RENAL ANGIOMYOLIPOMA POLYCYSTIC JEFFREY MELVIN JOHN
DISEASE
H. NEWHOUSE,
P. A. TUTSCHKA,
IN ADULT-TYPE
M.D.
M.D.
E. CLOUSE,
M.D.
A. LIBERTINO,
M.D.
From the Departments of Radiology and Urology, Harvard Medical School, New England Deaconess Hospital, and Massachusetts General Hospital, Boston, Massachusetts
- Two adults with renal angiomyolipomas and polycystic disease are presented with emphasis on the angiographic manifestations of the coexisting lesions. One patient had multiple episodes of gross hematuria and a perinephric abscess which resulted in bilateral nephrectomies; the second presented with hypertension and underwent unilateral nephrectomy to treat a hypervascular tumor. The clinical and radiographic diagnoses of the combined lesions are discussed and suggestions for management offered. _ ABSTRACT
tuberant abdomen and palpable kidneys. An excretory urogram was interpreted as showing polycystic kidneys. Seizures beginning at age ten were easily controlled with diphenylhydantoin (Dilantin). At age sixteen, gross hematuria developed after a fall. Hematuria reappeared spontaneously two years later; both episodes were successfully managed conservatively. Several months prior to admission she experienced gross hematuria and flank pain following strenuous activity. She was admitted to another hospital where cystoscopy showed blood coming from the right ureteral orifice; 14 units of whole blood were administered. The patient was transferred to the New England Deaconess Hospital where physical examination revealed a right upper quadrant mass and right costovertebral angle tenderness. Stigmata of tuberous sclerosis were noted in the skin, retinas, fingernails, and roentgenograms of the bones. Blood pressure was 130/80 mm. Hg, hemoglobin 8.5 mg. per 100 ml., hematocrit 35, and blood urea nitrogen 24 mg. and creatinine 2.6 mg. per 100 ml. Plain film of the abdomen showed both kidneys to be 21 cm. in length with no visible lucencies,
Renal angiomyolipomas are benign tumors which may occur as solitary lesions or as the multiple renal masses associated with the tuberous sclerosis complex. l-5 Their association with polycystic renal disease appears to be rare. A review of the literature reveals only 7 probable cases; 2 in babies with histologic evidence of infantile-type polycystic disease, 6,7 3 in patients with proved adult-type polycystic disease,8,g and 2 in adults whose kidneys revealed multiple superficial cysts on gross examination but in whom histologic examination was confined to the tumor.2’4 Angiomyolipomas are benign and slow growing. Nevertheless, they have occasioned nephrectomies both by mimicking malignant tumors” and by causing spontaneous hemorrhage.’ Both patients described herein had renal angiomyolipomas associated with polycystic disease and underwent potentially avoidable nephrectomies. Case Reports Case 1
A nineteen-year-old white female was admitted with a chief complaint of uncontrollable hematuria. At age two she was noted to have a pro-
UROLOGY
/ SEPTEMBER
1975
/ VOLUME
VI, NUMBER
3
G89
and urogram showed bilateral elongation, distortion, and compression of the pyelocalyceal systems. Selective right renal arteriography revealed a hypervascular mass in the midportion of the right kidney (Fig. 1A). Several interlobar arteries supplying the tumor lacked normal tapering, were enlarged, and contained small saccular aneurysms. Venous opacification was prolonged; the nephrogram formed an “onion peel” pattern (Fig. 1B). Multiple small lucent masses varied in size from several millimeters to 3 cm. Late films showed opacification of a sac-like structure in the middle pole of the kidney (Fig. 1C). Extravasation of contrast was not sufficient to localize a bleeding point. Arteriography, after 10 pg. of intra-arterial epinephrine, revealed moderate constriction of the tumor vessels and marked constriction of the remainder of the vasculature. Selective left renal arteriography showed multiple avascular masses; abnormal vessels with small saccular aneurysms were also present. The bleeding continued, and the patient received massive transfusions of leukocyte-free frozen blood. During right nephrectomy, frozen sections of the mass were interpreted as renal carcinoma; permanent sections were typical of angiomyolipoma. The kidney contained numerous cysts in the