Renal Hamartoma (Angiomyolipoma) and the Tuberous Sclerosis Complex Andresito B. Pacis, MD, and Calvin H. Norman, Jr., MD Brooklyn, New York
Renal hamartoma is found in 40 to 80 percent of patients with tuberous sclerosis. Microscopic demonstration of fat in the tissues of the mass is felt to be the most reliable diagnostic criterion of hamartoma. Characteristically, the angiographic appearance demonstrates a large, dilated feeding vessel passing through the mass with multiple, multisacculated aneurysmal dilatations appearing like bunches of grapes. There is a delicate neovascularity without A-V shunting and an onion-peel or whorl-like appearance in the venous phase. This case is presented to point out the close association of renal hamartoma and tuberous sclerosis and the need to search for renal hamartoma when the diagnosis of tuberous sclerosis is made. Hamartoma is defined as an abnormal, disorganized development of mature normal cells in an organ composed of identical cellular elements and represents a tumor-like nonneoplastic growth. Pathologically, renal hamartoma is classified into two types: 1. The small medullary hamartoma, which is rarely of clinical significance, although it may calcify. It is usually found only as an incidental finding at necropsy. 2. Mixed mesenchymal hamartoma, which is present in approximately 40 to 80 percent of patients with tuberous sclerosis. '
Incidence There is a predominance in females, usually adults. It usually affects the 20 to 40 age group.2 Those with solitary lesions in the kidney vary from 21 to 72 years, with an average of 48 years of age. Patients with tuberous sclerosis tend to be younger usually in the 11 to From the Department of Radiology, Catholic Medical Center, St. Mary's Hospital Division, Brooklyn, New York. Requests for reprints should be addressed to Dr. Calvin H. Norman, Jr., Department of Radiology, St. Mary's Hospital, 1298 St. Mark's Avenue, Brooklyn, NY 11213.
41 year age range with an average of 30.6 years of age.3 Although renal hamartomas in the pediatric age group are extremely rare, in the neonatal period it is as common as Wilm tumor.46f About 20 percent of renal hamartomas, reported and illustrated in the literature by angiography, are associated with tuberous sclerosis.7 The mixed mesenchymal hamartoma or the medullary type may occur in any organ, but is most common in the brain, liver, kidney, spleen, and lung. Aside from these general areas, lesions in the skin, retina, and heart are also sites of predilection.8-'0 Other findings in tuberous sclerosis are: 1. Skin anomalies take several forms. The most common, which is seen in about 50 percent of cases, is the so-called adenoma sebaceum. These are typically a group of nodules occuring in the area near the nose and on the face, which histologically show either increased dermal collagen or proliferation of sebaceous glands. In the second form, the so-called shagreen patches are found in the sacral area. The third form is characterized by cafe-au-lait spots, which are localized pigmentations in the skin. The last form is the subungual fibroma found around or beneath the nail beds.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 1, 1979
2. Retinal lesions, which consist of elevated nodules or plaques made of glial tissue similar to that in the CNS, are known as phacoma. 3. Cardiac lesions, in the form of rhabdomyomas, are localized abnormal myocardial muscle fibers distended with glycogen. They may be single or multiple. When single, rhabdomyomas usually occur in the absence of tuberous sclerosis. In tuberous sclerosis with renal hamartoma, the usual presenting clinical symptoms and signs are vague pain or discomfort in one or both flanks; gross or microscopic hematuria;3"'I and flank mass, which may be vague or definitely palpable. 12"13 Spontaneous massive retroperitoneal hemorrhage is rather rare, but has been reported.12'14
Case Report A 42-year-old black male patient presented with bilateral flank pain radiating to both lower quadrants of the abdomen. This had occurred on and off for the past eight months and was associated with hesitancy and straining, but no dribbling. The patient denied hematuria, frequency, burning, or pyuria. The physical examination revealed typical adenoma sebaceum (Figure 1) distributed around the nose in a "butterfly-like" pattern. There were pigmented papillary projections in the oral buccal mucosa and around the nail beds (Figure 2). Shagreen patches were present in the sacral area and lower lumbar region. There were no abnormalities seen in the retina, nor abnormal palpable masses in the abdomen. The lungs were clear. The patient did not show evidence of mental defi-
ciency. On the basis of the above physical and clinical findings, a clinical impression of tuberous sclerosis was made and further roentgenographic examinations were ordered. Plain film of the 55
Figure 1. The face shows the typical "adenoma sebaceum" distributed in a butterfly-like fashion around the nose.
