was referred to ou; institution for further evaluation and treatment. Approximately 1 week after the initial presentation the patient was asymptomatic and had a negative physical examination. Repeat blood counts and SMA studies were within normal limits. Urinalysis revealed persistent rnicrohematuria and urine culture was negative. A bone scan and venacavogram were

PRESENTATION OF CASEt

Dr. S. W. Siegel. A 29-year-old man presented to the local emergency room with complaints of colicky right flank pain associated with nausea, vomiting and diarrhea. These symptoms began abruptly and were approximately 12 hours in duration. The patient reported a single episode of pink discoloration of the urine several weeks previously but currently denied gross hematuria. He also denied a history of renal calculi, urinary tract infections or voiding complaints. A lumbar laminectomy had been performed previously. Otherwise, health was excellent. Review of the systems was negative and the patient specifically denied weakness, weight loss, anorexia, sweats or fever. He had no known allergies and was on no medication. The patient appeared acutely ill and anxious but otherwise he was well built and well nourished. The temperature was 37.SC, pulse regular at 100 beats per minute and blood pressure 156/60 mm. Hg. Physical examination was remarkable for right abdominal tenderness and guarding, and marked right costovertebral angle tenderness. There was no evidence of abdominal masses or organomegaly. No adenopathy was noted. The hematocrit was 46 per cent and the white blood count was 18,600 with a mild left shift. Blood urea nitrogen was 19 mg./dl. and serum creatinine was 1.1 mg./dl. Serum electrolytes and liver function tests were normal. Urinalysis revealed 6 to 10 white and 4 to 6 red blood cells per high power field. Initial urine culture was positive for group B f3 streptococcus. A chest x-ray and electrocardiogram were normal. The presumed diagnosis was acute pyelonephritis with a possible right renal calculus. Antibiotic therapy was initiated at this point and the diagnostic evaluation proceeded with an excretory urogram (IVP). DISCUSSION OF RADIOGRAPHIC FINDINGS

Dr. B. Risius. The ,v,aun"~'Y film of the IVP revealed no abnormal calcifications over the or course of the tract. On the 5-minute film there was significant delay in function of the right kidney compared to the left kidney. By 10 minutes there was opacification of the upper and lower pole calices on the right side, and these were dilated and effaced around an apparent mid polar mass. The left kidney and collecting system were normal (fig. 1). Doctor Siegel. Cystoscopy and right retrograde pyelography revealed a normal bladder except for some inflammatory changes noted around the right ureteral orifice. Doctor Risius. The right retrograde pyelogram also demonstrated calicectasis and caliceal effacement caused by a central renal mass. There also was a filling defect at the ureteropelvic junction. The remainder of the right ureter was normal (fig. 2). Doctor Siegel. At this point pelvic cytological washings and brushings were obtained, and ureteropyeloscopy was performed. *Requests for reprints: Department of Urology The Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, Ohio 44106. t Case present~d at monthly Clinicopathological Conference of the Sectwn of Urolog1c Oncology, Cleveland Clinic, March 18, 1985.

DIFFERENTIAL DIAGNOSIS

Doctor Risius. Upon review of all of the radiographic and correlating these with the clinical history and examination the differential diagnosis should be limited to disease processe~ that present with bilateral solid renal masses with involvement of the collecting system. Such entities include metastatic renal tumors, renal cell carcinoma, Wilms tumor and lymphoma. Dr. J. E. Pontes. This patient presented with bilateral renal masses proved on aspiration biopsy to be malignant. Bilateral renal tumors are either metastatic or primary. Among primary tumors bilateral adult Wilms tumor may present with the aforementioned clinical findings, although it is rare and only a single case of bilateral adult Wilms tumor has been described. 1 I have seen 4 cases of adult Wilms tumors, none of which was bilateral. The radiological aspects of these cases resemble this presentation. Among metastatic tumors lung carcinoma is the most common, 2 although unlikely in this case in view of the

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patient age and negative chest radiograph. Another possibility is renal involvement by lymphoproliferative disease. Although there is no evidence of systemic involvement, one rarely may see primary renal involvement as the initial manifestation of lymphomas. 3 DISCUSSION OF SURGERY PERFORMED