cortex and medulla. A large clot in a dilated middle pole calyx corresponded to the contrast collection seen at arteriography (Fig. 1D). Recovery was uneventful. Shortly before the last admission, spontaneous gross hematuria reappeared. Except for the surgical scar and hypertension (blood pressure 180/110 mm. Hg), her physical condition was unchanged. Blood urea nitrogen had risen to 35 mg. and creatinine to 4.2 mg. per 100 ml. Intermittent gross hematuria continued for two weeks. Angiography was considered on several occasions to locate the exact bleeding site and to control hemorrhage by embolization with autologous clot; on each occasion, spontaneous remission of hemorrhage forestalled the procedure. The patient then became septic with temperature rising as high as 104” F. Urine culture grew enterococcus. Antibiotic therapy was unsuccessful, and she underwent a left nephrectomy with prompt defervescence. Pathologic examination of the kidney revealed multiple cortical and medullary cysts and angiomyolipomas with evidence of an acute perinephric abscess. Case 2 A forty-eight-year-old white ferred to Walter Reed General
390
female was reHospital for re-
section of a renal tumor. Elevated blood pressure ranging from 1401100 to 170/110 mm. Hg had been detected several months prior to admission. The patient complained of intermittent headaches and dizziness but denied all other symptoms associated with hypertension, renal disease, or tuberous sclerosis. There was no family history of these diseases. Physical examination revealed a well-developed, well-nourished white female with pulse 88, respiration 22, and blood pressure 186/120 mm. Hg. Positive physical findings included a systolic murmur at the apex with a prominent apical impulse. The liver was palpable 3 fingerbreadths below the costal margin. There were no stigmata of tuberous sclerosis in the skin, retinas, or fingernails. The kidneys were not palpable. Complete blood count, serum electrolytes, blood urea nitrogen, creatinine, uric acid cholesterol, total albumin, bilirubin, alkaline phosphatase, lactic acid dehydrogenase, glutamic oxalic transaminase, and urine vanylmandelic acid were normal. Urinalysis revealed microscopic hematuria. Electrocardiography showed signs of left ventricular hypertrophy. No radiographs of the skull or long bones were obtained. The chest film was normal. Excretory urography revealed markedly enlarged kidneys. The nephrogram was faint and mottled with a “Swiss cheese” appearance; the pyelocalyceal systems were splayed. No abnormal fat densities were visible on plain film of the abdomen. Bilateral selective arteriography showed enlarged kidneys with stretched intrarenal vessels. There was a hypervascular mass involving the left upper pole with tortuous vessels, multiple arterial aneurysms 2 to 3 mm. in diameter, and a heterogeneous capillary phase (Fig. 2). At surgery, the liver was found to be covered with multiple superficial cysts. The right kidney contained multiple cysts; the wall of the largest of these was resected. There was a mass within the upper pole of the left kidney which was well encapsulated and did not involve the adrenal gland. The left kidney, adrenal gland, perirenal fat, and Gerota’s fascia were excised en bloc. Postoperative recovery was uneventful. Pathologic examination of the left kidney revealed multiple cortical and medullary cysts of various diameters. A large mass replaced the upper pole of the kidney and multiple smaller solid nodules were scattered throughout the renal parenchyma. These masses, tissue found in the lymph nodes of the left renal hilus and the tissue
UROLOGY
I
SEPTEMBER
1975 I
VOLUME
VI, NUMBER
3
FIGURE 1. Case 1. (A) Selective right renal arteriogram. Note arterial aneurysms in the vascular mass and elongated vessels stretched around multiple cystic regions. (B) Nephrogram phase. Note delay in passage of contrast through tumor vessels and multiple lucent cysts throughout the re(10 mainder of the kidney. (C) Arteriogram after intra-arterial epinephrine pg.). There is constriction of both normal and abnormal vessels. Note the collection of contrast material (arrows) corresponding to the large blood clot in Fig. 1D. (D) Right kidney specimen. There are multiple cysts throughout the kidney, and a large clot is present in a dilated middle pole calyx.
