Pediatr Radiol (1992) 22:231-232

Pediatric Radiology 9 Springer-Verlag 1992

Pedunculated focal nodular hyperplasia S. S a w h n e y 1, R. Jain I, R. Safaya 2, and M. Berry 1 1Department of Radiodiagnosis 2 Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110029, India Received: 12 August 1991; accepted: 12 October 1991

Case report

Discussion

A 12-year-old female was admitted to the Cardiothoracic Surgery Unit for surgical correction of tetrology of Fallot. On routine preoperative examination a large, firm, freely mobile intra-abdominal mass was detected, of which the patient was unaware, An abdominal US revealed a large, welldefined, lobulated mass of heterogenous echogenicity, with central hypoechoic branching linear areas and a small speck of calcification. The mass was continuous with the inferior edge of medial segment of left lobe of liver through a narrow pedicle, with no surrounding liver parenchyma. The pedicle contained dilated vascular channels (Fig. 1). Color Doppler examination revealed these vascular channels to be a branch of left hepatic artery, and a vein draining directly into the left hepatic vein. These vessels could be traced into the centre of the mass, from where branches radiating to the periphery of the mass could be seen. Rest of the hepatic parenchyma and other intra-abdominal organs were normal. Contrast enhanced CT scan of the abdomen revealed a large well-defined, lobulated, enhancing mass with centralhypodense branching areas containing enhancing blood vessels and calcification (Fig. 2). Communication between the inferior edge of the left lobe of liver and mass through a pedicle containing dilated tortuous vascular channels could be made out. On the basis of these radiological findings a diagnosis of pedunculated liver tumor was made. Further, characteristic features, viz; a central branching area (scar) containing blood vessels and calcification, helped us make a diagnosis ofpedunculated focal nodular hyperplasia (FNH). All radiological findings were confirmed at surgery - the pedicle was ligated and the mass excised in-toto. Histopathology was confirmatory for FNH. Interestingly, after excision of the mass, an appreciable decrease was noted in the degree of the patient's cyanosis.

F N H is classically described in m i d d l e aged females a n d is usually a s y m p t o matic. T h e mass is usually small (average 4 c m d i a m e t e r ; m a x i m u m r e p o r t e d size 20 cm). A c e n t r a l scar c o n t a i n i n g fibrous tissue, vascular c h a n n e l s a n d calcification is often p r e s e n t (60 % ) a n d is pat h o g n o m o n i c [1]. S o n o g r a p h y is sensitive for d e t e c t i o n of F N H , b u t the e c h o - p a t t e r n of these tumors is variable a n d non-specific - thus differentiation with o t h e r b e n i g n a n d m a l i g n a n t liver t u m o r s is n o t possible on the basis of U S alone [1]. O u r e x p e r i e n c e suggests that a c o m b i n a t i o n of C o l o r D o p p l e r a n d 2D U S i m a g i n g can help m a k e a specific diagnosis by d e m o n s t r a t ing the characteristic p a t t e r n of vascular supply, with m a j o r f e e d e r b l o o d vessels a n d their b r a n c h e s c o n t a i n e d within the central h y p o e c h o i c scar. C T a p p e a r a n c e s of F N H are n o n - s p e cific with a v a r i a b l e a t t e n u a t i o n p a t t e r n o n b o t h n o n - c o n t r a s t a n d contrast enh a n c e d scans, t h o u g h a h y p o d e n s e lesion o n p r e c o n t r a s t scan which b e c o m e s

Fig. 1. Longitudinal right paramedian scan. Pedicle is seen containing dilated blood vessel (Cursor in vessel draining into left hepatic vein) and connecting left lobe of liver (arrows) to mass (not seen in scan) Fig.2. Enhanced CT scan at L3 vertebral level. Homogeneous well defined encapsulated mass with central branching hypodense scar. Blood vessels (arrow)seen within scar Fig.3. Cut surface of excised mass. The mass is divided by broad fibrous trabecular strands into lobules of varying sizes. The fibrous septae are converging towards a central area of fibrosis which contains large blood vessels

232 h y p e r d e n s e with I V contrast is typical

[11. W e h a v e n o t c o m e across any p r e v i o u s r e f e r e n c e of a radiologically docum e n t e d case of p e d u n c u l a t e d F N H , t h o u g h in a large series of p e d i a t r i c patients, t h e r e was a m e n t i o n of t w o patients with p e d u n c u l a t e d F N H [2]. Pedunculated hepatocellular carcinomas h a v e b e e n r e p o r t e d in 0.3 % to 2.4 % of all patients with h e p a t o c e l l u l a r carcinom a [3]. M a n y e x p l a n a t i o n s h a v e b e e n p o s t u l a t e d for the p e d u n c u l a t e d appeara n c e of h e p a t i c masses, e. g., masses arising in c o n g e n i t a l l y displaced h e p a t i c lobules in Glisson's capsule, e c t o p i c liver tissue, accessory lobes, etc. [3]. Since F N H usually arises superficially, just u n d e r t h e capsule of liver and f r o m

the i n f e r i o r edge, it is not surprising that a p e d u n c u l a t e d mass can result, t h o u g h it is rare. Also, an association with a c o n g e n i t a l cardiac a n o m a l y ( t e t r o l o g y of F a l l o t in this p a t i e n t ) has n o t b e e n r e p o r t e d previously, t h o u g h a coincidental co-exist e n c e is possible. T h e d e c r e a s e in cyanosis following excision of t h e mass m a y b e e x p l a i n e d on t h e basis of a h y p e r v a s c u l a r t u m o r with a large a m o u n t of o x y g e n extraction.

2. Stocker JT, Ishak K G (1981) Focal nodular hyperplasia of the liver. A study of 21 pediatric cases. Cancer 48:336-345 3. Horie Y, Kotoh S, Yoshida H, Imaoka T, Suom T, Hirayama C (1983) Pednnculated hepatocellular carcinoma. Report of three cases and review of literature. Cancer 51: 746-751

References

Dr. S. Sawhney Department of Radiodiagnosis All India Institute of Medical Sciences Ansari Nagar New Delhi - 110029 India

1. Low V, Khangure MS (1990) Hepatic adenoma and focal nodular hyperplasia: a diagnostic dilemma. Australas Radiol 34: 124-130

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Continued from p. 228

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Pedunculated focal nodular hyperplasia.

Pediatr Radiol (1992) 22:231-232 Pediatric Radiology 9 Springer-Verlag 1992 Pedunculated focal nodular hyperplasia S. S a w h n e y 1, R. Jain I, R...
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