Cl&ical Radiology (1991) 43, 210 212
Case Report: Extra-hepatic Biliary Cystadenoma in Association with Adenomyomatosis of the Gall-bladder T. H O D G S O N ,
S. F O X * a n d P. B A Y J O O t
Departments of Radiology, *Pathology and eiSurgery, Royal Hallamshire Hospital, Sheffield Biliary cystadenoma is a rare cystic tumour of biliary origin. We report on the ultrasound, CT and angiographic appearances of an extra-hepatic biliary cystadenoma arising from the left main hepatic duct with compression of the quadrate lobe. We also describe the histological finding of adenomyomatosis of the gall-bladder in association with the cystadenoma, an association not previously reported. Hodgson, T., Fox, S. & Bayjoo, P. (1991). Clinical
Radiology 43, 2 1 0 - 2 1 2 . C a s e R e p o r t : E x t r a - h e p a t i c Biliary C y s t a d e n o m a in A s s o c i a t i o n w i t h A d e n o m y o m a t o s i s o f the G a l l - b l a d d e r
CASE REPORT A 56-year-old lady presented with a 4 month history of epigastric pain, worsened by food. There was a large non-tender mass in the right upper quadrant but clinical examination was otherwise unremarkable. Ultrasonography (Fig. 1) revealed a large, well-defined mass arising from the liver, composed of cystic spaces separated by septa, some of the septa containing bulky solid elements. Several of the cysts showed internal echoes consistent with debris but no calcification was shown. The gall-bladder was not visualized. CT (Fig. 2) confirmed the presence of a well encapsulated, cystic mass in the left lobe of liver and projecting inferiorly. The cysts had variable attenuation values of between 0 and 20 Hounsfield Units. After intravenous contrast some enhancement of the septae and cysts was seen and the absence of calcification confirmed. Prior to surgery, visceral angiography (Fig. 3a) revealed a large relatively avascular lesion lying within and inferior to the liver supplied by the left and right hepatic arteries causing stretching of vessels around its periphery and patchy increased vascularity within its centre. The right hepatic artery arose directly from the coeliac axis. The portal vein was stretched around the superior aspect of the mass (Fig. 3b) but there was no evidence of invasion, or of inferior vena caval obstruction. At laparotomy a multi-loculated mass, arising from the left main hepatic duct was seen, splaying the ducts and vessels. The mass extended into the liver causing quadrate lobe compression, with further extension
Fig. 2 A contrast enhanced CT scan showing the well-defined biliary cystadenoma with patchy enhancement of septae and cysts.
inferiorly. Communication of the mass with the hepatic ducts was confirmed by operative cholangiography (Fig. 4). Once separated from the left hepatic duct, the lesion was dissected easily from the quadrate lobe without necessitating hepatic resection. A cholecystectomy was performed and a T-tube inserted into the common bile duct. The patient made an uneventful recovery. T-tube cholangiography on the tenth day confirmed the restoration of normal bile duct anatomy. Histology showed the mass to be a completely excised benign biliary cystadenoma with associated adenomyomatosis of the gall-bladder.
DISCUSSION
Fig. I - Ultrasound demonstrating the multiple cystic spaces and septae characteristic of a biliary cystadenoma. Correspondence to: T. Hodgson, Department of Radiology, Royal Hallamshire Hospital, Glossop Road, Sheffield Sl0 2JF.