skull (Figure 3) revealed the classical appearance and distribution of brain calcification in the paraventricular region. A bone survey revealed the typical wavy periosteal reaction and thickening along the shafts of the metatarsals of both feet (Figure 4). Other bony findings described in the literature, such as the patchy localized densities or bony islands, which may simulate osteoblastic metastases or osteopoikilosis, 15 were not demonstrated. An intravenous urogram showed several hyperradiant translucent masses splaying and stretching the calyceal pattern of both intrarenal collecting systems, simulating a polycystic kidney. The kidneys were slightly enlarged. A bilateral selective renal arteriogram demonstrated stretching and splaying of the intrarenal vessels with a few clusters of small sac-like aneurysms (Figure 5). The onion-peel or whorl-like appearance in the late venous phase was not demonstrated. The main feeding artery, however, was prominently large. A radiologic diagnosis of renal hamartoma was made. At operation, a brownish, firm, yellowish, hard nodularity was noted in the lower pole of the right kidney. Palpable, hard nodules were similarly noted on the left side. There were dilated vessels on the surface of the kidney. Histologic sections from the surgically removed specimens from each kidney showed interlacing bundles of mature, smooth muscles with small, but dilated capillary lumena. There 56
Figure 2. Subungual fibromas are seen around the nail beds.
were areas of fat and fibrosis. These findings were consistent with an-
giomyolipoma.
Discussion The characteristic and most significant angiographic feature of renal hamartomas has been extensively described in the literature.9,13'16-18 The main feeding artery was large, dilated and passed through the mass. Multiple sacculated aneurysmal dilatation (bunch of grapes), whorled or onionpeeling in the venous phase and delicate neovascularity without A-V shunting of intrarenal vessels were noted. The onion peeling is produced by the contrast medium in small veins interspersed with layers of fat, giving concentric layers of density and radiolucencies.9.13 The aneurysmal dilatations showed histologically an absence of fragmentation of the internal elastic lamina, and abnormality of the intima, which is "heaped-up" in places giving the appearance of an eccentric lumen. The characteristic mass on the intravenous urogram and nephrotomogram presents as irregular radiolucencies when fat predominates. 19 Demonstration of fat within the mass is felt to be the most reliable diagnostic criterion of hamartoma.9"16"19 The preoperative diagnosis of renal hamartoma in a patient without tuberous sclerosis is difficult. Approximately 68 percent of renal hamartomas are not angiographically
distinguishable from renal cell carcinoma.20 Renal hamartoma shows a neoplastic response to intraarterial epinephrine (lack of vasoconstriction).21'23.27 It may also be hypervascularized.2'24-26 There is no explanation for the occurrence of solitary hamartoma in patients without tuberous sclerosis,16 although the tumors histopathologically are the same.2 Clarke et a17 in 1972 and McCallum, 17 in 1975, reviewed a total of 26 cases in the literature of renal hamartomas not associated with tuberous sclerosis. They described the following angiographic characteristics: 1. avascular radiolucent renal mass, one (four percent); 2. neovascularity indistinguishable from malignant renal tumors, 18 (68 percent); 3. multisacculated pseudoaneurysms of the pathologic vessels with whorled or onion-peel appearance on the venous phase and well-defined lucent areas in the nephrogram phase from the fatty elements, four (16 percent); and 4. delicate, discrete, neovascularity of renal hamartomas supplied by normal interlobar arteries, three (12 percent). The following guidelines have been proposed for the management of renal hamartomas: 1. In a patient with a unilateral solid tumor without stigmata of tuberous sclerosis, and in whom accurate differentiation between hamartoma and renal cell carcinoma by any reliable method
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 1, 1979
T
p
Figure 3. Brain calcification in the praventricular region. Figure 4. The right foot illustrates the "wavy periosteal reaction and thickening" along the shaft of the metatarsals.
