Clinical Radiology (1991) 43, 183-185

Biliary Cystadenoma and Cystadenocarcinoma: Some Unusual Features Y. K. CHEUNG, F. L. CHAN, L. L. Y. LEONG, R. J. COLLINS* and A. CHEUNG*

Department of Diagnostic Radiology and *Department of Pathology, Queen Mary Hospital, Hong Kong Biliary cystadenoma and cystadenocarcinoma are rare tumours which have a good prognosis after complete surgical removal. Correct pre-operative diagnosis depends on the imaging characteristics of the tumours. We have observed some unusual features including multifoeal turnouts with attachment to other abdominal organs, tumour presenting as cholangitic abscess, and distant metastasis after apparently complete resection by hemihepatectomy. Cheung,

Y.K., Chan, F.L., Leong, L.L.Y., Collins, R.J. & Cheung, A. (I 991). Clinical Radiology 43, 183 185. Biliary Cystadenoma and Cystadenocarcinoma: Some Unusual Features

Biliary cystadenomas and cystadenocarcinomas are rare tumours of biliary origin constituting fewer than 5% of the total intrahepatic cysts of bile duct origin (Ishak et al., 1977). They occur most commonly in middle-aged females. Clinical manifestations are non-specific; hence pre-operative diagnosis depends very much on imaging findings. Surgical resection often yields excellent results (Marsh et al., 1974). Their radiological features, particularly the appearances on computed tomography (CT) and ultrasound, have been described in previous reports (Stanley et al., 1983; Choi et al., 1989). In this article we report three pathologically proven examples of biliary cystadenoma or cystadenocarcinoma with unusual clinical or radiological features. CASE R E P O R T S Case 1. A 63-year-old w o m a n presenting with recent onset of jaundice had first noticed a painless epigastric mass 25 years previously. The mass had fluctuated in size but enlarged progressively, accompanied by episodic abdominal discomfort. Flattening of the abdomen often occurred after vomiting of yellowish fluid, sometimes accompanied by mucus in the stool. Shortly before admission she noticed further enlargement of the mass over 10 days with increasing abdominal pain. Jaundice and pruritis had been present for 5 days. Investigations revealed raised serum direct bilirubin and alkaline phosphatase levels. Plain radiographs showed a large soft tissue mass in the right upper abdomen, and abdominal ultrasonography revealed a large hepatic multilocular cystic mass with irregular septation and internal papillary projections. Mobile echogenic debris was observed (Fig. la), and there were multiple small peripheral cysts. CT demonstrated similar features with a small daughter cyst adjacent to a large multiloculated cystic mass. Small foci of calcification were noted in the septa. Following intravenous injection of contrast medium, the walls and septa enhanced (Fig. lb). Hepatic angiography revealed a huge avascular mass with stretching of the intrahepatic arterial branches and a thin rim of fine tortuous vessels in the papillary projections and in the wall, which demonstrated faint contrast accumulation in the parenchymal phase. At operation, a large encapsulated multiloculated cyst measuring 20 x 19 x 14 cm was found involving both lobes of the liver. A smaller cyst measuring 10 x 5 x 5 cm on the surface of the stomach was connected with the larger cyst by a 2 cm wide tissue bridge. Several small cysts measuring 2 3 cm in diameter were found on the liver surface separate from the largest cyst. Three litres of thick bile was aspirated from the main lesion before it was excised, leaving the smaller cysts behind. Histological examination confirmed that this was a biliary cystadenoma. Case 2. A 72-year-old male complained of distension and discomfort in the upper abdomen for more than a year. Abdominal ultrasound

Correspondence to: Dr Y. K. Cheung, Department of Diagnostic Radiology, Queen Mary Hospital, Pokfulam Road, Hong Kong.

(a)

(b) Fig. 1 - Case 1. (a) Ultrasonography shows an irregular cystic lesion with septation and papillary projections (white arrow). (b) CT scan after intravenous contrast administration shows enhancement of the wall and internal soft tissue elements. There are foci of calcification.

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CLINICAL RADIOLOGY

Fig. 3 - Case 3. CT scan shows hypodense mass in left lobe containing dilated bile ducts. There are no septa or papillary projections in the mass. (a) ary metastases with positive sputum cytology 1½years later; he was alive and receiving chemotherapy at the time of this report. Case 3. A 44-year-old man presented with left upper quadrant pain and fever for 1 week. Ultrasound examination showed a mixed echogenic mass occupying the left lateral segment, measuring 10 x 4.5 cm. It contained dilated bile ducts and cystic areas containing echogenic debris. No papillary projections or mural nodules were detected. CT showed a hypodense lesion with dilated bile ducts (Fig. 3). At laparotomy there was a 10 cm mass on the inferior surface of the left lateral segment, adherent to the anterior abdominal wall, containing dilated bile ducts and pus and communicating with the intrahepatic biliary system. Left lateral segmentectomy and choledochojejunostomy were performed. The patient recovered uneventfully. The mass was histologically proven to be a cystadenocarcinoma.