B i l i a r y c y s t a d e n o m a s c o m p r i s e less t h a n 5 % o f intrah e p a t i c cystic lesions o f biliary o r i g i n ( I s h a k et al., 1977; F o r r e s t et al., 1980). Biliary c y s t a d e n o m a s o c c u r p r e d o m i n a n t l y in m i d d l e - a g e d w o m e n , w i t h a r e p o r t e d age r a n g e o f 19 to 71 years ( F o r r e s t et al., 1980; W h e e l e r a n d E d m o n d s o n , 1985). T h e y are t h o u g h t to be d e v e l o p m e n tal in o r i g i n arising f r o m a b e r r a n t h a m a r t o m a t o u s bile d u c t s o r e c t o p i c rests o f e m b r y o n i c g a l l - b l a d d e r ( I s h a k et al., 1977; W h e e l e r a n d E d m o n t o n , 1985; S t a n l e y et al., 1983). T h e h i s t o l o g i c a l f i n d i n g o f g a l l - b l a d d e r a d e n o m y o m a t o s i s , itself a d e v e l o p m e n t a l d e f e c t ( C u s c h i e r i et al., 1982), a d d s s u p p o r t to the t h e o r y o f the o r i g i n o f biliary c y s t a d e n o m a s . T h e m a j o r i t y o f biliary c y s t a d e n o m a s
EXTRA-HEPATIC BILIARY CYSTADENOMA
211
(a)
Fig. 4 Operative cholangiography revealing filling of the biliary cystadenoma from the hepatic ducts. (A surgical clamp has been applied across the porta at the level of the cystic duct.)
(b) Fig. 3 Arterial (a) and venous (b) phase of a coeliac angiogram. The right hepatic artery arises directly from the coeliac axis (open arrow). A relatively avascular mass is noted supplied by the right and left hepatic arteries. The vessels are stretched around the periphery of the mass (arrows). In the late phase (b) the portal vein is also seen to be stretched around the mass superiorly (arrow) and some patchy vascularity is noted within the septae.
(85%) are entirely intra-hepatic, the remainder arising from the extra-hepatic ducts and gall-bladder. The ultrasound appearances of cystadenomas are of ovoid, cystic, anechoic masses with multiple septa. The cystic spaces occasionally demonstrate internal echoes suggesting infection or haemorrhage (Frick and Feinberg, 1982; Choi et al., 1989). Other reported ultrasound appearances include papillary ingrowths and nodules lying along the internal septa (Choi et al., 1989). The presence of solid nodular projections are thought to occur more frequently with cystadenocarcinoma than cystadenoma (Ishak et al., 1977; Wheeler and Edmonton, 1985). Computed tomography of biliary cystadenoma reveals rnulti-loculated cystic areas ranging from 0 to 30 Hounsfield Units (Genkins et al., 1988; Choi et al., 1989). The variation of attenuation is due to the cysts containing serous fluid, mucin, pus or cholesterol (Genkins et al.,
1988). Internal septations are easily seen which, as in our case, may enhance with intravenous contrast (Frick and Feinberg, 1982; Itai et al., 1983). Computed tomography may detect calcification in these lesions. Various authors state that calcification, particularly when the pattern is coarse and associated with nodular projections, is commoner in cystadenocarcinoma than cystadenoma (Choi et al., 1989; Korobkin et al., 1989). Angiography of biliary cystadenoma rarely aids the diagnosis but helps to delineate vascular anatomy prior to operative resection. The reported features include hypovascularity, with stretching and displacement of surrounding vessels associated with clusters of fine abnormal vessels at the periphery and penetrating into the lesion corresponding to the septa and papillary ingrowths (Genkins et al., 1988; Lewis et al., 1988). The liver parenchyma adjacent to the lesion may show a dense stain of contrast corresponding to compressed normal liver tissue (Forrest et al., 1980). Other lesions to be considered in the differential diagnosis of cystic liver conditions include benign hepatic cysts, necrotic neoplasms, echinococcosis, Caroli's disease and cystic hamartomas. The radiological appearances of cystadenomas are varied and it is rare for the diagnosis to be made pre-operatively. The treatment for all these lesions should be surgery with complete excision. Inadequate excision of both cystadenomas and cystadenocarcinomas will lead to recurrence in all cases (Lewis et al., 1988).
212
CLINICAL RADIOLOGY
Acknowledgements: We wish to thank Mr G. Jacob, Senior Lecturer in Surgery, for allowing us to report on this patient.