UROLOGY
i SEPTEMBER1975
/ VOLUMEVI,
NUMBER3
391
FIGURE 2. Case 2. Selective left renal arteriogram. A vascular mass containing multiple small s&cular aneurysms has replaced the upper pole. The mid portion of the kidney contains small avascular cysts. The lower pole was supplied by an accessory artery.
from the resected cyst wall of the right kidney all showed blood vessels, fat and smooth muscle consistent with angiomyolipoma. Comment Coexistence of renal tumors and polycystic disease creates difficult diagnostic and therapeutic problems. Each entity may produce palpable kidneys, pain, hematuria, and hypertension.‘s2 If the tumors are multiple, as in tuberous sclerosis, excretory urography may reveal the same enlarged kidneys with multiple masses, distorted collecting systems, and heterogeneous nephrogram seen in polycystic disease. The fatty components of angiomyolipomas may produce lucencies on plain films and the tumors may produce defects in the nephrogram which lack the thin walls and smooth borders of cysts. l*l”~ll At angiography, angiomyolipomas may reveal multiple small arterial aneurysms and a whorled appearance in the capillary phase
392
which may distinguish them from malignant tumors.‘,12-14 Polycystic disease causes stretching and bowing of otherwise normal vessels.15*16 Hypernephromas also enter the differential diagnosis of angiomyolipomas since both are vascular masses. Unfortunately, the small aneurysms and distinctive whorled nephrogram do not appear in all angiomyolipomasi2 and occasionally are seen in hypernephr0mas.l’ Since hypernephromas may metastasize to the kidneys or appear de novo in several renal sites, not all patients with multiple renal tumors can be assumed to have benign disease. Intraoperative diagnosis of angiomyolipomas may not be definite since their external appearance does not always permit them to be distinguished from hypernephromas.3 Clinical search for signs of tuberous sclerosis may be helpful in any patient with possibly benign renal masses; of the entire population of patients with angiomyolipomas, up to 50 per cent have been estimated to have this syndr0me.l’ These diagnostic difficulties should by no means prevent the physician from making a serious attempt to distinguish angiomyolipomas from malignant tumors. When the diagnosis cannot be made, these patients are often subjected to nephrectomy when less radical therapy might have been adequate.1,3-5 Patients with adult-type polycystic disease usually face an inexorable deterioration of renal function which is accelerated by unilateral nephrectomy. Nephrectomies should be avoided in patients with benign tumors and polycystic disease unless it is strongly believed that the natural history of the unresected tumor places the patient in greater peril than does life with a single polycystic kidney. Intraoperative examination of frozen sections of the mass may permit resection of the mass only despite the failure of such examination in Case 1. An isolated angiomyolipoma may be a forme fruste of the tuberous sclerosis syndrome.4’5 The discovery of multiple masses in Case 2 suggests that there is a continuous spectrum ranging from the isolated mass to the full-blown syndrome; similar findings have been reported in at least 3 other patients. 2 It is unlikely that angiomyolipomas in multiple sites represent metastases; probably true angiomyolipomas are hamartomas and never metastasize. 1,4*5 Gross hematuria or retroperitoneal hemorrhage has been found in a large fraction of patients with angiomyolipomas5 To date, however, no report of angiographic demonstration of active bleeding has appeared. The dense nephrogram
UROLOGY
I SEPTEMBER 1975 / VOLUMEVI, NUMBER3
on the selective arteriogram, as well as contrast in the collecting system, may be responsible for difficulty in visualizing active bleeding. Ifdemonstration of the bleeding site in Case 1 had been accomplished, surgical therapy more precise than total nephrectomy might have been feasible. Recently Biicheler et al. l8 have performed therapeutic embolization of a bleeding renal hamartoma during arteriography. Such procedure was contemplated in Case 1 but discarded because of the intermittent nature of the hematuria and the surgery which was ultimately made necessary by the perinephric abscess.
Department of Radiology Massachusetts General Hospital Boston, Massachusetts 02114 (DR. NEWHOUSE)
References 1. KHILNANI, M. B., and WOLF, B. S.: Hamartolipoma of the kidney: clinical and roentgen features, Am. J. Roentgenol. 86: 830 (1961). 2. KLAPPROTH, H. G., POUTASSE, E. F., and HAZARD, J. B.: Renal angiomyolipomas, Arch. Pathol. 67: 400 (1959). 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. PEROU, M. L., and GRAY, P. T.: Mesenchymal hamartomas of the kidney, ibid. 83: 240 (1969). 5. PRICE, E. B., and MOSTOFI, F. K.: Symptomatic angiomyolipoma of the kidney, Cancer 18: 761 (1965).
