Renal Hemangioma

Renal Hemangioma

Vol. 105, Jan. Printed in U.S.A. THE JOURNAL OF UROLOGY Copyright© 1971 by The Williams & Wilkins Co. RENAL HEMANGI01.VIA NORMAN E. PETERSON AND ...

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Vol. 105, Jan. Printed in U.S.A.

THE JOURNAL OF UROLOGY

Copyright© 1971 by The Williams & Wilkins Co.

RENAL HEMANGI01.VIA NORMAN E. PETERSON

AND

HOWARD T. THOMPSON

From the Division of Urology, University of Rochester and Strong l'vlemorial Hospital ancl the Department of Urology, Rochester General Hospital, Rochester, New Yark

Benign renal tumors are usually silent and asymptomatic, producing symptoms only when increasing size imposes pressure on adjacent tissues or organs. Exceptions to this general rule are villose papillomas and hemangiomas which may bleed early and Our recent experience with 2 patients having unusual signs and symptoms associated with the ultimate pathologic demonstration of renal hemang1oma prompted a review of the relevant literature. CASE REPORTS

Case 1. G. G. RGH a 64-yearold white housewife, was seen a urologist 5 years prior to admission to the hospital for evaluation of lower urinary tract symptoms. A 11011tender, asymptomatic right upper quadrant mass was palpated. Urologic evaluation was negative. The woman remained well until 3 weeks prior to the present hospitalization when she had fever, c:hills, malaise, anorexia and listlessness associated with gross total hematuria with clots and vague right lower quadrant aching pain. Immediate cystoscopy revealed a blood clot protruding from the right ureteral orifice. Excretory urography (IVP) demonstrated nonfunction of the right kidney. Physical examination vrns unremarkable with the exception of moderate dehydration and an easily palpable, non-movable, indurated mass in the area of the right kidney. Laboratory data were unremarkable except for leukocytosis of 12,000 and total serum protein of 4.7 gm. per cent with 2.5 gm. per cent albumin. urine culture was sterile. A renogram was normal on the left side with satisfactory concentration but there was obstruction to drainage on the right side. Chest x-ray and metastatic survey were unremarkable. Repeat cystoscopy demonstrated a blood clot protruding from the right ureteral orifice. Retrograde right ureteral catheterization obtained urine for cytology which was positive for malignant cells.

Accepted for publication February 5, 1970. 27

Contrast studies demonstrated an apparent space-occupying lesion involving the renal pelvis with suspected involvement of the adj accnt renal parenchyma (fig. 1, A). Nephrotomograms were non-contributory. Selective right renal angiography demonstrated a normal vascular pattern (fig. 1, B). The patient became septic with daily temperature elevations to 104 degrees without localizing signs. She did not respond to large doses of antibiotics. Repeated blood cultures were negative. The urine was sterilized \Yith appropriate drug therapy. Hydration was instituted and the serum protein levels and mild anemia were corrected with whole blood replacement. At surgical exploration the right kidney was surrounded by numerous superficial dilated veins and perirenal fibrosis. N ephroureterectomy was performed. The specimen demonstrated no evidence of malignancy despite the prior positive cytologic report and the suspicious radiologic and surgical appearance. There was a wellorganized perirenal hematoma and diffuse pyelitis and pyelonephritis. Abutting on the superior pole calyx was a large nest of dilated vessel8 eroding the calyceal mucosa, with infarction in the adjacent renal papilla compatible with renal papillary necrosis (fig. 2). Postoperatively the patient's temperature returned promptly to normal and convalescence was unremarkable. She was discharged 8 postoperatively and was well and asymptomatic at followup examination 1 year later. Comment. This case represents several features of how renal angioma may present but is atypical in the associated sepsis and inflammatory components. In retrospect, the old perirenal hematoma of unknown etiology accounted for the induration and fixation seen at physical examination. The acute inflammatory process and evidence of obstruction on renogram and pyelography were due to obstruction of the ureteropelvic junction by thrombus or sloughed papillary tissue. Case 2. W. :\I. K., S:\IH 64-72-81, a46-year-old

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Fm. 1. Case 1. A, right retrograde pyelogram demonstrates renal pelvic and calyceal distortion and space-occupying lesion. B, right selective renal arteriogram demonstrates normal vascular pattern without blush, pooling or distortion.

