THE JOURNAL OF UROLOGY Copyright © 1976 by The Williams & Wilkins Co.

Vol. 116, December

Printed in U.S.A.

ANGIOMYOLIPOMA: DIAGNOSIS AND TREATMENT DONALD E. WALKER, JOHN M. BARRY*

AND

CLARENCE V. HODGES

From the Division of Urology, Department of Surgery, University of Oregon Health Sciences Center, Portland, Oregon

ABSTRACT

Four cases of renal angiomyolipoma are presented, 3 of which were diagnosed in non-tuberous sclerosis patients. In 1 case diagnosis was made preoperatively and in another case it was made intraoperatively, allowing for preservation of functioning renal parenchyma. The second successful kidney transplant in a patient with tuberous sclerosis and renal failure is reported. One cannot always differentiate renal angiomyolipomas from adenocarcinoma. However, if the classical angiographic_firidirigs o(.~acculatedpseudo~anemysm~ surmlie_d by _the interlobular and interlooar arteries a_re present non~operative observalion grJi_rnited surgery with preservation 'ofrenar fiss'U:e is pgssible. Also, knowledge of the gross pathologic appearance arid tliesyn:drome of tuberous sclerosis will allow one to make a preoperative or intraoperative diagnosis with confidence. no caliceal distortion. An arteriogram demonstrated a hypovascular tumor fed by a renal capsular branch as well as a small branch from the internal iliac artery. No metastases were documented by the liver and bone scans, liver function tests, chest x-ray or metastatic bone survey. The sedimentation rate was elevated to 44 mm. per hour. Right radical nephrectomy was performed and tissue diagnosis was angiomyolipoma (fig. 1). The hypertension has persisted. 3

Angiomyolipoma, a neoplasm rarely occurring in the general population but commonly in patients with tuberous sclerosis, was first described by Bourneville in 1880. 1 • 2 Although it is a benign tumor it often clinically mimics a malignant lesion and is so treated. When angiomyolipoma presents bilaterally in association with tuberous sclerosis its diagnosis is less difficult than when it is unilateral and in a patient who does not have tuberous sclerosis. Herein are described 4 cases of renal angiomyolipoma, illustrating the clinical, radiographic and pathologic features that allow preoperative or intraoperative diagnosis and preservation of functioning renal tissue. CASE REPORTS

Case I. L. T., UOMS 45-10-16, a 60-year-old welder with tuberous sclerosis, had renal failure and hematuria owing to tumor in a solitary kidney. He had undergone right nephrectomy in 1953 for what was thought to have been a renal adenocarcinoma but review of the original microscopic sections and the sections from an open renal biopsy done prior to transfer revealed angiomyolipomas. The patient's father, 2 brothers, 1 sister and 2 children also had tuberous sclerosis. The patient displayed many features of tuberous sclerosis, including adenoma sebaceum, phakomas, subungual fibromas and shagreen patches, A retrograde pyelogram revealed distortion of the caliceal system with numerous filling defects. The arteriogram revealed several areas of neovascularity through the renal capsule. There were radiolucent areas in the lower pole. An emergency left nephrectomy was performed for massive bleeding on January 19, 1971. Renal failure was managed with hemodialysis until June when the patient received a kidney transplant from a brother who did not have tuberous sclerosis. Presently, he is doing well with normal renal function 4 ½ years after transplantation. Case 2. M. S., UOMS 1-81-60, a 57-year-old white woman, was admitted to the hospital in August 1973 for evaluation of hypertension of recent onset. Physical examination revealed a non-tender 6 by 7 cm. right lower quadrant abdominal mass that moved with respiration. There were no stigmas of tuberous sclerosis. A plain radiogram of the abdomen revealed speckled calcifications and a radiolucency in the region of the mass. An excretory urogram (IVP) displayed a normal renal contour and Accepted for publication May 21, 1976. Read at annual meeting of Western Section, American Urological Association, Coronado. California, February 22-26, 1976. * Requests for reprints: Division of Urology, University of Oregon Health Sciences Center, Portland, Oregon 97201.

FIG. 1. Case 2. Gross specimen. Pseudo-encapsulated angiomyolipoma attached to lower pole of right kidney. Extrarenal tumor attached to kidney occurs in a fourth of angiomyolipomas occurring in patients without tuberous sclerosis.