UROLOGY
I
SEPTEMBER 1975 / VOLUME VI, NUMBER 3
6. INGLIS, K.: The relation of the renal lesions to the cerebral lesions in the tuberous sclerosis complex, Am. J. Pathol. 30: 739 (1954). 7. POTTER, E. L.: Pathology of the Fetus and Infant, 2nd ed., Chicago, Year Book Medical Publishers, Inc., 1961, p. 441. 8. WRIGHT, F. W., LEDINGHAM, J. G. G., DUNNILL, M. S., and GRIEVE, N. W. T.: Polycystic kidneys, renal hamartomas, their variants and complications, Clin. Radiol. 25: 27 (1974). 9. SNOWDON, J, A.: Cerebral aneurysm, renal cysts and hamartomas in a case of tuberous sclerosis, Br. J. Ural. 46: 583 (1974). 10. LOVE, L., and FRANK, S. J. : Angiographic features of angiomyolipoma of the kidney, Am. J. Roentgenol. 95: 496 (1965). 11. CROSETT, A. D., JR.: Roentgenographic findings in the renal lesion of tuberous sclerosis, ibid. 98: 739 (1966). 12. CLARK, R. E., and PALUBINSKAS, A. J.: The angiographic spectrum of renal hamartomas, ibid. 114: 715 (1972). 13. BECKER, J. A., KINKHABWALA, H., POLLACK, H., and BOSNIAK, M. : Angiomyolipoma (hamartoma) of the kidney, Acta Radiol. Diag. 14: 561 (1973). 14. VIAMONTE, M., RAVEL, R., POLITANO, V., and BRIDGES, B.: Angiographic findings in a patient with tuberous sclerosis, Am. J, Roentgenol. 98: 723 (1966). 15. CORNELL, S . H . : Angiography in polycystic disease of the kidney, J. Ural. 103: 24 (1970). 16. ETTINGER, A., KAHN, P. C., and WISE, H. M., JR.: The importance of selective renal angiography in the diagnosis of polycystic disease, ibid. 102: 156 (1969). 17. WATSON, R. C., FLEMING, R. J., and EVANS, J. A.: Arteriography in the diagnosis of renal carcinoma, Radiology 91: 888 (1968). 18. B~~CHELER, E., WEIBHACH,L L., MUELLER, R., and THELEN, M .: Katheterembolisation eines blutenden Nierenhamartoms. Zugleich ein Beitrag zur angiographischen Diagnostik, Fortschr. Rontgenstr. 122: 107 (1975).
393
DISEASE
H. NEWHOUSE,
P. A. TUTSCHKA,
IN ADULT-TYPE
M.D.
M.D.
E. CLOUSE,
M.D.
A. LIBERTINO,
M.D.
From the Departments of Radiology and Urology, Harvard Medical School, New England Deaconess Hospital, and Massachusetts General Hospital, Boston, Massachusetts
- Two adults with renal angiomyolipomas and polycystic disease are presented with emphasis on the angiographic manifestations of the coexisting lesions. One patient had multiple episodes of gross hematuria and a perinephric abscess which resulted in bilateral nephrectomies; the second presented with hypertension and underwent unilateral nephrectomy to treat a hypervascular tumor. The clinical and radiographic diagnoses of the combined lesions are discussed and suggestions for management offered. _ ABSTRACT
tuberant abdomen and palpable kidneys. An excretory urogram was interpreted as showing polycystic kidneys. Seizures beginning at age ten were easily controlled with diphenylhydantoin (Dilantin). At age sixteen, gross hematuria developed after a fall. Hematuria reappeared spontaneously two years later; both episodes were successfully managed conservatively. Several months prior to admission she experienced gross hematuria and flank pain following strenuous activity. She was admitted to another hospital where cystoscopy showed blood coming from the right ureteral orifice; 14 units of whole blood were administered. The patient was transferred to the New England Deaconess Hospital where physical examination revealed a right upper quadrant mass and right costovertebral angle tenderness. Stigmata of tuberous sclerosis were noted in the skin, retinas, fingernails, and roentgenograms of the bones. Blood pressure was 130/80 mm. Hg, hemoglobin 8.5 mg. per 100 ml., hematocrit 35, and blood urea nitrogen 24 mg. and creatinine 2.6 mg. per 100 ml. Plain film of the abdomen showed both kidneys to be 21 cm. in length with no visible lucencies,
Renal angiomyolipomas are benign tumors which may occur as solitary lesions or as the multiple renal masses associated with the tuberous sclerosis complex. l-5 Their association with polycystic renal disease appears to be rare. A review of the literature reveals only 7 probable cases; 2 in babies with histologic evidence of infantile-type polycystic disease, 6,7 3 in patients with proved adult-type polycystic disease,8,g and 2 in adults whose kidneys revealed multiple superficial cysts on gross examination but in whom histologic examination was confined to the tumor.2’4 Angiomyolipomas are benign and slow growing. Nevertheless, they have occasioned nephrectomies both by mimicking malignant tumors” and by causing spontaneous hemorrhage.’ Both patients described herein had renal angiomyolipomas associated with polycystic disease and underwent potentially avoidable nephrectomies. Case Reports Case 1
A nineteen-year-old white female was admitted with a chief complaint of uncontrollable hematuria. At age two she was noted to have a pro-
UROLOGY
/ SEPTEMBER
1975
/ VOLUME
VI, NUMBER
3
G89
