FROM THE DEPARTMENTS OF DIAGNOSTIC RADIOLOGY (DIRECTOR: PROF. M. C. BEACHLEY), RADIATION THERAPY (DIRECTOR: PROF. T . A. HAZRA), A N D UROLOGY (DIRECTOR: PROF. W.

W . KOONTZ J R ) , MEDICAL COLLEGE O F VIRGINIA HOSPITAL, RICHMOND, VIRGINIA 23298, U.S.A.

MALIGNANT THYMOMA WITH RENAL METASTASES Report of a case S.-O.

HIETALA, T. A. H A Z R A and J . H . TEXTER J R

C a s e s of h i s t o l o g i c a l l y c o n f i r m e d m a l i g n a n t t h y m o m a m e t a s t a s i z i n g o u t s i d e t h e t h o r a x a r e r a r e . T h e first c a s e of m a l i g n a n t t h y m o m a m e t a s t a s i z i n g t o t h e k i d ­ n e y s , l y m p h n o d e s , liver, s p l e e n , c r a n i a l n e r v e s , a n d eyes w a s r e p o r t e d b y M E I G S & DE S C H W E I N I T Z ( 1 8 9 4 ) . Since then, 3 1 a d d i t i o n a l cases w i t h e x t r a t h o r a c i c

spread

of m a l i g n a n t t h y m o m a h a v e b e e n r e p o r t e d , ( L E I M A N N & S M I T H 1 9 2 6 , D A N I S C H & NEDELMANN

1 9 2 8 ,BEDFORD

1930,O'GARA

et coll.

1 9 5 8 ,ERICSON &

HOOK 1960,

B E R N A T Z et coll. 1 9 6 1 , H A S N E R & W E S T E N G A A R D 1 9 6 3 , M O T T E T 1 9 6 4 , R A C H M A N I N O F F &

F E N T R E S S 1 9 6 4 , R O S E N et coll. 1 9 6 6 , F R I E D M A N 1 9 6 7 , G R A V A N I S 1 9 6 8 ) . T h e r e s e e m s

t o b e n o p r e v i o u s r e p o r t s of a n g i o g r a p h i c f i n d i n g s i n m e t a s t a s i z i n g t h y m o m a . T h e p u r p o s e of t h i s c o m m u n i c a t i o n is t o r e p o r t a c a s e i n o r d e r t o f u r t h e r e m p h a s i z e t h e u n p r e d i c t a b l e clinical b e h a v i o r of t h y m o m a s a n d a l s o t o d e s c r i b e t h e r a d i o g r a p h i c a n d especially t h e a n g i o g r a p h i c f i n d i n g s i n a c a s e of t h y m o m a m e t a s t a s i z i n g t o t h e kidney. C a s e report T h e patient was a 25-year-old white male with a history of a mediastinal m a s s diagnosed a t r o u t i n e chest r a d i o g r a p h y a b o u t 16 m o n t h s before the present admission. T h e preliminary diagnosis w a s l y m p h o m a , b u t further evaluation with a n exploratory t h o r a c o t o m y revealed Submitted for publication 29 March 1977. Acta Radiologica Diagnosis 19 (1978) Fasc. 2 22-785833