does not exist, nephrectomy is indicated. 2. In a patient with multiple solid tumors, in the absence of tuberous
sclerosis, multiple angiomyolipomas should be considered and at exploration, a definite diagnosis should be made on one kidney before removing the other, if both are involved. 3. In a patient with unilateral solid tumor with stigmata of tuberous sclerosis, an open biopsy is in order and partial nephrectomy should be considered. 4. In a patient with solid tumors with stigmata of tuberous sclerosis, conservative management should be employed, unless there is massive enlargement of the tumor and severe symptoms necessitate exploration.
Comment aongiograpyieod fl.ob adfntv In a review prcdr. of the literature, Theapresencofat includumr withinli et at3 ing their own 18 cases, Becker angiohave observed that the typical tenlaresiont appar tohe theohamartoma mostsvreli of renal graphic appearance ablediagomsti xportin neesign.1 can closely mimic renal cefl carcinoma, and, in fact, have been seen in some cases of carcinoma. Likewise, both lesions exhibited a neoplastic response to intraarterial epinephrine. Therefore,
In patients where a diagnosis of tuberous sclerosis has been definitely established, a compromising operation should be avoided and resection should be withheld, if at all possible. These patients require as much functioning renal tissue as can be preserved. Many patients with tuberous sclerosis live for years with these tumors with only slowly decreasing renal function.27 Literature Cited 1. Wright FW, Ledingham GG, Dunnill MS, et al: Polycystic kidneys, renal hamartomas, disease variants and complications. Clin Radiol 25:27-43, 1974 2. Klapproth HJ, Pontasse EF, Hazard B: Renal angiomyolipoma: Report of 4 cases. Arch Path 67:400-411, 1959 3. Becker JA, Kinkhabwala M, Pollack H, et al: Angiomyolipoma (Hamartoma) angiographic review. Acta Radiol [Diag] (Stockh) 14(5):561-568, 1973 4. Favarra BE, Johnson W, Ito J: Renal tumors in neonatal period. Cancer 22:845-855, 1968 5. Kay S, Pratt CB, Salzberg AM: Hamartoma (Lipoangiomyoma) of the kidney. Acta Radiol [Diag] (Stockh) 7:417-421, 1968 6. Poole C, Viamonte M, Jr: Unusual renal masses in the pediatric age group. Am J Roentgenol Radium Ther NucI Med 109:368379, 1970 7. Clark ER, Palubinskas AJ: The angiographic spectrum of renal hamartoma. Am J Roentgenol Radium Ther NucI Med 114(4):715-721, 1972 8. Bissada K, White J, Chao N, et al: Tuberous sclerosis complex and renal angiomyolipoma. Urology 6(1 ):105-1 13, 1975 9. Viamonte M Jr, Politano V, Ravel R, et al: Angiographic findings in a patient with tuberous sclerosis. Am J Roentgenol Radium Ther NucI Med 98:723-733, 1966 10. Moolten SF: Hamartial nature of tuberous sclerosis complex and its bearing on tumor problem: Report of cases with tumor anomaly of the kidney and adenoma sebaceum. Arch Intern Med 69:589-623, 1942
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 1, 1979
Figure 5. Film of the right selective renal arteriogram showing the main large feeding artery and clusters of sac-like aneurysmally dilated vessels. Note the hyperradiant translucency in the middle and lower pole representing the high fat content of the tumor. 