and urogram showed bilateral elongation, distortion, and compression of the pyelocalyceal systems. Selective right renal arteriography revealed a hypervascular mass in the midportion of the right kidney (Fig. 1A). Several interlobar arteries supplying the tumor lacked normal tapering, were enlarged, and contained small saccular aneurysms. Venous opacification was prolonged; the nephrogram formed an “onion peel” pattern (Fig. 1B). Multiple small lucent masses varied in size from several millimeters to 3 cm. Late films showed opacification of a sac-like structure in the middle pole of the kidney (Fig. 1C). Extravasation of contrast was not sufficient to localize a bleeding point. Arteriography, after 10 pg. of intra-arterial epinephrine, revealed moderate constriction of the tumor vessels and marked constriction of the remainder of the vasculature. Selective left renal arteriography showed multiple avascular masses; abnormal vessels with small saccular aneurysms were also present. The bleeding continued, and the patient received massive transfusions of leukocyte-free frozen blood. During right nephrectomy, frozen sections of the mass were interpreted as renal carcinoma; permanent sections were typical of angiomyolipoma. The kidney contained numerous cysts in the cortex and medulla. A large clot in a dilated middle pole calyx corresponded to the contrast collection seen at arteriography (Fig. 1D). Recovery was uneventful. Shortly before the last admission, spontaneous gross hematuria reappeared. Except for the surgical scar and hypertension (blood pressure 180/110 mm. Hg), her physical condition was unchanged. Blood urea nitrogen had risen to 35 mg. and creatinine to 4.2 mg. per 100 ml. Intermittent gross hematuria continued for two weeks. Angiography was considered on several occasions to locate the exact bleeding site and to control hemorrhage by embolization with autologous clot; on each occasion, spontaneous remission of hemorrhage forestalled the procedure. The patient then became septic with temperature rising as high as 104” F. Urine culture grew enterococcus. Antibiotic therapy was unsuccessful, and she underwent a left nephrectomy with prompt defervescence. Pathologic examination of the kidney revealed multiple cortical and medullary cysts and angiomyolipomas with evidence of an acute perinephric abscess. Case 2 A forty-eight-year-old white ferred to Walter Reed General
390
female was reHospital for re-
section of a renal tumor. Elevated blood pressure ranging from 1401100 to 170/110 mm. Hg had been detected several months prior to admission. The patient complained of intermittent headaches and dizziness but denied all other symptoms associated with hypertension, renal disease, or tuberous sclerosis. There was no family history of these diseases. Physical examination revealed a well-developed, well-nourished white female with pulse 88, respiration 22, and blood pressure 186/120 mm. Hg. Positive physical findings included a systolic murmur at the apex with a prominent apical impulse. The liver was palpable 3 fingerbreadths below the costal margin. There were no stigmata of tuberous sclerosis in the skin, retinas, or fingernails. The kidneys were not palpable. Complete blood count, serum electrolytes, blood urea nitrogen, creatinine, uric acid cholesterol, total albumin, bilirubin, alkaline phosphatase, lactic acid dehydrogenase, glutamic oxalic transaminase, and urine vanylmandelic acid were normal. Urinalysis revealed microscopic hematuria. Electrocardiography showed signs of left ventricular hypertrophy. No radiographs of the skull or long bones were obtained. The chest film was normal. Excretory urography revealed markedly enlarged kidneys. The nephrogram was faint and mottled with a “Swiss cheese” appearance; the pyelocalyceal systems were splayed. No abnormal fat densities were visible on plain film of the abdomen. Bilateral selective arteriography showed enlarged kidneys with stretched intrarenal vessels. There was a hypervascular mass involving the left upper pole with tortuous vessels, multiple arterial aneurysms 2 to 3 mm. in diameter, and a heterogeneous capillary phase (Fig. 2). At surgery, the liver was found to be covered with multiple superficial cysts. The right kidney contained multiple cysts; the wall of the largest of these was resected. There was a mass within the upper pole of the left kidney which was well encapsulated and did not involve the adrenal gland. The left kidney, adrenal gland, perirenal fat, and Gerota’s fascia were excised en bloc. Postoperative recovery was uneventful. Pathologic examination of the left kidney revealed multiple cortical and medullary cysts of various diameters. A large mass replaced the upper pole of the kidney and multiple smaller solid nodules were scattered throughout the renal parenchyma. These masses, tissue found in the lymph nodes of the left renal hilus and the tissue
UROLOGY
I
SEPTEMBER
1975 I
VOLUME
VI, NUMBER
3