DISCUSSION

(b) Fig. 2 - Case 2. (a) Ultrasonography reveals septation and large papillary projection (asterisk). Mural nodules (arrow) and solid components are also seen. (b) CT scan shows a cystic mass with septa and soft tissue elements. Small foci of calcification are present.

revealed a large multilocular mass with septa and internal projections (Fig. 2a). Abdominal CT confirmed the presence of a cystic mass with septa and solid components. Foci of calcification were noted. The wall and septa demonstrated contrast enhancement (Fig. 2b). Hepatic angiography showed a large avascular intrahepatic mass causing stretching of the hepatic vessels. A thick rim of fine tortuous vessels was noted in the wall with contrast accumulation during the parenchymal phase Fine needle aspiration of the mass under ultrasonographic guidance yielded a few millilitres of clear yellowish mucoid fluid which on cytological examination contained cells suggestive of an adenocarcinoma. At operation an encapsulated multicystic tumour 17 x 10 x 8 cm found in the right lobe was removed by a right hemihepatectomy. Histological examination confirmed that this was a biliary cystadenocarcinoma. The patient recovered uneventfully but developed puImon-

B i l i a r y c y s t a d e n o m a s are s l o w - g r o w i n g t u m o u r s w i t h a t e n d e n c y to b e c o m e m a l i g n a n t ( M a r s h et al., 1974). T h e m a l i g n a n t c o u n t e r p a r t , biliary c y s t a d e n o c a r c i n o m a s h a v e a b e t t e r p r o g n o s i s t h a n h e p a t o c e l l u l a r c a r c i n o m a or b i l i a r y c h o l a n g i o c a r c i n o m a ( I s h a k et al., 1977). F o r b o t h biliary cystadenoma and cystadenocarcinoma, complete e x c i s i o n offers t h e best c h a n c e o f c u r e ( C a h i l l et al., 1982). W h i l e t h e t w o c o n d i t i o n s c a n n o t be d i f f e r e n t i a t e d by m e d i c a l i m a g i n g , their c h a r a c t e r i s t i c a p p e a r a n c e s m a y a l l o w p r e - o p e r a t i v e d i a g n o s i s a n d a s s e s s m e n t o f their extent. T h e l e s i o n u s u a l l y p r e s e n t s in m i d d l e - a g e d f e m a l e s as a n a b d o m i n a l mass. T h e p r e s e n t i n g clinical f e a t u r e s are n o n - s p e c i f i c ( M a r s h et al., 1974) a n d c o r r e c t d i a g n o s i s often depends on characteristic imaging appearances. T h e u s u a l a p p e a r a n c e o n C T is o f a single, m u l t i l o c u l a r low-density mass with papillary excrescences, nodular t h i c k e n i n g o f i n t e r n a l septa, a n d m u r a l n o d u l e s w h i c h m a y s h o w c o n t r a s t e n h a n c e m e . n t ( C h o i et al., 1989). C a l c i f i c a t i o n is a n u n c o m m o n f i n d i n g ( S h o r t et al., 1971; M a r s h et al., 1974), b u t calcific foci in the s e p t a a n d wall (seen in t w o o f o u r p a t i e n t s ) are o c c a s i o n a l l y d e t e c t e d on C T ( C h o i et al., 1989). T h e u l t r a s o u n d findings w e r e d e s c r i b e d by F o r r e s t et al. (1980). A single m u l t i l o c u l a r cystic lesion w i t h s e p t a t i o n a n d p a p i l l a r y p r o j e c t i o n s , s i m i l a r to a c y s t a d e n o m a o f the o v a r y o r p a n c r e a s , is said to be c h a r a c t e r i s t i c . T h e septa

