THE Jou R,aAL OF U ROLOGY
Vol. 114. O ctobe r
C opyri ~ht © 1975 by The Williams & Wilk ins Co .
Printed in U.S .A.
CALCIFICATIO N IN AN ANGIOMYOLIPOMA : A CAS E REPORT T O N Y M . DEET HS
AND
G. LELAN D M E LSO:--i
From th e M allinckrodt Ins t itut e of Radiolog\' , Washin!.!lon Un iuersitv S chool of M edicine, St. L ouis , M issouri
ABSTRACT
A patient with tuberous scleros is a nd an giomyoli po m a of both kidneys is described in whom both tumors demonstra t ed associa t ed calcification. Th is findin g has not been previously stressed in the literature and is important in that ignorance of its occurrence in this tumor might result in an error of diagnosis and nephrectomy in a pat ient who can ill- afford the loss of renal parenchy ma. Althou gh rena l a ngiom yolipom a (ha m a rtom a ) is a rel at ively ra re lesion wit h only a bout 200 cases reported, 1• 2 the pla in fi lm, urogra phic a nd arteriographic features have been extensively described. 3 - 5 However, specific preoperative diagnosis is possible only rarely since the a rteriographic ' atures oft his ]psi o n >1rP not 11s 11>1 ll v c' h >1 r:w1 Prist i" and diagnosis rests primarily on the pla in film demonstra tion of mottled , rela tivelv ra diolucent areas wit hin the tumor secondary to its high fa t content. Accurate di agnosis of th ese benign t umors is important t o prevent unnecessary nephrectomy. 1 • • This is especially crit ical in patients with tuberous sclerosis and multifocal tumors since the common complication of renal hemorrhage would be tragic if it occurred in a patient who had undergone nephrectom y. Calcifica tion within these lesions has not been stressed in the urologic literature a nd, if ignored , it may resul t in the erron eous diagnosis of rena l carcinoma . Therefore. we herein present a case. describing calcification of such a tumor.
lower pole a nd a 3 cm. mass in the left u p per pole. Both lesions we re noted to h ave peripheral eggs hell calcification wit h areas of mott led rad iolucency interna lly (see fi gure ). A retrogra d e pyelogra m showed mass effect on the cal ices in th e ri ght lower pole. Both les ions were a vascular at arteriogr:1phy
ln-tr:1cranial cu lcific utiun w a :.-.-; u u lcd
u 11
::,;kull
films . On the basis of t he aforem entioned fin d ings we believed tha t the renal lesions were most likely a n giom yoli pomas. since the patien t had tuherous sclerosis. a nd tha t thera py should be conservat ive. preserv ing as much renal tiss ue as possible . The patient was started on chronic dialysis with imp rovement of renal function . At :{-year fo llowup t he pa tient has continued t o do well wit h preserv a tion of so me rena l funct ion and no cha nge in the ren al tumors . DISC l 'SS ION
Angiomyolipoma occurs in :l distinct patterns : 1) asy mptom atic small inci dent a l les ions at a utopsy , 2) single la rge asy mptom at ic lesions in wo m en in the fifth a nd sixth decades a nd :1) multi ple les ions CASE REPORT 7 A 47-yea r-old black ma n was a dmitted to t he in pat ients with tuberous sclerosis . M ore t ha n ha lf of t he report ed cases of a ngio myoli poma occ urred hospita l for diagnosis a nd t reat ment of renal fa il in p atients wit h tu berous scleros is and u p t o 80 per ure . H e was in good healt h until 2 weeks before h tuberous sclerosis have hos pit alization when he ex perienced increased fa - cent of pat ients wit 8 tigue, weakness and drowsiness. Phys ical examina- angiomyolipomas . ' · The clinical s ignifica nce of this tumor lies in its high incidence in those tion revealed cha nges consist ent wit h con gestive pat ients with