FIGURE 1. Case 1. (A) Selective right renal arteriogram. Note arterial aneurysms in the vascular mass and elongated vessels stretched around multiple cystic regions. (B) Nephrogram phase. Note delay in passage of contrast through tumor vessels and multiple lucent cysts throughout the re(10 mainder of the kidney. (C) Arteriogram after intra-arterial epinephrine pg.). There is constriction of both normal and abnormal vessels. Note the collection of contrast material (arrows) corresponding to the large blood clot in Fig. 1D. (D) Right kidney specimen. There are multiple cysts throughout the kidney, and a large clot is present in a dilated middle pole calyx.
UROLOGY
i SEPTEMBER1975
/ VOLUMEVI,
NUMBER3
391
FIGURE 2. Case 2. Selective left renal arteriogram. A vascular mass containing multiple small s&cular aneurysms has replaced the upper pole. The mid portion of the kidney contains small avascular cysts. The lower pole was supplied by an accessory artery.
from the resected cyst wall of the right kidney all showed blood vessels, fat and smooth muscle consistent with angiomyolipoma. Comment Coexistence of renal tumors and polycystic disease creates difficult diagnostic and therapeutic problems. Each entity may produce palpable kidneys, pain, hematuria, and hypertension.‘s2 If the tumors are multiple, as in tuberous sclerosis, excretory urography may reveal the same enlarged kidneys with multiple masses, distorted collecting systems, and heterogeneous nephrogram seen in polycystic disease. The fatty components of angiomyolipomas may produce lucencies on plain films and the tumors may produce defects in the nephrogram which lack the thin walls and smooth borders of cysts. l*l”~ll At angiography, angiomyolipomas may reveal multiple small arterial aneurysms and a whorled appearance in the capillary phase
392
which may distinguish them from malignant tumors.‘,12-14 Polycystic disease causes stretching and bowing of otherwise normal vessels.15*16 Hypernephromas also enter the differential diagnosis of angiomyolipomas since both are vascular masses. Unfortunately, the small aneurysms and distinctive whorled nephrogram do not appear in all angiomyolipomasi2 and occasionally are seen in hypernephr0mas.l’ Since hypernephromas may metastasize to the kidneys or appear de novo in several renal sites, not all patients with multiple renal tumors can be assumed to have benign disease. Intraoperative diagnosis of angiomyolipomas may not be definite since their external appearance does not always permit them to be distinguished from hypernephromas.3 Clinical search for signs of tuberous sclerosis may be helpful in any patient with possibly benign renal masses; of the entire population of patients with angiomyolipomas, up to 50 per cent have been estimated to have this syndr0me.l’ These diagnostic difficulties should by no means prevent the physician from making a serious attempt to distinguish angiomyolipomas from malignant tumors. When the diagnosis cannot be made, these patients are often subjected to nephrectomy when less radical therapy might have been adequate.1,3-5 Patients with adult-type polycystic disease usually face an inexorable deterioration of renal function which is accelerated by unilateral nephrectomy. Nephrectomies should be avoided in patients with benign tumors and polycystic disease unless it is strongly believed that the natural history of the unresected tumor places the patient in greater peril than does life with a single polycystic kidney. Intraoperative examination of frozen sections of the mass may permit resection of the mass only despite the failure of such examination in Case 1. An isolated angiomyolipoma may be a forme fruste of the tuberous sclerosis syndrome.4’5 The discovery of multiple masses in Case 2 suggests that there is a continuous spectrum ranging from the isolated mass to the full-blown syndrome; similar findings have been reported in at least 3 other patients. 2 It is unlikely that angiomyolipomas in multiple sites represent metastases; probably true angiomyolipomas are hamartomas and never metastasize. 1,4*5 Gross hematuria or retroperitoneal hemorrhage has been found in a large fraction of patients with angiomyolipomas5 To date, however, no report of angiographic demonstration of active bleeding has appeared. The dense nephrogram
UROLOGY
I SEPTEMBER 1975 / VOLUMEVI, NUMBER3
on the selective arteriogram, as well as contrast in the collecting system, may be responsible for difficulty in visualizing active bleeding. Ifdemonstration of the bleeding site in Case 1 had been accomplished, surgical therapy more precise than total nephrectomy might have been feasible. Recently Biicheler et al. l8 have performed therapeutic embolization of a bleeding renal hamartoma during arteriography. Such procedure was contemplated in Case 1 but discarded because of the intermittent nature of the hematuria and the surgery which was ultimately made necessary by the perinephric abscess.
