ANGIOMYOLIPOMA CARCINOMA PATRICK

IN SAME KIDNEY

B. KAVANEY,

IRA FIELDING,

AND RENAL CELL

M.D.

M.D.

From the Department of Surgery (Urology) Stanford University Medical Center, Stanford, and the Kaiser Foundation Hospital, Hayward, California

ABSTRACT - A case of renal cell carcinoma and angiomyolipoma occurring in the same kidney in a patient without stigmata of tuberous sclerosis is reported. This case was managed as malignant renal tumor, and the patient underwent radical nephrectomy. Urologic and angiographic literature regarding angiomyolipoma is reviewed, and we find the neovascularity of this benign tumor is only rarely distinguishable from malignant renal tumor.

The combination of angiomyolipoma and renal cell carcinoma has not been previously reported in the same patient or within the same kidney. There is a growing body of literature regarding angiomyolipoma, but there are no reports of malignancy associated with this relatively rare, benign tumor. l-4 Several reports in the radiologic-angiographic literature suggest a characteristic angiographic appearance for angiomyolipoma.5-7 We present herein a case of both hypemephroma and angiomyolipoma in the same kidney, a clinical condition that has not previously been reported. Angiographically, the angiomyolipoma was indistinguishable from the renal cell carcinoma, even in retrospect, and was managed as a malignant renal tumor. Case Report The patient is a forty-eight-year-old Filipino female seen in the emergency room of Kaiser Hospital with a history of sudden onset of left flank pain of several hours’ duration, followed by gross hematuria. She denied recent or past history of chills, fever, urinary infection, or calculous disease. On physical examination, the patient had colicky-type pain located in the left renal region.

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She was afebrile, and her blood pressure was 180/99 mm. Hg. A large tender mass could be palpated extending into the left flank. Past medical history included a vaginal hysterectomy in I959 for menometrorrhagia, and diuretic therapy for low-grade hypertension for one year. She had experienced intermittent left flank pain every three to four weeks for approximately two years, associated with dark or cloudy urine lasting for one to two days. The family history was noncontributory for renal disease. Laboratory studies included a hemoglobin of 10.3 Gm. per 100 ml., hematocrit 31, blood urea nitrogen 15 mg. and creatinine 0.7 mg. per 100 ml., and normal serum electrolytes. Urinalysis showed a full field of red blood cells. Intravenous urogram revealed a large mass in lower pole of left kidney with hydronephrosis of upper pole calyces (Fig. 1A and B). On cystoscopic examination the bladder was normal; retrograde urogram showed no evidence of obstruction to the ureter and confirmed the mass lesion in the lower pole. Retrograde aortic and selective renal arteriography demonstrated a 15-cm. dumbbell-shaped mass with neovascularity and extensive arteriovenous formation (Fig. 1C and D). Preoperative diagnosis was malignant renal tumor, presumably renal cell carcinoma.

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Liver scan and function studies, lung tomograms, and bone scan were negative for metastasis. Radical left nephrectomy with retroperitoneal lymph node dissection was performed through a

FIGURE 1. (AandB)Excretoryurogram (A) at joe minutes shows large mass extendingfiom lower pole, and (B) at fortyfive minutes conj+ms large mass lower pole of left kidney and hydronephrosis of upper portion of kidney. (C) Aortic arteriogram (early phase) demonstrating 15-cm., mass with marked1 dumbbell-shaped neovascularity and early arteriovenous fistula fonation. (D) Selective left renal arteriogram (early phase) showing tumor neovascularity with lucent center in lower mass.

transperitoneal incision. There were substantial blood vessels in the region of the lower pole of the left kidney. Pathologic examination of the gross specimen showed two separate masses within the kidney,

’ ~

1 ~ I

FIGURE 2. (A) Kidney sectioned, demonstrating two separate distinctive tumors, renal cell carcinoma in midzone of kidney, and angiomyolipoma in lower pole. Microscopic section (B) from renal cell carcinoma with clear cells forming primitive tubules; (C) j?om angiomyolipoma with mature fat cells, smooth muscle cells, and characteristic abnormal vessels.

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each 6 cm. in diameter and well encapsulated in gross appearance (Fig. 2A). The mass in the midzone of the kidney was a renal cell carcinoma while the mass in the lower portion (a mottled gray-pink, meaty color with a yellowish center) was an angiomyolipoma, completely separated from the renal cell tumor. Additional sections showed microinvasion of the capsule by the clear cell renal tumor, but no capsular perforation by the tumor and no tumor in perirenal fat or hilar nodes (Fig. 2B and C). The patient recovered and was discharged on the tenth postoperative day. She is alive and well one year postoperatively, without evidence of metastasis and is now normotensive. Repeat intravenous urogram continues to show a normal right kidney and renal function remains normal.