tuberous scleros is . it s m ult ifoca l heart failure. Adenoma sebaceum was not ed over t o present as hem atu ria or his nose a nd cheeks a nd d ark pigmented mac ulae occurrence. its t endency 0 9 were p resent on his chest. In addition, th ere was a ren a l hemorrhage, · ' its beni gn na t u re• and the y in d ifferenti ati n g it fro m hypernephroma difficult ·,;ague full ness a nd t enderness to palpation over t he or sarco ma.' T he rela ti vely rad iolucent fa tty areas ri gh t fl ank . Blood pressure was 160/ 100, blood urea nitrogen 190 m g. per di. , creatinine ~2 mg. per d i. of t he tu mor may b e the sol e d istinctive rad iointo the tumor is said t o and hemoglobin 7.0 gm. per di. F a mily history was gra phic finding. ' Bleeding a bolish t his sign. 5 · • . 9 M ost angiomyolipomas a re significant in that both of the pa tient's sons ha d had tuberous sclerosis. one dy ing of a brain tum or cl inically s ilent but h emorrhage into the m ass may occur a nd res ult in fever, fl a nk pa in, hem a turia or a t age 22 a nd the other of chronic rena l fa ilure a t ra pid enla rge ment of the mass. 3 Renal fa ilure has ;:ge 20. not been desc ribed as a prom inen t fi ndin g in t h ese An excret ory urogram demonst ra l ed no n-func pa tient s . 8 T he a ngiographic spectrum of these tion of t h e kidn eys with a n 11 c m. m ass in t he ri ght tumors has been we ll descri bed a nd includes avasAcc epted for publication May 16 , 197fi. cular radiolucent masses ( 4 per cent). neovascular6 1:3
t:
614
DE ETI-I S AND MEI.SON
A. a nt ero post e rior view o f ri g ht k id1w,· a nd H. ri gh t poste rior oblique view of le ft kidn ey with patient in s upin e pos iti o n demon s trat e peripheral c un·ilin ear calcific atio ns in renal masses (arrows). A m o rph ous areas of calcification may re present pe riphe ral ca lc ifi cation see n en face. Mottled ra diolu cen t areas represe ntin g fat a re not well a pprec iated on th ese film s.
ity indistinguis habl e from ma lign a nt tumors ((i8 per cent), characteristic pseudoa neurys ms of the a rteries with whorled vessels in th e veno us phase and lucent areas in the n ephrogra m ph ase from the fatty elements (16 per cent) and characteristic discret e neovasc ul a rity of the tumor su ppli ed by normal intralobar artery (12 per cent). 3 The import a nt feature of this case is the unus ua l presence of calcium in the les ion s . To our knowl edge the only report of ca lcifi ca tion in an angiom yo lipom a was in a ca se of a tumor of the rena l capsul e. H owever. the report does not include a radiogram nor a full description of the calcification. 11 In our case the calcificat ion in the left renal mass is peripheral and curvilinear (eggshell) while on the righ t it has some amorphous components but is also periph era l in locat ion. According to a recent st ud:,;. the incidence of malignancy in ren al ma sses with peripheral calcification is approximately 20 per cen t. 1 2 Thus. it is critical in a pat ient with calc ifi cat ion in a renal mass that the preopera tive diagnosis or ang iomyolipoma be considered sin ce distinction between this tumor and a malign a nt tumor ca n usu a lly not be ma de at the operat ing table. The diagn os is in t his case was ma de prim a ri!Y on th e bas is of this clini ca l history a nd th e presence of mottl ed lucen cy in the ren a l masses . Arteriographv was non-cont ribu tory . In view of the patient's anemia and th e propensity of these tumors to hemorrh age it is likely that th e ren a l ca lcifi cation s res ulted from peripheral hem a tnm a. Of addition:11 note in this case is the fact th at co rrelation of clinical a nd radiologic findin gs has resu lted 1t1 optimal m a nage ment oft he patient. 1. •