BILIARY CYSTADENOMA AND CYSTADENOCARCINOMA

are of irregular thickness and distribution, and the locules formed by the septation vary in distribution, size and shape. Papillary growths project from the cyst wall and septa. Although purulent material is occasionally found within the tumour (Ishak et al., 1977; Choi et al., 1989), the characteristic imaging appearances are usually retained. A tumour resembling a cholangitic abscess containing dilated ducts and mobile echogenic material (Case 3), has not been reported previously. Roemer et al. (1981) found that percutaneous cyst aspiration was useful in some cases, usually permitting differentiation from haematoma, abscess, or cyst complicated by haemorrhage or infection. Iemoto et al. (1983) correctly diagnosed a case of cystadenocarcinoma by biopsy under ultrasound guidance. Endoscopic aspiration cytology has also been used for diagnosis (Kanamori et aI., 1985). Fine needle aspiration was performed in our second case using ultrasound guidance, and the adenocarcinoma cells contained in the aspirated material confirmed the diagnosis before exploration. Multifocal intrahepatic cystadenoma is a rare finding (Ishak et al., 1977; Itai et al., 1983; Choi et al., 1989). The small size of satellite lesions, or their close relationship to the main lesion, can make them difficult to detect preoperatively. Moreover, scattered deep-seated lesions render complete removal impossible. Occasionally, these daughter lesions may even grow out of the liver and attach to other abdominal organs (Case 1). The clinical history in this patient also suggested communication of the cyst with the gastric lumen so that vomiting of yellowish fluid would relieve abdominal distension. The treatment of choice is complete tumour removal by hemihepatectomy (Marsh et al., 1974), but if this is impossible, then other procedures such as aspiration, drainage, marsupialization and enucleation (Ishak et al., 1977) or percutaneous cyst drainage with infusion of chemotherapeutic agents (Kanamori et al., 1985) can be tried_ As these lesser procedures are associated with a higher rate of recurrence and malignant transformation (Short et al., 1971; Cahill et al., 1982), close follow-up by ultrasound or CT of abdomen is indicated. Although there were isolated case reports of peritoneal spillage during operation (Ishak et al., 1977; Iemoto et al., 1981), complete removal of these tumours yields excellent results

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and is usually curative (Marsh et al., 1974; Ishak et al., 1977). Distant metastasis after complete removal of tumour has not been reported previously, and the occurrence of pulmonary metastases in our second patient shows the importance of long-term follow-up, even when the primary tumour is apparently completely removed by hemihepatectomy. Acknowledgement: The authors wish to thank H. Y. Yeung, Department of Pathology, for his help and the photographic service for the preparation of the illustrations.

REFERENCES Cahill, C J, Bailey, ME & Smith, MGM (1982). Mucinous cystadenomas of the liver. Clinical Oncology, 8, 171-177. Choi, BI, Lim, JH, Han, MC, Lee, DH, Kim, SH, Kim, YI & Kim, CW (1989). Biliary eystadenoma and eystadenocareinoma: CT and sonographic findings. Radiology, 171, 57 61. Forrest, ME, Cho, KJ, Shields, J J, Wicks, JD, Silver, TM & McCormick, TL (1980). Biliary cystadenomas: sonographic-angiographicpathologic correlations. American Journal of Roentgenology, 135, 723-727. Iemoto, Y, Kondo, Y & Fukamachi, S (1981). Biliary cystadenocarclnoma with peritoneal carcinomatosis. Cancer, 48, 1664-1667. Iemoto, Y, Kondo, Y, Nakano, T, Tsuchiya K &Ohto M (1983). Biliary cystadenocarcinoma diagnosed by liver biopsy performed under ultrasonographic guidance. Gastroenterology, 84, 399-403. Ishak, KG, Willis, GW, Cummins, SD & Bullick, AA (1977). Biliary eystadenoma and eystadenocarcinoma: report of 14 cases and review of the literature. Cancer, 38, 322-338. Itai, Y, Araki, T, Furui, S, Yashiro, N, Ohtomo, K & Iio, M (1983). Computed tomography of primary intrahepatic biliary malignancy. Radiology, 147, 485-490. Kanamori, H, Kawahara, H, Oh, S, Mine, T, Osawa, I-I, Murakami, T et al. (1985). A case of biliary cystadenocarcinoma with recurrent jaundice: diagnostic evaluation of computed tomography. Cancer, 55, 2722-2724. Marsh, JL, Dahms, B & Longmire, WP Jr (1974). Cystadenoma and cystadenoearcinoma of the biliary system. Archives of Surgery, 109, 41 43. Roemer, CE, Ferrucei, JT Jr, Mueller, PR, Simeone, JF, van Sonnenberg, E & Wittenberg, J (1981). Hepatic cysts: diagnosis and therapy by sonographic needle aspiration. American Journal of Roentgenology, 136, 1065 1070. Short, WF, Nedwich, A, Levy, HA & Howard, JM (1971). Biliary cystadenoma: report of a case and review of the literature. Archives of Surgery, 102, 78-80. Stanley, J, Vujic, I, Schabel, SI, Gobien, RP & Reines, HD (1983). Evaluation of biliary cystadenoma and eystadenocarcinoma. Gastrointestinal Radiology, 8, 245-248.

Biliary cystadenoma and cystadenocarcinoma: some unusual features.

Biliary cystadenoma and cystadenocarcinoma are rare tumours which have a good prognosis after complete surgical removal. Correct pre-operative diagnos...
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