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Fm. 2. Case 3. A, ultrasound examination. Compact renal pelvis and calices with solid lower pole tumor displaying numerous internal echoes on low gain. This is believed to be owing to variable densities of fat, blood vessels and smooth muscle in tumors. B, selective renal arteriogram of right kidney. Sacculated aneurysmal dilatation of interlobar and interlobular arteries with tortuous vessels. No early venous filling.

Case 3. V. M., UOMS 44-89-00, a 31-year-old white woman, had persistent urinary retention following a hemorrhoid operation. Physical examination was normal. A right lower pole mass was found on IVP. Ultrasonography indicated a solid tumor with numerous internal echos (fig. 2, A). An arteriogram revealed an intraparenchymal, lower pole lesion with aneurysms of the interlobar and interlobular arteries characteristic of an angiomyolipoma (fig. 2, B). An uncomplicated lower pole nephrectomy was done with frozen section microscopic confirmation of the diagnosis and adequacy of the surgical margins. There was no evidence of recurrence when the woman was seen 2 ½ years postoperatively. Case 4. 0. C., KH 94-86-80, a 35-year-old woman, was found to have an abdominal mass on the right side during a routine 8-week prenatal examination. She did not have tuberous sclerosis. A limited IVP revealed a mass displacing the double collecting system of the right kidney anteriorly. Ultrasound documented a solid tumor immediately adjacent to the kidney. A barium enema showed that the colon was anterior to the mass. An exploratory operation was done through a right subcostal abdominal incision and a large retroperitoneal tumor was removed with a wedge of renal cortex because the mass was attached to the lower pole of the kidney by a small pedicle. Clinical diagnosis was angiomyolipoma because of its gross appearance and location. This diagnosis was confirmed by frozen section microscopic examination of the excised specimen. The woman delivered a normal baby at term. She was free of recurrence 1 year postoperatively. Fm. 3. Case. 4. Histology of angiomyolipoma, medium power. Three components of tumor displayed: adult fat, tortuous arteries with eccentric lumina and smooth muscle cells streaming away from vessles in pinwheel pattern.

DISCUSSION

At least 175 cases of angiomyolipoma have been reported in patients ranging in age from 12 to 71 years. 2 • • Half of the cases

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haveoccmred in patients with no stiglllas of tuberous scleros,is . '· 4 In this clinical setting the tumors are usually single, unilateral and, for. unknown reasons, on the right side 80 per cent of the time:•. 5 Female outnuIT1ber male patienfs 4 to L' Sixtyto 80- peccent of p_atientswithtuberous sclerosis have renal angiomyolipomas. These are usuaily oilalerar; multiple and are diagnosed at an average age· ofsf ~vears," 5;· 6 17 years younger than their non-tuberous sclerosis counterparts. Male equal female patients. 6 • 7 Although symptoms usually depend upon the number of tumors and their proximity to the collecting system many are asymptomatic and are discovered incidentally, Pain and flank mass are the most frequent complaints with hematuria and symptoms of urinary tract infection occurring much less commonly, 2 At least 8 cases of retroperitoneal hemorrhage have been reported, 8 Five cases of bilateral involvement and resultant renal failure have been reported 1 and only 1 previous patient had been transplanted successfully. 6 • 9 There have been 3 cases of renal vein extension and 1 case of nodal involvement 7 but no cases of distant metastasis, Without surgical intervention the natural course for these tumors is local growth and extension. 4 Characteristically~ these lesions are within the parenchyma but in 25 per cent of the cases the tumor is extrarenal, stretching the capsule over its surface and occ.asionally extending into the perirenal fat. 4 On cut surface these tumors vary from yellow to gray, according to the proportion of fat to spindle cells. Histologically, angiomyolipomas are composed of fat, smooth muscle and blood vessels (fig. 3). The fat is of the adult type with lipoblasts and occasional multinucleated giant cells, The vascular portion consists of thicked-walled, small and medium-sized arteries that are tortuous, in groups and form microaneurysms. They usually have an eccentric lumen, an indefinite media and adventitia, and often no internal elastic laminae. Elongated spindle-shaped smooth muscle cells stream from the vessel surfaces in a whorling pattern. Interlacing bundles of smooth muscle also are found with moderate anaplasia but few mitotic figures. 4 An IVP may reveal mass lesions involving the kidney, which, if bilateral and multiple, can resemble polycystic kidneys. 1 • 5 Calcification occurs in 6 per cent of the cases and radiolucence, the result of increased fat content, occurs in only 9 per cent. 5 One-fourth are extracapsula:r and the only abnormality is renal displacement ..< Viamonte and associates have reported arteriographic findings peculiar to renal angiomyolipomas. 10 These include the presence of many small but regular outpouchings resembling berry_aneurysms ar.ising from the inter1obar arid interlobular artei:ies and resembling a duster of grapes, These lesions are in sharp contrast to the irregular size and contour manifested by the focal ectasia prominent in vessels of renal adenocarcinoma. The. capillary phase may display a sunburstor whorled 1 • 6 appeara11ce similar to that seen in myomatous uterine fissu~ b_ecause onhe circumferential peripheral vessels. Pooling and puddling may or may not be seen. 1 • 4 , 5 Arteriovenous shunting has not been reported in angiomyolipomas but it is not unco1nmon in. renal adenocarcinoma. The response of vessels to