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malignant t h y m o m a . T h e t h y m u s could not be completely excised because it was partly a d h e r e n t t o the great vessels. T h e patient received, postoperatively, radiation therapy to t h e mediastinum and to the left infraclavicular region. A b o u t 9 m o n t h s after surgery, the patient was admitted again t o the Urological Service because of painless gross h e m a t u r i a . Pertinent physical findings revealed n o cervical or supracalvicular a d e n o p a t h y . T h e a b d o m e n was soft and the liver a n d spleen were not pal­ pable. L a b o r a t o r y d a t a showed hemoglobin 14.5 g % , W B C 8 900, B U N 15 a n d creatinin 1.0 g ° „ . U l t r a s o n o g r a p h y was performed and d e m o n s t r a t e d a predominantly solid mass in the lower a n d anterior part of the right kidney. Radiographic findings. U r o g r a p h y d e m o n s t r a t e d a delayed excretion of contrast m e d i u m o n the right side a n d a lower pole renal mass. A retroperitoneal mass displaced the kidney anteriorly. Cystoscopy and right retrograde pyelography (Fig. 1) were performed a n d re­ vealed multiple intrinsic filling defects with the a p p e a r a n c e of blood clots in the right ureteropelvic system a n d a superior displacement of the lower pole calyces. Multiple n o d u l a r in­ filtrations consistent with metastases were present o n chest films as well as an elevation of the left h e m i d i a p h r a g m suggesting s o m e degree of phrenic nerve paralysis. A b d o m i n a l a o r t o g r a p h y d e m o n s t r a t e d n o changes in the a b d o m i n a l a o r t a or the major vessels. T h e right kidney was supplied by 2 arteries and the left by a single artery (Fig. 2 a). T h e mass in the lower pole of the right kidney was avascular (Fig. 2 b). T h e intrarenal arterial branches were displaced a n d stretched. T h e selective angiography did not reveal any ad­ ditional findings c o m p a r e d to the non-selective angiography. T h e patient was treated with c h e m o t h e r a p y (Cytoxan. Prednisone, Oncovin). During this treatment the patient had several episodes of h e m a t u r i a . Repeated intravenous urography

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339

Fig. 2. a) Selective nephroangiography of the main renal artery. No hypervascularity. A dilated capsular artery, b) Late parenchymal phase. The lower pole of the right kidney is distorted and en­ larged by an avascular mass.

demonstrated that the condition was essentially u n c h a n g e d since previous e x a m i n a t i o n s . T h e patient was, therefore, operated u p o n . Operative findings. T h e kidney contained a huge, hard m a s s invading the r e t r o p e r i t o n e u m but did not a p p e a r to be attached t o the psoas muscle. T h e lower pole was entirely replaced by a hard, greyish-beige mass. Similar lesions of small size were found in the upper pole a n d t h r o u g h o u t the substance of the kidney. Several large nodes were identified at the level of the renal hilum. T h e liver a p p e a r e d t o be u n r e m a r k a b l e . Para-aortic nodes were palpated on the left side. O n palpation of the left kidney, s o m e nodularity, consistent with t u m o r extension in the left kidney, was discovered. Histology. Macroscopic examination d e m o n s t r a t e d multiple, white, a n d fishy-flesh like r o u n d nodules t h r o u g h o u t the entire kidney extending into the pelvis of the kidney. T h e largest of the masses measured a b o u t 6 c m x 5.8 c m x 6 c m ; the smallest was a p p r o x i m a t e l y 1 cm in diameter. O n microscopic e x a m i n a t i o n (Fig. 3), the t u m o r nodules d e m o n s t r a t e d p r e d o m i n a n t l y epithelial c o m p o n e n t s with scattered r o u n d cells. T h e cells in the majority of the t u m o r were a r r a n g e d in a spindle pattern. T h e r e were occasional mitosis and nucleoli. T h e t u m o r nodules h a d replaced much of the renal p a r e n c h y m a sparing the glomeruli. T h e t u m o r was involving the renal pelvis and had involved the perirenal fat but the major renal vessels were n o t invaded. Microscopically the appearance of the t u m o r was consistent with a spindle epi­ t h e l i o m a type of metastatic t h y m o m a .

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S.-O. H1ETALA, T . A . H A Z R A A N D J . H . TEXTER J R

Fig. 3. Microscopy. Predomi­ nantly epithelial thymoma with spindle cells and scattered lym­ phocytes, x 222. Follow-up. A year a n d a half after t h e t h o r a c o t o m y a n d resection of the t h y m o m a , t h e patient developed nausea a n d vomiting a n d also headache. Physical examination d e m o n ­ strated bilateral papilledema b u t w a s otherwise u n r e m a r k a b l e . Pertinent laboratory d a t a revealed n o r m a l E M I scan, isotope brain scan a n d E E C L u m b a r puncture was performed and cytology from the spinal fluid proved t o b e positive. T h e patient was treated with radia­ tion therapy for cerebral metastasis from previously diagnosed malignant t h y m o m a .

