ClinicalRadiology (1991) 43, 433-434

Case Report: Bilateral Massive Internal Jugular Vein Thrombosis in Carcinoma of the Thyroid: CT Evaluation S. T H O M A S , S. S A W H N E Y * a n d B. M. L. K A P U R

Departments of Surgery and *Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India Superior vena cava syndrome, an uncommon manifestation of carcinoma of the thyroid, is usually due to retrosternal extension of the growth. Rarely, however, there may be direct tumour extension into the great veins of the neck, which may cause sudden death. Only eight such cases have been reported in the English literature. The case reported by us is the first to be documented by computed tomography. Patients with carcinoma of the thyroid with the superior vena cava syndrome should undergo computed tomography to differentiate external compression due to goitre from intraluminal tumour. Thomas, S., Sawhney, S. & Kapur, B.M.L. (1991). Clinical Radiology 43, 433-434. Case Report: Bilateral Massive I n t e r n a l J u g u l a r Vein T h r o m b o s i s in C a r c i n o m a o f the T h y r o i d : C T E v a l u a t i o n

Follicular a n d anaplastic c a r c i n o m a s of the t h y r o i d are aggressive neoplasms. Characteristically they show microscopic evidence of vascular invasion, b u t massive a n g i o i n v a s i o n with t u m o u r extension into the great veins is extremely rare ( G r a h a m , 1924). O n l y eight well docum e n t e d instances o f this c o m p l i c a t i o n have been reported previously - in most of them the diagnosis was m a d e at autopsy. Here we report the first such case to be d o c u m e n t e d by c o m p u t e d t o m o g r a p h y (CT), review the literature a n d discuss the clinical features a n d m a n a g e m e n t o f this u n u s u a l condition.

CASE REPORT A sixty-year-old male presented with a history of episodes of 'dizziness' of 3 months duration and thyroid enlargement of one month duration associated with anorexia and weight loss. Examination revealed a euthyroid individual with a firm thyroid mass involving both lobes of the gland. The lower limit of the thyroid was well felt and there did not appear to be any retrosternal extension. However, there were dilated veins over the neck with flow of blood from above downwards. Routine haemogram and serum chemistry were within normal limits. A thyroid scan was not possible due to low radioactive iodine uptake (2.9% at 2 h and 1.2% at 24 h). Fine-needleaspiration cytology revealed poorly differentiated carcinoma. CT showed both lobes of the thyroid to be grossly enlarged, with several areas of low attenuation in both the lobes, suggestiveof tumour infiltration. There was evidence of extension of tumour into the paratracheal regions. Both internal jugular veins were distended with a central high attenuating tumour thrombus which had the same attenuation as the primary thyroid tumour (40 60 HU). The walls of the vein were enhancing a~ada hypodense rim was seen around the central thrombus probably representing a blood clot (Figs 1 and 2). Dilated superficial neck veins were also seen, suggestiveof collaterals (Fig. 1). The patient collapsed and died one day after CT before any operative intervention could be undertaken. A post-mortem examination was refused by the relatives.

DISCUSSION Follicular a n d anaplastic c a r c i n o m a s o f the thyroid behave aggressively a n d patients with u n t r e a t e d t u m o u r s m a y die f r o m local invasion, t u m o u r recurrence, or metastasis to lungs, bones or other organs (Tollefson et Correspondence to: Dr Shaji Thomas, 653 Laxmibai Nagar, New Delhi 110 023, India.

Fig. 1 - Contrast enhanced CT scan at the level of the larynx showing bilateral dilated internal jugular veins with central tumour thrombi (straight arrow) and superficial neck collaterals (curved arrow).

al., 1973). A less recognized cause of death from this n e o p l a s m is massive a n g i o i n v a s i o n of the cervical veins with direct extension into the great veins of the chest a n d to the heart. This c o m p l i c a t i o n was first reported by K a u f m a n n in 1879 a n d to date there have been only eight well d o c u m e n t e d instances o f this c o n d i t i o n ( G r a h a m , 1924; Holt, 1934; T h o m p s o n et al., 1978; Perez a n d Brown, 1984). A review o f the cases previously reported shows that all the eight patients h a d large palpable goitres a n d six o f them h a d clinical evidence o f superior v e n a cava syndrome. N o n e of t h e m h a d evidence of d i s t a n t metastasis. Six cases (all reported p r i o r to 1936) died within 2 m o n t h s o f the diagnosis o f c a r c i n o m a of the thyroid - the extent of the t h r o m b u s being d e t e r m i n e d only at autopsy. Radical surgery with r e m o v a l o f the i n t r a v a s c u l a r t h r o m bus was a t t e m p t e d in only two patients having follicular c a r c i n o m a , both o f w h o m did well after surgery ( T h o m p son et al., 1978; Perez a n d Brown, 1984). T h o m p s o n (1978) performed a t h y r o i d e c t o m y a n d a radical neck dissection a l o n g with r e m o v a l of the i n t r a v a s c u l a r t u m o u r - the p a t i e n t was alive 2 years after surgery. I n 1984, Perez u n d e r t o o k a near total t h y r o i d e c t o m y a n d