HEFERE NCES
L.. Scott. R. , ,Jr. and Seybold , H. M.: Renal a ngiomyo lipoma (hamartom a): rev iew of th e lit era ture a nd re port of 7 cases. J. Uro l., 105: :i2 .1m1 _ Rusc h . C.: Hena l h ama rtoma (a ngiom yolipoma ): report o f three cases .. J. Uro l. . 67: 82:\, 1%:2. C lark , R E. an d Paluhinskas , A. ,J. : The angiographic spect rum o frrn 11 l hamartom a. Amer. ,J. Roentgen., 114: 'il G. 197:2. Crosett. A. D.. ,Jr.: Hoe nt genogra phi c findin gs in the re n a l les ion of tuhero us sc leros is. Amer. ,J. Roentgen .. 98: ,:\9. 19o(i. Khilnani. M. T. and W o lf. B. S.: Hama rtolipoma of the kidn e\': clini cal and roentgen features. Amer. .J. Ro e nt gen .. 86: 8:\0. 19fi l. Tan. (;_ C ., England , E. ,J. and Low. A. I.: Angi omyolipoma: a rare a nd inte resting t um our of the kidn ey . Au st. N. Zea l. J. S urg. , 42: 75. 1972. E vans ..). A. and Hosniak, M. A.: The Kidney. C hi cago: Year Hook Medical Publishers, Inc. , 1971. Go lji . H .: TuhProus sc leros is a nd renal neo pl asm. ,J. U rol.. 8!i: 919. 19GI Allen, T. D. a nd Ris k. W.: Renal angiomyolipoma. J. Uro l.. 94: 20:\. 19fi", Keshin . ,J. G.: Three cases of renal h ama rt o n\ a : two cases prese ntin g w ith s po nt a n eous rupture an d nrnssi\' e ret roperiton e al hemorrh:-1ge. ,J. Urol. . 94: :t\(i. l!lfi:'i. Seahury. ,J. C., ,Jr .. Ensor. R. D. a nd Wolfe, W . G.: /\n giomvo lipom a of th e kidney: a benign tumor demon s tratin g n eo-vasnd a riz a t ion h~· arteriog raphy . Report of :2 cases. ,J. Urol., 98: 562, 1967. Danie l. W . W .. Hartman , G. W. , Witt en , D. M. , F a rrow . C. M. a nd K ela lis. P. P.: Calcified ren a l
1. M cCu llough. D.
2. :i.
4.
5.
6.
7.
8.
9. 10.
11.
1:2.
rn
Vol. 114. O ctobe r
C opyri ~ht © 1975 by The Williams & Wilk ins Co .
Printed in U.S .A.
CALCIFICATIO N IN AN ANGIOMYOLIPOMA : A CAS E REPORT T O N Y M . DEET HS
AND
G. LELAN D M E LSO:--i
From th e M allinckrodt Ins t itut e of Radiolog\' , Washin!.!lon Un iuersitv S chool of M edicine, St. L ouis , M issouri
ABSTRACT
A patient with tuberous scleros is a nd an giomyoli po m a of both kidneys is described in whom both tumors demonstra t ed associa t ed calcification. Th is findin g has not been previously stressed in the literature and is important in that ignorance of its occurrence in this tumor might result in an error of diagnosis and nephrectomy in a pat ient who can ill- afford the loss of renal parenchy ma. Althou gh rena l a ngiom yolipom a (ha m a rtom a ) is a rel at ively ra re lesion wit h only a bout 200 cases reported, 1• 2 the pla in fi lm, urogra phic a nd arteriographic features have been extensively described. 3 - 5 However, specific preoperative diagnosis is possible only rarely since the a rteriographic ' atures oft his ]psi o n >1rP not 11s 11>1 ll v c' h >1 r:w1 Prist i" and diagnosis rests primarily on the pla in film demonstra tion of mottled , rela tivelv ra diolucent areas wit hin the tumor secondary to its high fa t content. Accurate di agnosis of th ese benign t umors is important t o prevent unnecessary nephrectomy. 1 • • This is especially crit ical in patients with tuberous sclerosis and multifocal tumors since the common complication of renal hemorrhage would be tragic if it occurred in a patient who had undergone nephrectom y. Calcifica tion within these lesions has not been stressed in the urologic literature a nd, if ignored , it may resul t in the erron eous diagnosis of rena l carcinoma . Therefore. we herein present a case. describing calcification of such a tumor.