Discussion T h e r e a s o n f o r t h e t h o r a c i c l i m i t a t i o n i n c a s e s of m a l i g n a n t t h y m o m a is n o t a p ­ p a r e n t s i n c e d i s c o n t i n u o u s m e t a s t a s e s i n t h e p l e u r a h a v e b e e n o b s e r v e d fairly fre­ q u e n t l y a n d t h e p r e d o m i n a n t i n t r a t h o r a c i c s p r e a d of t h y m o m a s h a s b e e n e m p h a s i z e d previously (CASTLEMAN 1 9 6 2 ) . Occasional cases have been reported where there have b e e n t r u e d i s t a n t m e t a s t a s e s t o t h e liver ( B E R N A T Z e t coll., L E I M A N N & S M I T H , H A S N E R &

WESTENGAARD, O ' G A R A

et coll., M O R G A N & D U D L E Y 1 9 5 5 , LATTES 1 9 6 2 ) , k i d n e y

(ERICSON & H O O K , M E I G S & DE SCHWEINITZ), and brain and meninges (HASNER

&

W E S T E N G A A R D , R A C H M A N I N O F F & FENTRESS). It h a s been discussed as t o whether t h e liver m e t a s t a s e s a r e t h e r e s u l t of h e m a t o g e n i c d i s s e m i n a t i o n o r if t h e y r e p r e s e n t t u m o r spread through implantation. Cases have been described ( K A T Z 1 9 5 3 ) where t h e t u m o r g r e w t h r o u g h t h e d i a p h r a g m t o t h e p e r i t o n e a l c a v i t y . H o w e v e r , several r e p o r t e d c a s e s , a s well a s t h e p r e s e n t c a s e , fulfill t h e c r i t e r i a f o r m e t a s t a s i z i n g t h y ­ moma. T h e a g e s a t t h e t i m e of m i c r o s c o p i c d i a g n o s i s h a v e r a n g e d in p r e v i o u s p u b l i c a t i o n s ( L E G G & BRADY 1 9 5 5 , CHATTEN & K A T Z 1 9 7 6 , SALYER & EGGLESTON 1 9 7 6 ) from 1 0

y e a r s t o 7 6 y e a r s w i t h a m e a n a g e of a b o u t 5 0 y e a r s . T h y m o m a s a r e r a t h e r u n u s u a l b e l o w 2 5 y e a r s of a g e a l t h o u g h 2 c a s e s of t h y m o m a in c h i l d r e n h a v e b e e n d e s c r i b e d . T h e o c c u r r e n c e a m o n g sexes is a b o u t e q u a l . T h e p r e s e n t i n g s y m p t o m s i n m o s t c a s e s of t h y m o m a h a v e b e e n m y a s t h e n i a g r a v i s , t h o r a c i c p a i n , a n d v a r i o u s s i g n s of r e s p i r a t o r y d i s t r e s s . T h e i n c i d e n c e of m y a s t h e n i a g r a v i s is 2 5 p e r c e n t o r m o r e . M a n y t u m o r s a r e a s y m p t o m a t i c a s in t h e p r e s e n t c a s e a n d a r e d i s c o v e r e d o n r o u t i n e c h e s t films. F u r t h e r d i a g n o s t i c w o r k - u p h a s i n v o l v e d m o r e extensive radiologic m e t h o d s only t o a limited extent. T h e r e a r e n o previous

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M A L I G N A N T T H Y M O M A W I T H R E N A L METASTASES

r e p o r t s of a n g i o g r a p h y in c a s e s of m a l i g n a n t t h y m o m a w i t h m e t a s t a s e s t o t h e k i d n e y . It a p p e a r s t h a t t h e r a d i o g r a p h i c findings are nonspecific, d e m o n s t r a t i n g a m a s s lesion in t h e k i d n e y . T h e a n g i o g r a p h i c f i n d i n g s i n t h i s c a s e w e r e t h o s e of a n

avascular

t u m o r infiltrating the kidney p a r e n c h y m a . Similar findings o n conventional