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Fig. 2 - Magnified view of the contrast enhanced CT scan showing enhancing tumour thrombus within the left internal jugular vein (arrow) with surrounding hypodense rim representing blood clot. t h r o m b e c t o m y in a n o t h e r p a t i e n t w h o also did well after surgery. I n a review o f 11 cases o f internal j u g u l a r vein t h r o m b o s e s d i a g n o s e d by C T ( Z e r h o u n i et al., 1980; A l b e r t y n a n d A l c o c k , 1987), the typical findings seen in all cases were a d i s t e n d e d vein with e n h a n c i n g walls, low a t t e n u a t i n g i n t r a l u m i n a l filling defects a n d a d j a c e n t soft tissue swelling. D i d i e r et al. (1987) have described the C T a p p e a r a n c e s o f inferior v e n a c a v a t u m o u r t h r o m b i arising f r o m a b d o m i n a l m a l i g n a n t n e o p l a s m s in 19 cases. T h e y showed e n h a n c i n g h e t e r o g e n e o u s i n t r a l u m i n a l t u m o u r t h r o m b i as well as low d e n s i t y t h r o m b i s u r r o u n d e d b y a r i m o f contrast. H o w e v e r , because o f the p r e d o m i n a n t l y h y p o d e n s e t h r o m b i seen, they c o u l d n o t confidently differentiate t u m o u r t h r o m b i f r o m b l o o d clots. T h e wall e n h a n c e m e n t is t h o u g h t to be due to opacification b y the v a s a v a s o r u m ( F i s h m a n et al., 1984). M a n y i m p o r t a n t differences were n o t e d in o u r case. T h e t h r o m b u s in the internal j u g u l a r vein was; e n h a n c i n g a n d high a t t e n u a t i n g a n d h a d the same a t t e n u a t i o n as the p r i m a r y t h y r o i d t u m o u r (40-60 H U ) . The lumen o f the internal j u g u l a r vein was e n l a r g e d with e n h a n c e m e n t o f the walls o f the vein. Between the central t h r o m b u s a n d the internal j u g u l a r vein wall, a h y p o d e n s e r i m was seen p r o b a b l y representing a b l o o d clot. D i l a t e d superficial veins in the neck were seen due to bilaterally o b s t r u c t e d internal j u g u l a r veins. B o t h i n t e r n a l j u g u l a r veins were directly infiltrated by the t u m o u r a n d c o u l d n o t be m a d e o u t s e p a r a t e l y at the D 1 - D 3 levels. Thus, a l t h o u g h a prec o n t r a s t scan was n o t a v a i l a b l e with us, a d i a g n o s i s o f t u m o u r t h r o m b u s was m a d e b a s e d o n the fact t h a t the

central t h r o m b u s was o f a higher d e n s i t y t h a n the s u r r o u n d i n g b l o o d clot a n d its density was the s a m e as t h a t o f the p r i m a r y t u m o u r . T u m o u r t h r o m b u s is the i n t r a l u m i n a l extension o f c a n c e r cells a r o u n d which fibrin is d e p o s i t e d , atlowing further g r o w t h into the l u m e n o f the vessel ( G r a h a m , 1924; H o l t , 1934). T h e t u m o u r t h r o m b u s m a y extend a l o n g the veins a n d into the h e a r t c h a m b e r s h i n d e r i n g b l o o d flow a n d o b s t r u c t i n g c a r d i a c action. S u d d e n d y s p n o e a a n d generalized v e n o u s stasis in the p e r i p h e r a l c i r c u l a t i o n m a y occur a n d d e a t h m a y be sudden, similar to t h a t f r o m a massive p u l m o n a r y e m b o l i s m . I n p a t i e n t s with t h y r o i d c a r c i n o m a with the s u p e r i o r v e n a c a v a s y n d r o m e , an e n h a n c e d C T scan o r a v e n o g r a m is r e c o m m e n d e d to differentiate external c o m p r e s s i o n due to a s u b s t e r n a l goitre f r o m i n t r a l u m i n a l t u r n o u t as the cause. T h e presence o f massive i n t r a v a s c u l a r invasion s h o u l d n o t be a c o n t r a i n d i c a t i o n for resection to palliate i m p e n d i n g s u p e r i o r vena c a v a o b s t r u c t i o n . P r e - o p e r a t i v e p l a n n i n g for resection o f the g r e a t veins is extremely i m p o r t a n t . C a r d i o p u l m o n a r y b y p a s s s h o u l d be available if there is t u m o u r extension into the h e a r t a n d p r o s t h e t i c o r a u t o g e n o u s graft s h o u l d be available if resection o f the s u p e r i o r vena cava is necessary. A r e such extensive o p e r a t i v e p r o c e d u r e s justified in patients with such far a d v a n c e d t h y r o i d c a r c i n o m a s ? In patients with well differentiated t h y r o i d c a r c i n o m a s with no evidence o f d i s t a n t metastasis a n d with the risk o f d e a t h f r o m t u m o u r e m b o l i s m or o b s t r u c t i o n to the tricuspid valve, the b a l a n c e o f o p i n i o n s h o u l d f a v o u r surgery where the facilities a n d expertise are available. T h e residual t u m o u r , if any, can be t r e a t e d with r a d i o a c tive i o d i n e after surgery.

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