lower pole a nd a 3 cm. mass in the left u p per pole. Both lesions we re noted to h ave peripheral eggs hell calcification wit h areas of mott led rad iolucency interna lly (see fi gure ). A retrogra d e pyelogra m showed mass effect on the cal ices in th e ri ght lower pole. Both les ions were a vascular at arteriogr:1phy
ln-tr:1cranial cu lcific utiun w a :.-.-; u u lcd
u 11
::,;kull
films . On the basis of t he aforem entioned fin d ings we believed tha t the renal lesions were most likely a n giom yoli pomas. since the patien t had tuherous sclerosis. a nd tha t thera py should be conservat ive. preserv ing as much renal tiss ue as possible . The patient was started on chronic dialysis with imp rovement of renal function . At :{-year fo llowup t he pa tient has continued t o do well wit h preserv a tion of so me rena l funct ion and no cha nge in the ren al tumors . DISC l 'SS ION
Angiomyolipoma occurs in :l distinct patterns : 1) asy mptom atic small inci dent a l les ions at a utopsy , 2) single la rge asy mptom at ic lesions in wo m en in the fifth a nd sixth decades a nd :1) multi ple les ions CASE REPORT 7 A 47-yea r-old black ma n was a dmitted to t he in pat ients with tuberous sclerosis . M ore t ha n ha lf of t he report ed cases of a ngio myoli poma occ urred hospita l for diagnosis a nd t reat ment of renal fa il in p atients wit h tu berous scleros is and u p t o 80 per ure . H e was in good healt h until 2 weeks before h tuberous sclerosis have hos pit alization when he ex perienced increased fa - cent of pat ients wit 8 tigue, weakness and drowsiness. Phys ical examina- angiomyolipomas . ' · The clinical s ignifica nce of this tumor lies in its high incidence in those tion revealed cha nges consist ent wit h con gestive pat ients with tuberous scleros is . it s m ult ifoca l heart failure. Adenoma sebaceum was not ed over t o present as hem atu ria or his nose a nd cheeks a nd d ark pigmented mac ulae occurrence. its t endency 0 9 were p resent on his chest. In addition, th ere was a ren a l hemorrhage, · ' its beni gn na t u re• and the y in d ifferenti ati n g it fro m hypernephroma difficult ·,;ague full ness a nd t enderness to palpation over t he or sarco ma.' T he rela ti vely rad iolucent fa tty areas ri gh t fl ank . Blood pressure was 160/ 100, blood urea nitrogen 190 m g. per di. , creatinine ~2 mg. per d i. of t he tu mor may b e the sol e d istinctive rad iointo the tumor is said t o and hemoglobin 7.0 gm. per di. F a mily history was gra phic finding. ' Bleeding a bolish t his sign. 5 · • . 9 M ost angiomyolipomas a re significant in that both of the pa tient's sons ha d had tuberous sclerosis. one dy ing of a brain tum or cl inically s ilent but h emorrhage into the m ass may occur a nd res ult in fever, fl a nk pa in, hem a turia or a t age 22 a nd the other of chronic rena l fa ilure a t ra pid enla rge ment of the mass. 3 Renal fa ilure has ;:ge 20. not been desc ribed as a prom inen t fi ndin g in t h ese An excret ory urogram demonst ra l ed no n-func pa tient s . 8 T he a ngiographic spectrum of these tion of t h e kidn eys with a n 11 c m. m ass in t he ri ght tumors has been we ll descri bed a nd includes avasAcc epted for publication May 16 , 197fi. cular radiolucent masses ( 4 per cent). neovascular6 1:3
t:
614
DE ETI-I S AND MEI.SON
A. a nt ero post e rior view o f ri g ht k id1w,· a nd H. ri gh t poste rior oblique view of le ft kidn ey with patient in s upin e pos iti o n demon s trat e peripheral c un·ilin ear calcific atio ns in renal masses (arrows). A m o rph ous areas of calcification may re present pe riphe ral ca lc ifi cation see n en face. Mottled ra diolu cen t areas represe ntin g fat a re not well a pprec iated on th ese film s.