Fm. 2. Case 1. A, gross cut specimen demonstrates locus of dilated vessels adjacent to upper calyceal system and adjacent renal papillary infarction. B, enlarged section through vascular tumor. Negress, gravida 12, Para 12, was well until 2 days prior to hospitalization when gross, total, painless hematuria with clots developed. She had had medically controlled hypertension for 4 years. One year prior to hospitalization an IVP revealed pyelocaliectasis on the right with a suggestion of intrapelvic calculus. A renogram was normal on 2 occasions. Physical examination revealed an obese woman without localizing signs or symptoms except for a blood pressure of 160/110 and gross hematuria. Laboratory data

were normal. Urine cultures were sterile. An IVP again demonstrated a filling defect in the right renal pelvis (fig. 3, A). Renogram reflected diminished drainage of the right kidney. Cystoscopy revealed suspicious lesions of the urinary trigone and bladder mucosa about the right ureteral orifice which were biopsied. A urine specimen was obtained for cytology. Retrograde studies confirmed the pyelographic findings (fig. 3, B). Despite negative cytology and biopsy report of squamous metaplasia, the history and

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FIG. 3. Case 2. A, IVP reveals intrapelvic filling defect of right kidney. B, retrograde pyelogram confirms presence of intrapelvic filling defect. symptoms prompted the decision to perform nephroureterectomy. The specimen demonstrated a normal renal pelvis but underlying the upper pole calyceal system there was a complex of enlarged and dilated vessels eroding the mucosa which was confirmed microscopically to be an angioma (fig. 4). Convalescence was uneventful and the patient remains well and asymptomatic 18 months postoperatively. Comment. Although this case depicts many typical features of renal angioma it further demonstrates how the vascular complex, dilated with circulating blood in vivo, could efface the renal collecting system and suggest a spaceoccupying intrapelvic lesion which might easily be overlooked in the collapsed cadaveric state. DISCUSSIO:\T

Hemangiomas are common in skin and mucous membranes and their importance is usually proportional to their size. Other tissues most commonly involved include liver, kidney, bone, intestine and muscle. According to Virchow, the kidneys are second only to the liver in incidence of these vascular tumors in internal organs. Hemangiomas are congenital neoplastic

lesions involving blood and lymph vessels that arise from endothelium and grow by budding, pushing into adjacent tissues but not communicating with surrounding vessels. Therefore these tumors are not truly invasive. Grossly they are soft, spongy, dark red, poorly encapsulated tumors and microscopically they are composed of various-sized, epithelium-lined spaces (small, capillary; large, cavernous) filled with red blood cells and thrombi. Connective tissue septae may condense to form a capsule. Through 1967 more than 150 cases of renal hemangioma have been reported. Cases are nearly equally distributed between sexes and the right and left kidneys are essentially equally involved. Multiple tumors may be present in up to 12 per cent of cases, causing criticism of papillectomy and heminephrectomy advocated by some clinicians. 1 - 3 Less than 1 per cent of the 1 Weyrauch, H. M. and Berger, M. M.: Hemangioma of kidney: report of case simulating pyeloureteri tis cystica; review of literature. Stanford Med. Bull., 9: 43, 1951. 2 Dorman, H. N. and Fowler, H. A.: Hemangioma of the kidney: report of an additional case. J. Urol., 55: 348, 1946. 3 Hamm, F. C. : Angioma of the kidney. J. U rol., 55: 143, 1946.

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Frn. 4. Case 2. Gross cut specimen demonstrates upper pole submucosal vascular tumor. tumors are bilateral. Lesions may range in size from pinhead and microscopic to measurements of 12 by 5 by 5 cm. The majority of tumors measure 1 to 2 cm. 4 Anatomically more than 90 per cent of reported lesions have resided in submucosal, papillary or medullary positions2 in which erosion, thrombosis or infarction can lead to gross hematuria. Parenchymal and subcapsular lesions have been described and are responsible for the larger tumors reported and account for the 2 cases of perirenal hematoma. 2 In addition, Raff and Podolsky reviewed 68 cases of renal hemangioma prior to 1947 and found 11 cases of renal pelvic lesions, to which they added another. 5 Another case was added by Anderson and associates in 1953. 6 Patients with clinically apparent renal angiomas ranged between 4 days old and 72 years 4 Lazarus, J. A. and Marks, M. S.: Renal hemangioma. Uro!. & Cutan. Rev., 51: 500, 1947. 5 Raff, L. G. and Podolsky, W. G.: Hemangioma of the kidney. New York J. Med., 51: 1536, 1951. 6 Anderson, J.B., Lee, J. J., Hancock, R. A. and Black, S. R.: Hemangioma of the kidney pelvis. J. Urol., 70: 869, 1953.