11. Gilbert EC, Edison M, Urschel WP: Tuberous sclerosis associated with bilateral renal hamartomas: Report of a case. Med Ann DC 35:322-324, 1966 12. Allen TD, Risk W: Renal angiomyolipoma. J Urol 94:203-207, 1965 13. Khrlnam M, Wolf BS: Hamartoma of the kidney, clinical and roentgenographic manifestation. Am J Roentgenol Radium Ther NucI Med 86:830-841, 1961 14. Vasko JS, Brockman SK, Bomar RL: Renal angiomyolipoma-a rare case of spontaneous massive retroperitoneal hemorrhage. Ann Surg 161:577-681, 1965 15. Ashby DW, Rampage D: Lesions of the vertebra and innominate bones in tuberous sclerosis. Br J Radiol 30:274-277, 1957 16. Won J: Angiographic manifestations of renal hamartoma. Angiology 28(6):416-420, 1977 17. McCallum R: The pre-operative diagnosis of renal hamartoma. Clin Radiol 26:257260, 1975 18. Wilins JP, Dosogne M, Collette M: Un case d' hamartome renal. Acta Urol BeIg 41 (3):432-436, 1973 19. Adelman BP: Angiomyolipoma of kidney. Am J Roentgenol Radium Therapy Nucl Med 95:403-405, 1965 20. Falkner S, Junhagen P, Rais D, et al: Hamartoma, (Lipoangiomyoma) of the kidney. Acta Radiol [Diag] (Stockh) 7:417424, 1968 21. Abrams HL, Boijsen E, Borjstrom KE: Effect of epinephrine on the renal circulation: Angiographic observations. Radiology 79:911922, 1962 22. Palmisano JP: Renal hamartoma (angiomyolipoma): Its angiographic appearance and response to intraarterial epinephrine. Radiology 88:249-252, 1967 23. Rockoff SD, Doppman J, Block JB, et al: Variable response of tumor vessels to intraarterial epinephrine: An angiographic study in man. Invest Radiol 1:205-213, 1966 24. Blackwell JB, Sanders JM, Vaughn BF: Renal Hamartoma. Aust NZ J Surg 34:128-132, 1964 25. Lowe L, Frank SJ: Angiographic features of angiomyolipoma of the kidney. Am J Roentgenol Radium Ther NucI Med 95:406409, 1965. 26. Landgren WJ: Renal hamartomata, case report and review, Australas Radiol 3:232-237, 1966 27. Farrow GM, Harrison EG, Jones DR: Renal angiomyolipoma-clinico-pathologic study of 32 cases. Cancer 22:556-563, 1968 57
Renal hamartoma is found in 40 to 80 percent of patients with tuberous sclerosis. Microscopic demonstration of fat in the tissues of the mass is felt to be the most reliable diagnostic criterion of hamartoma. Characteristically, the angiographic appearance demonstrates a large, dilated feeding vessel passing through the mass with multiple, multisacculated aneurysmal dilatations appearing like bunches of grapes. There is a delicate neovascularity without A-V shunting and an onion-peel or whorl-like appearance in the venous phase. This case is presented to point out the close association of renal hamartoma and tuberous sclerosis and the need to search for renal hamartoma when the diagnosis of tuberous sclerosis is made. Hamartoma is defined as an abnormal, disorganized development of mature normal cells in an organ composed of identical cellular elements and represents a tumor-like nonneoplastic growth. Pathologically, renal hamartoma is classified into two types: 1. The small medullary hamartoma, which is rarely of clinical significance, although it may calcify. It is usually found only as an incidental finding at necropsy. 2. Mixed mesenchymal hamartoma, which is present in approximately 40 to 80 percent of patients with tuberous sclerosis. '
Incidence There is a predominance in females, usually adults. It usually affects the 20 to 40 age group.2 Those with solitary lesions in the kidney vary from 21 to 72 years, with an average of 48 years of age. Patients with tuberous sclerosis tend to be younger usually in the 11 to From the Department of Radiology, Catholic Medical Center, St. Mary's Hospital Division, Brooklyn, New York. Requests for reprints should be addressed to Dr. Calvin H. Norman, Jr., Department of Radiology, St. Mary's Hospital, 1298 St. Mark's Avenue, Brooklyn, NY 11213.