Comment Multiple reports in the literature regarding including several autopsy angiomyolipoma, series, indicate there has never been an association with renal cell carcinoma. 1,2,8,g Renal angiomyolipoma is a mesenchymal hamartoma, a benign tumor composed of tissue normally present in the kidney but abnormal in quantity, arrangement, and degree of maturation. It occurs rarely among the general population but is common in patients with tuberous sclerosis; 40 to 80 per cent of patients with tuberous sclerosis have renal angiomyolipoma which are usually bilateral, multiple, and represent incidental findings at autopsy. ‘3’ Seabury and Wolfe in 1967lO found 150 cases of angiomyolipoma which were not associated with tuberous sclerosis. Renal involvement was usually unilateral and solitary; females outnumbered males, 4 to 1. The usual age at detection was forty to sixty years. The gross appearance of the renal angiomyolipoma depends on the predominant cell type; it may appear as yellow, gray, or beefy red, depending on the proportion of fat to smooth muscle cells. The mass may present an angiomatous appearance. Hemorrhage and necrosis can be present and gross differentiation from renal cell carcinoma may be impossible. In the Price and Mostofi* histopathologic review of 30 symptomatic cases, the tumors (whether single or multiple) are composed of adipose tissue, smooth muscle, blood vessels, and a supporting stroma of connective tissue. There was no histologic difference between tumors in

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patients with or without tuberous sclerosis. They comment that these tumors are frequently misdiagnosed microscopically as sarcoma, usually liposarcoma. However, no documented case of metastasis from an angiomyolipoma has been reported. Angiographically, the angiomyolipoma in this patient was indistinguishable from the renal cell carcinoma, even in retrospect. Clark and Palubinskas,3 in a review of the literature in 1972, noted that 22 arteriographically studied renal hamartomas had been reported. Of the 19 reported hamartomas unassociated with tuberous sclerosis, the following angiographic features were noted. Only 1 angiomyolipoma was without evidence of pathologic vessels; the remaining I8 hamartomas had a striking pathologic vascularity, characterized by the usual tortuosity, great abundance, abnormal distribution, and branching pattern of the vessels. All were diagnosed as malignant renal tumors. In two articles, it was suggested that the angiographic appearance of an angiomyolipoma is characteristic. 5,6 Viamonte et al. 5 suggested that the increased hypervascularity and the pseudoaneurysms of the intrarenal arteries, resembling a “cluster of grapes,” may be characteristic. Khilnani, Aarons, and Beranbaum’ also showed the multisacculated pseudoaneurysms of the pathologic vessels; in addition, a “whorled onion peel” appearance of the venous phase was thought to be similar to that of myomatous uterine tissue. The specific angiographic appearance of this neoplasm, however, is the exception as summarized by Clark and Palubinskas.3,1’ Neovascularity indistinguishable from malignant renal tumors was observed in 68 per cent of the cases; the characteristic multisacculated pseudoaneurysm of the pathologic vessels, and the “whorled onion peel” venous phase, were present in only 16 per cent of their patients. Symptoms of renal angiomyolipoma-usually results from hemorrhage into or around the tumors. This may cause flank pain, shock, an enlarging mass, hematuria, and gastrointestinal symptoms varying from mild to acute abdominal pain. Hemoperitoneum occurs rarely. When several series were combined, it was found that 87 per cent of the patients had pain (usually severe), 47 per cent had palpable masses, and 40 per cent had hematuria.‘,2,8,g When a unilateral, solid tumor with neovascularity occurs in patients who have no signs of tuberous sclerosis, it will usually be impossible to differentiate from renal cell carcinoma, and

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radical nephrectomy course to follow.

will probably

be the safest

Stanford University Medical Center Stanford, California 94305 (DR. KAVANEY) References MCCULLOUGH,D. I., SCOTT, R., and SEYBALD, H. M.: Renal angiomyolipoma (hamartoma): review of the literature and report of 7 cases, J. Urol. 105: 32 (1971). ALLEN, T., and RISH, W.: Renal angiomyohpoma, ibid. 94: 203 (1965). CLARK, R. E., and PALUBINSKAS,A. J.: The angiographic spectrum of renal hamartoma, Am. J. Roentgenol. 114: 715 (1972). FARROW, G. M., HARRISON, E. G., JR., UTZ, D. C., and JONES, D. R.: Renal angiomyolipoma. A clinicopathologic study of 32 cases, Cancer 22: 564 (1968).

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5. VIAMONTE, J,, JR., RAVEL, R., POLITANO, V., and BRIDGE, B.: Angiographic findings in patients with tuberous sclerosis, Am. J. Roentgenol. 98: 723 (1966). 6. KHILNANI, M. T., AARONS, R. M., and BERANBAUM, E. R.: Angiographic features of hamartoma of the kidney: case report, Radiology 90: 999 (1968). 7. SILBIGER, M. L., and PETERSON,C. C.: Renal angiomyolipoma: its distinctive angiographic characteristics, J. Ural. 106: 363 (1971). 8. PRICE, E., and MOSTOFI, F. K.: Symptomatic angiomyolipoma of the kidney, Cancer 18: 761 (1965). 9. HAJDU, S., and FOOTE, F.: Angiomyohpoma of the kidney: report of 27 cases and review of the literature, J. Urol. 102: 396 (1969). 10. SEABURY,J, C., andWOLFE, W. G.: Angiomyohpoma of the kidney: a benign tumor demonstrating neovascularity by arteriography. Report of 2 cases, ibid. 98: 562 (1967). 11. WATSON, R. C., FLEMING, R. J., and EVANS, J. A.: Arteriography in the diagnosis of renal carcinoma, Radiology 91: 888 (1968).

UROLOGY / NOVEMBER 1975 I VOLUME VI, NUMBER 5

Angiomyolipoma and renal cell carcinoma in same kidney.

A case of renal cell carcinoma and angiomyolipoma occurring in the same kidney in a patient without stigmata of tuberous sclerosis is reported. This c...
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