REFERENCES Choi, BI, Lim, JE, Han, MC, Lee, DH, Kim, SH, Kim, YI et al. (1989). Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. Radiology, 171, 57-61_ Cuschieri, A, Giles, GR & Moossa, AR. (1982). Essential Surgical Practice. Wright, London. p. 1060. Forrest, ME, Cho, KJ, Shields, JJ, Wicks, JD, Silver, TM & McCormick, TLK (1980). Biliary cystadenomas: sonographic-angiographic-pathologic correlations. American Journal of Roentgenology, 135, 723 727. Frick, MP & Feinberg, SB (1982). Biliary cystadenoma. American Journal of Roentgenology, 139, 393 395. Genkins, SM, Tucker, JA, Seigler, HF & Dunnick, WR (1988). Case report--biliary cystadenoma with mesenchymal stroma: CT and angiographic appearances. Journal of Computer Assisted Tomography, 12, 527 529.
Ishak, KG, Willis, GW, Cummins, SD & Bullock, AA (1977). Biliary cystadenoma and cystadenocarcinoma--report of 14 cases and review of the literature. Cancer, 38, 322 328. Itai, Y, Araki, T, Furui, S, Yashiro, N, Ohtomo, K & Iio, M. (1983). Computed tomography of primary intrahepatic biliary malignancy. Radiology, 147, 485 490. Korobkin, M, Stephens, DH, Lee, JKT, Stanley, RJ, Fishman, EK, Francis, IR et al. (1989). Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. American Journal of Roentgenology, 153, 507 511. Lewis, WD, Jenkins, RL, Munson, L, ReMine, SG, Cady, B e t al. (1988). Surgical treatment of biliary cystadenom~a report of 15 cases. Archives of Surgery, 123, 563-568. Stanley, J, Vujic, I, Schabel, SI, Gobien, RP & Reines, HD (1983). Evaluation of biliary cystadenoma and cystadenocarcinoma. Gastrointestinal Radiology, 8, 245 248. Wheeler, DA & Edmondson, HA (1985). Cystadenoma and mesenchymal stroma (CMS) in the liver and bile ducts. Cancer, 56, 1434 1445.
Case Report: Extra-hepatic Biliary Cystadenoma in Association with Adenomyomatosis of the Gall-bladder T. H O D G S O N ,
S. F O X * a n d P. B A Y J O O t
Departments of Radiology, *Pathology and eiSurgery, Royal Hallamshire Hospital, Sheffield Biliary cystadenoma is a rare cystic tumour of biliary origin. We report on the ultrasound, CT and angiographic appearances of an extra-hepatic biliary cystadenoma arising from the left main hepatic duct with compression of the quadrate lobe. We also describe the histological finding of adenomyomatosis of the gall-bladder in association with the cystadenoma, an association not previously reported. Hodgson, T., Fox, S. & Bayjoo, P. (1991). Clinical
Radiology 43, 2 1 0 - 2 1 2 . C a s e R e p o r t : E x t r a - h e p a t i c Biliary C y s t a d e n o m a in A s s o c i a t i o n w i t h A d e n o m y o m a t o s i s o f the G a l l - b l a d d e r
CASE REPORT A 56-year-old lady presented with a 4 month history of epigastric pain, worsened by food. There was a large non-tender mass in the right upper quadrant but clinical examination was otherwise unremarkable. Ultrasonography (Fig. 1) revealed a large, well-defined mass arising from the liver, composed of cystic spaces separated by septa, some of the septa containing bulky solid elements. Several of the cysts showed internal echoes consistent with debris but no calcification was shown. The gall-bladder was not visualized. CT (Fig. 2) confirmed the presence of a well encapsulated, cystic mass in the left lobe of liver and projecting inferiorly. The cysts had variable attenuation values of between 0 and 20 Hounsfield Units. After intravenous contrast some enhancement of the septae and cysts was seen and the absence of calcification confirmed. Prior to surgery, visceral angiography (Fig. 3a) revealed a large relatively avascular lesion lying within and inferior to the liver supplied by the left and right hepatic arteries causing stretching of vessels around its periphery and patchy increased vascularity within its centre. The right hepatic artery arose directly from the coeliac axis. The portal vein was stretched around the superior aspect of the mass (Fig. 3b) but there was no evidence of invasion, or of inferior vena caval obstruction. At laparotomy a multi-loculated mass, arising from the left main hepatic duct was seen, splaying the ducts and vessels. The mass extended into the liver causing quadrate lobe compression, with further extension
Fig. 2 A contrast enhanced CT scan showing the well-defined biliary cystadenoma with patchy enhancement of septae and cysts.