41 year age range with an average of 30.6 years of age.3 Although renal hamartomas in the pediatric age group are extremely rare, in the neonatal period it is as common as Wilm tumor.46f About 20 percent of renal hamartomas, reported and illustrated in the literature by angiography, are associated with tuberous sclerosis.7 The mixed mesenchymal hamartoma or the medullary type may occur in any organ, but is most common in the brain, liver, kidney, spleen, and lung. Aside from these general areas, lesions in the skin, retina, and heart are also sites of predilection.8-'0 Other findings in tuberous sclerosis are: 1. Skin anomalies take several forms. The most common, which is seen in about 50 percent of cases, is the so-called adenoma sebaceum. These are typically a group of nodules occuring in the area near the nose and on the face, which histologically show either increased dermal collagen or proliferation of sebaceous glands. In the second form, the so-called shagreen patches are found in the sacral area. The third form is characterized by cafe-au-lait spots, which are localized pigmentations in the skin. The last form is the subungual fibroma found around or beneath the nail beds.
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 1, 1979
2. Retinal lesions, which consist of elevated nodules or plaques made of glial tissue similar to that in the CNS, are known as phacoma. 3. Cardiac lesions, in the form of rhabdomyomas, are localized abnormal myocardial muscle fibers distended with glycogen. They may be single or multiple. When single, rhabdomyomas usually occur in the absence of tuberous sclerosis. In tuberous sclerosis with renal hamartoma, the usual presenting clinical symptoms and signs are vague pain or discomfort in one or both flanks; gross or microscopic hematuria;3"'I and flank mass, which may be vague or definitely palpable. 12"13 Spontaneous massive retroperitoneal hemorrhage is rather rare, but has been reported.12'14
Case Report A 42-year-old black male patient presented with bilateral flank pain radiating to both lower quadrants of the abdomen. This had occurred on and off for the past eight months and was associated with hesitancy and straining, but no dribbling. The patient denied hematuria, frequency, burning, or pyuria. The physical examination revealed typical adenoma sebaceum (Figure 1) distributed around the nose in a "butterfly-like" pattern. There were pigmented papillary projections in the oral buccal mucosa and around the nail beds (Figure 2). Shagreen patches were present in the sacral area and lower lumbar region. There were no abnormalities seen in the retina, nor abnormal palpable masses in the abdomen. The lungs were clear. The patient did not show evidence of mental defi-
ciency. On the basis of the above physical and clinical findings, a clinical impression of tuberous sclerosis was made and further roentgenographic examinations were ordered. Plain film of the 55
Figure 1. The face shows the typical "adenoma sebaceum" distributed in a butterfly-like fashion around the nose.
skull (Figure 3) revealed the classical appearance and distribution of brain calcification in the paraventricular region. A bone survey revealed the typical wavy periosteal reaction and thickening along the shafts of the metatarsals of both feet (Figure 4). Other bony findings described in the literature, such as the patchy localized densities or bony islands, which may simulate osteoblastic metastases or osteopoikilosis, 15 were not demonstrated. An intravenous urogram showed several hyperradiant translucent masses splaying and stretching the calyceal pattern of both intrarenal collecting systems, simulating a polycystic kidney. The kidneys were slightly enlarged. A bilateral selective renal arteriogram demonstrated stretching and splaying of the intrarenal vessels with a few clusters of small sac-like aneurysms (Figure 5). The onion-peel or whorl-like appearance in the late venous phase was not demonstrated. The main feeding artery, however, was prominently large. A radiologic diagnosis of renal hamartoma was made. At operation, a brownish, firm, yellowish, hard nodularity was noted in the lower pole of the right kidney. Palpable, hard nodules were similarly noted on the left side. There were dilated vessels on the surface of the kidney. Histologic sections from the surgically removed specimens from each kidney showed interlacing bundles of mature, smooth muscles with small, but dilated capillary lumena. There 56
Figure 2. Subungual fibromas are seen around the nail beds.
were areas of fat and fibrosis. These findings were consistent with an-
giomyolipoma.