old with a peak between 30 and 407 • 8 and with 70 per cent of reported cases occurring in patients between 20 and 50 years old. 9 Rappoport has commented that 85 per cent of all patients with renal angioma will experience hematuria before age 40, whereas renal carcinoma will usually involve patients older than 40. 10 The recognition of renal angioma as a cause of otherwise unexplained hematuria prompted speculation that its frequency might be greater than expected, leading to several series of autopsy reviews. In 6 separate autopsy reviews involving 132,000 cases, only 4 renal angiomas were found.11- 14 However, critics of these studies claim such lesions are often extremely small and collapsed in the cadaveric state and may easily be overlooked. 15 • 16 Edward and associates reviewed 13 cases of renal angioma and found 4 cases associated with hematuria while 9 cases were clinically silent. 8 Of these latter 9 cases, 7 were found at autopsy and 2 were present in kidneys nephrectomized for other reasons. Dukes did a nephrectomy on 9 patients with the diagnosis of unilateral essential hematuria who were otherwise clinically and radiographically normal.17 Of these 9 kidneys removed, 3 contained renal papillary angiomas. Symptoms typically associated with renal angioma include hematuria, pain and the passage 7

McCrea, L. E.: Hemangioma of kidney. Urol.

& Cutan. Rev., 55: 670, 1951.

8 Edward, H. G., DeWeerd, J. H. and Woolner, L.B.: Renal hemangiomas. Mayo Clin. Proc., 37: 545, 1962. 9 Judd, E. S. and Simon, H. E.: Angioma of kidney. Surg., Gynec. & Obst., 46: 711, 1928. 10 Rappaport, A. E.: Hematuria due to papillary hemangioma of renal pelvis. Arch. Path., 40: 84, 1945. ll Kidd, F.: Cavernous angioma of the kidney. Proc. Roy. Soc. Med., 17: 56, 1924. 12 Bell, E. T.: A classification of renal tumors with observations on the frequency of the various types. J. Urol., 39: 238, 1938. 13 Riley, A. and Swann, W. J.: Angioma of the kidney. Urol. & Cutan. Rev., 45: 377, 1941. 14 Rottino, A. and Mohan, H.: Renal hemangioma: an obscure cause of hematuria. J. Urol., 51: 601, 1944. 15 Mackey, W. A.: Hemangioma of the kidney. Brit. J. Surg., 18: 308, 1931. 16 Webb-Johnson, A. and Turner-Warwick, W.: Essential hematuria: are lesions of a renal papilla a frequent cause? Brit. J. Urol., 13: 127, 1941. 17 Dukes, C. E.: Lettsomian lectures on "clinical pathology": past, present and future. Trans. Med. Soc. London, 65: 375, 1949.

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of clots. Hematuria may be constant but more frequently is intermittent; the longest reported duration of hematuria was 36 years. Pain may be due either to the passage of blood clots down the ureter or to the pressure of interstitial bleeding. Evaluation of the patient with suspected renal angioma becomes the evaluation of the patient with hematuria. Correct preoperative diagnosis relies heavily on x-ray contrast data for localization of the bleeding and delineation of the nature of the lesion. Although such preoperative diagnosis is rare it has been achieved. 18 • 19 Attention has been drawn to a mottled radiographic appearance involving that portion of the renal collecting system containing an angioma. Anderson and Rasmussen demonstrated such a lesion by selective arteriography leading to successful preoperative diagnosis, 19 The diagnosis should be considered in any patient less than 40 years old with unilateral hematuria and in whom tumor and calculus can be excluded. Treatment includes observation, nephrectomy, heminephrectomy, papillectomy and radiation. Observation is not contraindicated in the healthy patient with mild to moderate hematuria who is otherwise clinically and radiographically well 18 Butt, A. J. and Perry, J. Q.: Hemangioma of the kidney. J. U rol., 65: 15, 1951. 19 Anderson, J. B. and Rasmussen, T.: Renal hemangioma diagnosed preoperatively by selective renal angiography. Acta Radio!., 2: 201, 1964.

or when marginal renal function or the absence of an opposite kidney dictates such a course. Heminephrectomy and papillectomy have been successfully performed and remain available for specific indications. The percentages of multiple and bilateral tumors are not strong deterrents to this management. Radiation has been used successfully in a patient in whom hematuria developed from the remaining kidney after nephrectomy for bleeding renal angioma. This mode of therapy remains available for the appropriate case. Finally, nephrectomy remains the treatment of choice due to the difficulty in differentiating hemangiomas from more aggressive tumors as well as the need to control profuse and threatening hemorrhage often associated with these otherwise benign lesions. SUMMARY

Renal hemangioma occurs with sufficient frequency to warrant its consideration in any case of unilateral hematuria, particularly when tumor and calculus can be ruled out effectively. The tendency of this lesion to occur in patients less than 40 years old may assist diagnosis. Although symptoms may be lacking or minimal, hernaturia, which may be profuse and emergent, and other symptoms attendant to the hematuria are typical. Two representative cases are presented.