Doctor Siegel. Our intention was to perform an abdominal exploration and right renal biopsy to establish the diagnosis and to determine if these tumors were amenable to a radical operation. The patient was explored through an anterior chevron incision. The intraperitoneal contents were normal except

for the mesentery, which was studded with small lymph nodes. One of these nodes was biopsied and found to be normal. The right kidney then was mobilized within Gerota's fascia and a firm mass was palpated over its posterosuperior aspect. The exposed surface of the mass was smooth and pink, and a wedge biopsy was obtained. The frozen section revealed a small cell malignancy consistent with nonHodgkin's lymphoma. At this point the procedure was concluded and consultation was obtained from the oncology service. Later, a bone marrow biopsy was normal. DISCUSSION OF PATHOLOGICAL FINDINGS

Dr. R. Tubbs. The mass excised from the kidney replaced the renal parenchyma. Occasional intact renal tubules were apparent. The neoplasm lacked architectural differentiation, growing as individual cells without formation of syncytia, or rosettes or spindling component (fig. 5). Rather, individual lymphoid cells with inconspicuous cytoplasm, and frequent mitoses and focal

FIG. 1. IVP, IO-minute film, shows right hydronephrosis with mid polar mass. Left side is normal.

FIG. 2. Right retrograde pyelogram, drainage film, shows intrapelvic filling defect and associated calicectasis. Distal ureter is normal.

FIG. 3. A, CT scan reveals large solid mid and upper polar mass on right side that is of decreased density compared to surrounding renal parenchyma after contrast material administration. Mass involves parenchyma and pelvis. B, higher cut reveals intraparenchymal mass on left side. Aspiration needle biopsy was obtained from this lesion.

BILATERAL SOLID RENAL MASSES IN YOUNG MAN

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FIG. 4. A, Right renal arteriogram reveals simple renal artery with draping of mid and upper pole branches over central, avascular mass. B, left renal arteriogram is normal.

FIG. 5. Photomicrograph shows malignant lymphoma of high grade, small noncleaved cell type (International Working Formulation) involving kidney. 85 fixed tissue, H & E, reduced from X640.

cellular necrosis comprised the tumor. Cell size was relatively small. Nuclei were round or oval with minimal nuclear clefting in a few cells. Small nucleoli in nuclei with dispersed chromatin were identified. Based on the cytopathological features, the process was classified as a malignant lymphoma of high grade, small noncleaved cell type, using the International Working Formulation. 4 The neoplasm also could be designated as undifferentiated nonBurkitt's lymphoma in the modified Rappaport scheme, and small noncleaved follicular center cell lymphoma in the Lukes-Collins classification. Immunotyping was consistent with the secretory blast stage of B cells. FINAL PATHOLOGICAL DIAGNOSIS

Malignant B cell lymphoma of high grade, small noncleaved cell type, with primary bilateral renal involvement. DISCUSSION

Doctor Risius. The history and radiographic findings certainly are consistent with renal involvement by lymphoma. It

is unusual that extrarenal sites of lymphomatous involvement have not been identified. Primary renal lymphoma is exceedingly rare. 3 Most believe that renal lymphoma is secondary to hematogenous dissemination in approximately 90 per cent of the cases, and that direct invasion of the kidney by lymphoma in the retroperitoneum occurs in 10 per cent. 5 In a large autopsy series renal involvement was present in about a third of the lymphoma patients. 5 Bilateral involvement was present in 75 per cent of these cases, the majority of which were present as multiple nodules. In another series 48 per cent of the patients had a solitary mass, 29 per cent had multiple masses, 19 per cent had complete parenchymal replacement and only 9 per cent had bilateral disease. 6 Before the advent of ultrasound and CT scanning, the IVP was the primary method to evaluate the urinary tract in lymphoma patients. Diffuse renal enlargement was the most common urographic presentation and was much more common than large discrete masses. It was believed that reticulum cell sarcoma and lymphosarcoma were much more likely to involve the urinary tract than was Hodgkin's disease. 7 CT scanning now is readily available and most patients with lymphoma undergo abdominal imaging during the evaluation. CT has the ability to image all lesions and has become useful in the detection of renal involvement in lymphoma patients. Chilcote and Borkowski recently reported the CT findings in patients with renal lymphoma. 8 In this series the incidence of renal involvement was similar between nonHodgkin's and Hodgkin's lymphomas. Bilateral renal involvement was present in 53 per cent of the patients. The most common appearance when first imaged was that of multiple nodular masses (86 per cent). Progression from discrete nodular masses to diffuse infiltration was documented in some patients who had serial scans. All of their patients had retroperitoneal adenopathy concurrent with renal involvement. Dr. K. V. Shepard. A few reported cases of primary renal lymphoma exist and most of them are poorly documented. 3 In 1956 Silber reported a case of primary lymphoma of the kidney that was resected radically. Two years later, after no interval treatment, a laparotomy was negative for recurrent tumor. The patient was followed for 5 years and no further evidence of lymphoma was discovered. Approximately 35 per cent of all extranodal primary lymphomas involve the gastrointestinal tract, particularly the stomach. Other common sites are the pharynx, skin, skeleton, dura and parotid glands. All other sites account for less than 1 per cent of the cases. As far as secondary involvement of the kidney by tumors, lung and breast carcinomas are the most common and lymphoma is third.