Discussion The characteristic and most significant angiographic feature of renal hamartomas has been extensively described in the literature.9,13'16-18 The main feeding artery was large, dilated and passed through the mass. Multiple sacculated aneurysmal dilatation (bunch of grapes), whorled or onionpeeling in the venous phase and delicate neovascularity without A-V shunting of intrarenal vessels were noted. The onion peeling is produced by the contrast medium in small veins interspersed with layers of fat, giving concentric layers of density and radiolucencies.9.13 The aneurysmal dilatations showed histologically an absence of fragmentation of the internal elastic lamina, and abnormality of the intima, which is "heaped-up" in places giving the appearance of an eccentric lumen. The characteristic mass on the intravenous urogram and nephrotomogram presents as irregular radiolucencies when fat predominates. 19 Demonstration of fat within the mass is felt to be the most reliable diagnostic criterion of hamartoma.9"16"19 The preoperative diagnosis of renal hamartoma in a patient without tuberous sclerosis is difficult. Approximately 68 percent of renal hamartomas are not angiographically
distinguishable from renal cell carcinoma.20 Renal hamartoma shows a neoplastic response to intraarterial epinephrine (lack of vasoconstriction).21'23.27 It may also be hypervascularized.2'24-26 There is no explanation for the occurrence of solitary hamartoma in patients without tuberous sclerosis,16 although the tumors histopathologically are the same.2 Clarke et a17 in 1972 and McCallum, 17 in 1975, reviewed a total of 26 cases in the literature of renal hamartomas not associated with tuberous sclerosis. They described the following angiographic characteristics: 1. avascular radiolucent renal mass, one (four percent); 2. neovascularity indistinguishable from malignant renal tumors, 18 (68 percent); 3. multisacculated pseudoaneurysms of the pathologic vessels with whorled or onion-peel appearance on the venous phase and well-defined lucent areas in the nephrogram phase from the fatty elements, four (16 percent); and 4. delicate, discrete, neovascularity of renal hamartomas supplied by normal interlobar arteries, three (12 percent). The following guidelines have been proposed for the management of renal hamartomas: 1. In a patient with a unilateral solid tumor without stigmata of tuberous sclerosis, and in whom accurate differentiation between hamartoma and renal cell carcinoma by any reliable method
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 1, 1979
T
p
Figure 3. Brain calcification in the praventricular region. Figure 4. The right foot illustrates the "wavy periosteal reaction and thickening" along the shaft of the metatarsals.
does not exist, nephrectomy is indicated. 2. In a patient with multiple solid tumors, in the absence of tuberous
sclerosis, multiple angiomyolipomas should be considered and at exploration, a definite diagnosis should be made on one kidney before removing the other, if both are involved. 3. In a patient with unilateral solid tumor with stigmata of tuberous sclerosis, an open biopsy is in order and partial nephrectomy should be considered. 4. In a patient with solid tumors with stigmata of tuberous sclerosis, conservative management should be employed, unless there is massive enlargement of the tumor and severe symptoms necessitate exploration.
Comment aongiograpyieod fl.ob adfntv In a review prcdr. of the literature, Theapresencofat includumr withinli et at3 ing their own 18 cases, Becker angiohave observed that the typical tenlaresiont appar tohe theohamartoma mostsvreli of renal graphic appearance ablediagomsti xportin neesign.1 can closely mimic renal cefl carcinoma, and, in fact, have been seen in some cases of carcinoma. Likewise, both lesions exhibited a neoplastic response to intraarterial epinephrine. Therefore,