Department of Radiology Massachusetts General Hospital Boston, Massachusetts 02114 (DR. NEWHOUSE)
References 1. KHILNANI, M. B., and WOLF, B. S.: Hamartolipoma of the kidney: clinical and roentgen features, Am. J. Roentgenol. 86: 830 (1961). 2. KLAPPROTH, H. G., POUTASSE, E. F., and HAZARD, J. B.: Renal angiomyolipomas, Arch. Pathol. 67: 400 (1959). 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. PEROU, M. L., and GRAY, P. T.: Mesenchymal hamartomas of the kidney, ibid. 83: 240 (1969). 5. PRICE, E. B., and MOSTOFI, F. K.: Symptomatic angiomyolipoma of the kidney, Cancer 18: 761 (1965).
UROLOGY
I
SEPTEMBER 1975 / VOLUME VI, NUMBER 3
6. INGLIS, K.: The relation of the renal lesions to the cerebral lesions in the tuberous sclerosis complex, Am. J. Pathol. 30: 739 (1954). 7. POTTER, E. L.: Pathology of the Fetus and Infant, 2nd ed., Chicago, Year Book Medical Publishers, Inc., 1961, p. 441. 8. WRIGHT, F. W., LEDINGHAM, J. G. G., DUNNILL, M. S., and GRIEVE, N. W. T.: Polycystic kidneys, renal hamartomas, their variants and complications, Clin. Radiol. 25: 27 (1974). 9. SNOWDON, J, A.: Cerebral aneurysm, renal cysts and hamartomas in a case of tuberous sclerosis, Br. J. Ural. 46: 583 (1974). 10. LOVE, L., and FRANK, S. J. : Angiographic features of angiomyolipoma of the kidney, Am. J. Roentgenol. 95: 496 (1965). 11. CROSETT, A. D., JR.: Roentgenographic findings in the renal lesion of tuberous sclerosis, ibid. 98: 739 (1966). 12. CLARK, R. E., and PALUBINSKAS, A. J.: The angiographic spectrum of renal hamartomas, ibid. 114: 715 (1972). 13. BECKER, J. A., KINKHABWALA, H., POLLACK, H., and BOSNIAK, M. : Angiomyolipoma (hamartoma) of the kidney, Acta Radiol. Diag. 14: 561 (1973). 14. VIAMONTE, M., RAVEL, R., POLITANO, V., and BRIDGES, B.: Angiographic findings in a patient with tuberous sclerosis, Am. J, Roentgenol. 98: 723 (1966). 15. CORNELL, S . H . : Angiography in polycystic disease of the kidney, J. Ural. 103: 24 (1970). 16. ETTINGER, A., KAHN, P. C., and WISE, H. M., JR.: The importance of selective renal angiography in the diagnosis of polycystic disease, ibid. 102: 156 (1969). 17. WATSON, R. C., FLEMING, R. J., and EVANS, J. A.: Arteriography in the diagnosis of renal carcinoma, Radiology 91: 888 (1968). 18. B~~CHELER, E., WEIBHACH,L L., MUELLER, R., and THELEN, M .: Katheterembolisation eines blutenden Nierenhamartoms. Zugleich ein Beitrag zur angiographischen Diagnostik, Fortschr. Rontgenstr. 122: 107 (1975).
393
![[Renal angiomyolipoma].](/assets/img/hold.png)