ity indistinguis habl e from ma lign a nt tumors ((i8 per cent), characteristic pseudoa neurys ms of the a rteries with whorled vessels in th e veno us phase and lucent areas in the n ephrogra m ph ase from the fatty elements (16 per cent) and characteristic discret e neovasc ul a rity of the tumor su ppli ed by normal intralobar artery (12 per cent). 3 The import a nt feature of this case is the unus ua l presence of calcium in the les ion s . To our knowl edge the only report of ca lcifi ca tion in an angiom yo lipom a was in a ca se of a tumor of the rena l capsul e. H owever. the report does not include a radiogram nor a full description of the calcification. 11 In our case the calcificat ion in the left renal mass is peripheral and curvilinear (eggshell) while on the righ t it has some amorphous components but is also periph era l in locat ion. According to a recent st ud:,;. the incidence of malignancy in ren al ma sses with peripheral calcification is approximately 20 per cen t. 1 2 Thus. it is critical in a pat ient with calc ifi cat ion in a renal mass that the preopera tive diagnosis or ang iomyolipoma be considered sin ce distinction between this tumor and a malign a nt tumor ca n usu a lly not be ma de at the operat ing table. The diagn os is in t his case was ma de prim a ri!Y on th e bas is of this clini ca l history a nd th e presence of mottl ed lucen cy in the ren a l masses . Arteriographv was non-cont ribu tory . In view of the patient's anemia and th e propensity of these tumors to hemorrh age it is likely that th e ren a l ca lcifi cation s res ulted from peripheral hem a tnm a. Of addition:11 note in this case is the fact th at co rrelation of clinical a nd radiologic findin gs has resu lted 1t1 optimal m a nage ment oft he patient. 1. •
HEFERE NCES
L.. Scott. R. , ,Jr. and Seybold , H. M.: Renal a ngiomyo lipoma (hamartom a): rev iew of th e lit era ture a nd re port of 7 cases. J. Uro l., 105: :i2 .1m1 _ Rusc h . C.: Hena l h ama rtoma (a ngiom yolipoma ): report o f three cases .. J. Uro l. . 67: 82:\, 1%:2. C lark , R E. an d Paluhinskas , A. ,J. : The angiographic spect rum o frrn 11 l hamartom a. Amer. ,J. Roentgen., 114: 'il G. 197:2. Crosett. A. D.. ,Jr.: Hoe nt genogra phi c findin gs in the re n a l les ion of tuhero us sc leros is. Amer. ,J. Roentgen .. 98: ,:\9. 19o(i. Khilnani. M. T. and W o lf. B. S.: Hama rtolipoma of the kidn e\': clini cal and roentgen features. Amer. .J. Ro e nt gen .. 86: 8:\0. 19fi l. Tan. (;_ C ., England , E. ,J. and Low. A. I.: Angi omyolipoma: a rare a nd inte resting t um our of the kidn ey . Au st. N. Zea l. J. S urg. , 42: 75. 1972. E vans ..). A. and Hosniak, M. A.: The Kidney. C hi cago: Year Hook Medical Publishers, Inc. , 1971. Go lji . H .: TuhProus sc leros is a nd renal neo pl asm. ,J. U rol.. 8!i: 919. 19GI Allen, T. D. a nd Ris k. W.: Renal angiomyolipoma. J. Uro l.. 94: 20:\. 19fi", Keshin . ,J. G.: Three cases of renal h ama rt o n\ a : two cases prese ntin g w ith s po nt a n eous rupture an d nrnssi\' e ret roperiton e al hemorrh:-1ge. ,J. Urol. . 94: :t\(i. l!lfi:'i. Seahury. ,J. C., ,Jr .. Ensor. R. D. a nd Wolfe, W . G.: /\n giomvo lipom a of th e kidney: a benign tumor demon s tratin g n eo-vasnd a riz a t ion h~· arteriog raphy . Report of :2 cases. ,J. Urol., 98: 562, 1967. Danie l. W . W .. Hartman , G. W. , Witt en , D. M. , F a rrow . C. M. a nd K ela lis. P. P.: Calcified ren a l
1. M cCu llough. D.
2. :i.
4.
5.
6.
7.
8.
9. 10.
11.
1:2.
rn