In patients where a diagnosis of tuberous sclerosis has been definitely established, a compromising operation should be avoided and resection should be withheld, if at all possible. These patients require as much functioning renal tissue as can be preserved. Many patients with tuberous sclerosis live for years with these tumors with only slowly decreasing renal function.27 Literature Cited 1. Wright FW, Ledingham GG, Dunnill MS, et al: Polycystic kidneys, renal hamartomas, disease variants and complications. Clin Radiol 25:27-43, 1974 2. Klapproth HJ, Pontasse EF, Hazard B: Renal angiomyolipoma: Report of 4 cases. Arch Path 67:400-411, 1959 3. Becker JA, Kinkhabwala M, Pollack H, et al: Angiomyolipoma (Hamartoma) angiographic review. Acta Radiol [Diag] (Stockh) 14(5):561-568, 1973 4. Favarra BE, Johnson W, Ito J: Renal tumors in neonatal period. Cancer 22:845-855, 1968 5. Kay S, Pratt CB, Salzberg AM: Hamartoma (Lipoangiomyoma) of the kidney. Acta Radiol [Diag] (Stockh) 7:417-421, 1968 6. Poole C, Viamonte M, Jr: Unusual renal masses in the pediatric age group. Am J Roentgenol Radium Ther NucI Med 109:368379, 1970 7. Clark ER, Palubinskas AJ: The angiographic spectrum of renal hamartoma. Am J Roentgenol Radium Ther NucI Med 114(4):715-721, 1972 8. Bissada K, White J, Chao N, et al: Tuberous sclerosis complex and renal angiomyolipoma. Urology 6(1 ):105-1 13, 1975 9. Viamonte M Jr, Politano V, Ravel R, et al: Angiographic findings in a patient with tuberous sclerosis. Am J Roentgenol Radium Ther NucI Med 98:723-733, 1966 10. Moolten SF: Hamartial nature of tuberous sclerosis complex and its bearing on tumor problem: Report of cases with tumor anomaly of the kidney and adenoma sebaceum. Arch Intern Med 69:589-623, 1942
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 1, 1979
Figure 5. Film of the right selective renal arteriogram showing the main large feeding artery and clusters of sac-like aneurysmally dilated vessels. Note the hyperradiant translucency in the middle and lower pole representing the high fat content of the tumor. 11. Gilbert EC, Edison M, Urschel WP: Tuberous sclerosis associated with bilateral renal hamartomas: Report of a case. Med Ann DC 35:322-324, 1966 12. Allen TD, Risk W: Renal angiomyolipoma. J Urol 94:203-207, 1965 13. Khrlnam M, Wolf BS: Hamartoma of the kidney, clinical and roentgenographic manifestation. Am J Roentgenol Radium Ther NucI Med 86:830-841, 1961 14. Vasko JS, Brockman SK, Bomar RL: Renal angiomyolipoma-a rare case of spontaneous massive retroperitoneal hemorrhage. Ann Surg 161:577-681, 1965 15. Ashby DW, Rampage D: Lesions of the vertebra and innominate bones in tuberous sclerosis. Br J Radiol 30:274-277, 1957 16. Won J: Angiographic manifestations of renal hamartoma. Angiology 28(6):416-420, 1977 17. McCallum R: The pre-operative diagnosis of renal hamartoma. Clin Radiol 26:257260, 1975 18. Wilins JP, Dosogne M, Collette M: Un case d' hamartome renal. Acta Urol BeIg 41 (3):432-436, 1973 19. Adelman BP: Angiomyolipoma of kidney. Am J Roentgenol Radium Therapy Nucl Med 95:403-405, 1965 20. Falkner S, Junhagen P, Rais D, et al: Hamartoma, (Lipoangiomyoma) of the kidney. Acta Radiol [Diag] (Stockh) 7:417424, 1968 21. Abrams HL, Boijsen E, Borjstrom KE: Effect of epinephrine on the renal circulation: Angiographic observations. Radiology 79:911922, 1962 22. Palmisano JP: Renal hamartoma (angiomyolipoma): Its angiographic appearance and response to intraarterial epinephrine. Radiology 88:249-252, 1967 23. Rockoff SD, Doppman J, Block JB, et al: Variable response of tumor vessels to intraarterial epinephrine: An angiographic study in man. Invest Radiol 1:205-213, 1966 24. Blackwell JB, Sanders JM, Vaughn BF: Renal Hamartoma. Aust NZ J Surg 34:128-132, 1964 25. Lowe L, Frank SJ: Angiographic features of angiomyolipoma of the kidney. Am J Roentgenol Radium Ther NucI Med 95:406409, 1965. 26. Landgren WJ: Renal hamartomata, case report and review, Australas Radiol 3:232-237, 1966 27. Farrow GM, Harrison EG, Jones DR: Renal angiomyolipoma-clinico-pathologic study of 32 cases. Cancer 22:556-563, 1968 57