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The tumor in our case was a high grade nonHodgkin's lymphoma. The frequency of this tumor is relatively rare. The male-to-female ratio is slightly in favor of male patients, with 2 peak ages of presentation, childhood and around 60 years old. Approximately a third of the patients with high grade lymphoma present with stage I or II disease. The median survival in patients who are treated is approximately 1 to 2 years. Before the advent of aggressive chemotherapy, the 5-year survival rate was less than 10 per cent and long-term survival was rare. The treatment of stage I disease has included radiation therapy alone at some institutions. This therapy can be dangerous because of the aggressive nature of the tumor, and most investigators advocate chemotherapy alone or with radiation therapy. With stages II to IV disease, chemotherapy is the mainstay of treatment, plus or minus radiation therapy to the bulky disease. The most commonly used regimen for aggressive lymphoma is cyclophosphamide, doxorubicin, vincristine and prednisone. This regimen can be given with or without bleomycin and is the gold standard to which other treatments are to be compared. 9 With this regimen patients have a 50 per cent or greater chance of obtaining a remission. Approximately a third to a fourth of the patients who achieve remission live for 5 years or longer. SUMMARY

Doctor Pontes. Bilateral solid renal masses·presenting in a young patient in the absence of systemic manifestations or genetic abnormalities, such as Von Hippel-Lindau's disease, are difficult diagnostic challenges. Primary renal lymphomas are exceedingly rare. More commonly, renal involvements are caused by lymphoma in the retroperitoneal area. It is important

for a urologist to be aware of this diagnostic possibility, since unnecessary removal of a kidney and extensive surgery may be avoided, and because appropriate staging can be performed at exploration. REFERENCES 1. Bettendorf, U.: Doppelseitiger Wilms-Tumor bei einem Erwachsenen. Zentralbl. Alig. Path., 117: 394, 1973.

2. Bennington, J. L. and Beckwith, J.B.: Tumors of the kidney, renal pelvis, and ureter. In: Atlas of Tumor Pathology. Washington, D. C.: Armed Forces Institute of Pathology, 2nd series, fasc. 12, 1975. 3. Silber, S. J. and Chang, C. Y.: Primary lymphoma of kidney. J. Urol., 110: 282, 1973. 4. The Non-Hodgkin's Lymphoma Pathologic Classification Project: National Cancer Institute sponsored study of classification of non-Hodgkin's lymphomas: summary and description of a working formulation for clinical usage. Cancer, 49: 2112, 1982. 5. Richmond, J., Sherman, R. S., Diamond, H. D. and Craver, L. F.: Renal lesions associated with malignant lymphomas. Amer. J. Med., 32: 184, 1962. 6. Hartmann, D. S., Davis, C. J., Jr., Goldman, S. M., Friedman, A. C. and Fritzsche, P.: Renal lymphoma: radiologic-pathologic correlation of 21 cases. Radiology, 144: 759, 1982. 7. Lalli, A. F.: Lymphoma and the urinary tract. Radiology, 93: 1051, 1969. 8. Chilcote, W. A. and Borkowski, G. P.: Computed tomography in renal lymphoma. J. Comput. Assist. Tomogr., 7: 439, 1983. 9. Jones, S. E., Grozea, P. N., Metz, E. N., Haut, A., Stephens, R. L., Morrison, F. S., Butler, J. J., Byrne, G. E., Jr., Moon, T. E., Fisher, R., Haskins, C. L. and Coltman, C. A., Jr.: Superiority of adriamycin-containing combination chemotherapy in the treatment of diffuse lymphoma: a Southwest Oncology Group study. Cancer, 43: 417, 1979.