epinephrine has been variable.'· 6 • 11 Clark and Palubinskas reviewed 26 cases angiographically and noted the following descriptions: 1) neovascularity indistinguishable from hypernephromas-68 per cent; 2) pseudo-aneurysms, radiolucency, onion peel effect-16 per cent; 3) delicate sacculated pseudoaneurysms supplied by interlobular and interlobar arteries-12 per cent and 4) avascular radiolucency-4 per cent. 12 Few descriptions of sonographic examinations of angiomyolipoma have been reported but it is the opinion of our ultrasound department that a solid lesion with numerous internal echoes from the changes in tissue density between fat, muscle and packed vessels may well be found to be characteristic of angiomyolipomas. SUMMARY

It is apparent that several different patterns of angiomyolipoma occur, some of which have characteristic findings on IVP, arteriography, gross pathology and possibly ultrasonography, that allow for preoperative or intraoperative diagnoses and conservative therapy with preservation of renal parenchyma, Our approach to treatment for angiomyolipomas is as follows: 1) bilateral disease with tuberous sclerosis-treat complications only, 2) unilateral disease with tuberous sclerosis-open renal biopsy, 3) unilateral disease with angiographic criteria fulfilled-partial nephrectomy if possible and 4) unilateral disease indistinguishable from hypernephroma-radical nephrectomy. REFERENCES

1. Chonko, A. M., Weiss, 8. M., Stein, J. H. and Ferris, T. F.: Renal involvement in tuberous sclerosis. Amer. J. Med., 56: 124, 1974. 2. Ma, M. K. G. and Chan, K. W.: Renal angiomyolipoma-report of 5 cases. Brit. J. Urol., 46: 481, 1974. 3. McCullough, D. L., Scott, R., Jr. and Seybold, H. M.: Renal angiomyolipoma (hamartoma): review of the literature and report of 7 cases. J. Urol., 105: 32, 1971. 4. 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. 5. Becker, J. A., Kinkhabwala, M., Pollack, H. and Bosniak, M.: Angiomyolipoma (hamartoma) of the kidney. An angiographic review. Acta Radio!., Diag., 14: 561, 1973. 6. Bissada, N. K., White, H.J., Sun, C. N., Smith, P. L., Barbour, G. L. and Redman, J." F-:-: Tuberous sclerosis complex and renal angiomyolipoma: collective review. Urology, 6: 105, 1975. 7. Scott, M. B., Halpern, M. and Cosgrove, M. D.: Renal angiomyolipoma: two varieties. Urology, 6: 768, 1975. 8. Vasko, J. S., Brockman, S. K. and Bomar, R. L.: Renal angiomyolipoma: a rare case of spontaneous massive retroperitoneal hemorrhage. Ann. Surg., 161: 577, 1965. 9. Jochimsen, P. R., Braunstein, P. M. and Najarian, J. S.: Renal allotransplantation for bilateral renal tumors. J.A.M.A., 210: 1721, 1969. 10. Viamonte, M., Jr., Ravel, R., Politano, V. and Bridges, B.: Angiographic findings in a patient with tuberous sclerosis. Amer. J. Roentgen., 98: 723, 1966. 11. Schield, P. N.: Epinephrine effect in vascular pattern of a renal lipoma. Radiology, 95: 657, 1970. 12. Clark, R. E. and Palubinskas, A. J.: The angiographic spectrum of renal hamartoma. Amer. J. Roentgen., 114: 715, 1972.

Angiomyolipoma: diagnosis and treatment.

Four cases of renal angiomyolipoma are presented, 3 of which were diagnosed in non-tuberous sclerosis patients. In 1 case diagnosis was made preoperat...
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