films

a n d a t a n g i o g r a p h y a r e o b s e r v e d in c a s e s of m a l i g n a n t l y m p h o m a i n v o l v i n g t h e k i d n e y s . T h e r a d i o l o g i c m e t h o d s , i n c l u d i n g a n g i o g r a p h y , i n d i c a t e t h e p r e s e n c e of t u m o r in t h e k i d n e y b u t d o n o t a l l o w a specific d i a g n o s i s . T h e t h y m o m a s a r e u s u a l l y c o m p o s e d of t w o cell t y p e s ; l y m p h o c y t e s a n d s o - c a l l e d e p i t h e l i a l cells. O n e of t h e s e cell t y p e s m a y d o m i n a t e o r t h e r e m a y b e a m i x e d t y p e of t h y m o m a c o n s i s t i n g of a p p r o x i m a t e l y e q u a l n u m b e r s of t h e t w o cell t y p e s diffusely i n t e r m i x e d . I n s o m e e p i t h e l i a l t u m o r s , as in t h e p r e s e n t c a s e , t h e s p i n d l e cells c o n s t i ­ t u t e t h e v a s t m a j o r i t y of t h e t u m o r . It h a s b e e n s h o w n p r e v i o u s l y t h a t i n v a s i v e g r o w t h of t h e t h y m o m a s c a n n o t b e p r e d i c t e d b y t h e m i c r o s c o p i c a p p e a r a n c e . I n v a s i o n o r i m p l a n t s m a y t h u s o c c u r w i t h all t y p e s of t h y m o m a s . I t h a s a l s o b e e n s h o w n t h a t t h e r e is n o a p p a r e n t r e l a t i o n of t h e h i s t o l o g i c t y p e e i t h e r t o t h e a s s o c i a t e d s y n d r o m e s o r t o t h e p r o g n o s i s . T h e single m o s t i m p o r t a n t f a c t o r d e t e r m i n i n g t h e p r o g n o s i s of a p a t i e n t w i t h t h y m o m a is t h e g r o s s b e h a v i o r a n d t h e e x t e n t of t h e t u m o r . A 5-year s u r v i v a l in a p r e v i o u s series ( B E R N A T Z e t coll.) w a s 8 0 p e r c e n t f o r t h e p a t i e n t s w i t h n o n - i n v a s i v e t u m o r s a n d o n l y 23 p e r c e n t f o r t h o s e w i t h i n v a s i v e l e s i o n s . T h e b e n e f i t of c o m p l e t e s u r g i c a l e x c i s i o n w h e n e v e r p o s s i b l e , e v e n i n t h e p r e s e n c e of o b v i o u s l y invasive t u m o r s , has been emphasized. R a d i a t i o n t h e r a p y following biopsy alone h a s b e e n r e p o r t e d a s b e i n g successful in o c c a s i o n a l c a s e s . I t h a s a d e f i n i t e r o l e i n t h e m a n a g e m e n t of p a t i e n t s w i t h t u m o r s w h i c h c a n n o t b e c o m p l e t e l y r e s e c t e d .

S U M M A R Y A patient with metastasizing t h y m o m a is presented. This case, as well as 31 previously reported cases in the literature, d e m o n s t r a t e that t h y m o m a m a y give distant metastases a n d that the behavior of t h e t u m o r s c a n n o t be predicted o n histologic g r o u n d s . T h e case r e p o r t e d here is the first case where a n g i o g r a p h y was performed.

Z U S A M M E N F A S S U N G Ein Fall mit einem metastasierenden T h y m o m wird beschrieben. Dieser Fall, sowie 31 in der Literatur berichtete Falle, zeigen, dass T h y m o m e F e r n m e t a s t a s e n verursachen k o n n e n und dass das Verhalten des T u m o r s nicht auf histologischer Basis vorhergesagt werden k a n n . D e r beschriebene Fall ist der erste bei d e m A n g i o g r a p h i e v o r g e n o m m e n w u r d e .

R E S U M E Presentation d'un cas de t h y m o m e d o n n a n t deja publies dans la litterature, m o n t r e n t q u e distance et que le comportement des t u m e u r s histologiques. Le cas presente ici est le premier

des metastases. C e cas, ainsi q u e les 31 cas le t h y m o m e peut d o n n e r des metastases a ne peut pas etre prevu sur des a r g u m e n t s cas o u u n e angiographie a ete effectuee.

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Malignant thymoma with renal metastases. Report of a case.

FROM THE DEPARTMENTS OF DIAGNOSTIC RADIOLOGY (DIRECTOR: PROF. M. C. BEACHLEY), RADIATION THERAPY (DIRECTOR: PROF. T . A. HAZRA), A N D UROLOGY (DIRECT...
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