inferiorly. Communication of the mass with the hepatic ducts was confirmed by operative cholangiography (Fig. 4). Once separated from the left hepatic duct, the lesion was dissected easily from the quadrate lobe without necessitating hepatic resection. A cholecystectomy was performed and a T-tube inserted into the common bile duct. The patient made an uneventful recovery. T-tube cholangiography on the tenth day confirmed the restoration of normal bile duct anatomy. Histology showed the mass to be a completely excised benign biliary cystadenoma with associated adenomyomatosis of the gall-bladder.
DISCUSSION
Fig. I - Ultrasound demonstrating the multiple cystic spaces and septae characteristic of a biliary cystadenoma. Correspondence to: T. Hodgson, Department of Radiology, Royal Hallamshire Hospital, Glossop Road, Sheffield Sl0 2JF.
B i l i a r y c y s t a d e n o m a s c o m p r i s e less t h a n 5 % o f intrah e p a t i c cystic lesions o f biliary o r i g i n ( I s h a k et al., 1977; F o r r e s t et al., 1980). Biliary c y s t a d e n o m a s o c c u r p r e d o m i n a n t l y in m i d d l e - a g e d w o m e n , w i t h a r e p o r t e d age r a n g e o f 19 to 71 years ( F o r r e s t et al., 1980; W h e e l e r a n d E d m o n d s o n , 1985). T h e y are t h o u g h t to be d e v e l o p m e n tal in o r i g i n arising f r o m a b e r r a n t h a m a r t o m a t o u s bile d u c t s o r e c t o p i c rests o f e m b r y o n i c g a l l - b l a d d e r ( I s h a k et al., 1977; W h e e l e r a n d E d m o n t o n , 1985; S t a n l e y et al., 1983). T h e h i s t o l o g i c a l f i n d i n g o f g a l l - b l a d d e r a d e n o m y o m a t o s i s , itself a d e v e l o p m e n t a l d e f e c t ( C u s c h i e r i et al., 1982), a d d s s u p p o r t to the t h e o r y o f the o r i g i n o f biliary c y s t a d e n o m a s . T h e m a j o r i t y o f biliary c y s t a d e n o m a s
EXTRA-HEPATIC BILIARY CYSTADENOMA
211
(a)
Fig. 4 Operative cholangiography revealing filling of the biliary cystadenoma from the hepatic ducts. (A surgical clamp has been applied across the porta at the level of the cystic duct.)
(b) Fig. 3 Arterial (a) and venous (b) phase of a coeliac angiogram. The right hepatic artery arises directly from the coeliac axis (open arrow). A relatively avascular mass is noted supplied by the right and left hepatic arteries. The vessels are stretched around the periphery of the mass (arrows). In the late phase (b) the portal vein is also seen to be stretched around the mass superiorly (arrow) and some patchy vascularity is noted within the septae.
(85%) are entirely intra-hepatic, the remainder arising from the extra-hepatic ducts and gall-bladder. The ultrasound appearances of cystadenomas are of ovoid, cystic, anechoic masses with multiple septa. The cystic spaces occasionally demonstrate internal echoes suggesting infection or haemorrhage (Frick and Feinberg, 1982; Choi et al., 1989). Other reported ultrasound appearances include papillary ingrowths and nodules lying along the internal septa (Choi et al., 1989). The presence of solid nodular projections are thought to occur more frequently with cystadenocarcinoma than cystadenoma (Ishak et al., 1977; Wheeler and Edmonton, 1985). Computed tomography of biliary cystadenoma reveals rnulti-loculated cystic areas ranging from 0 to 30 Hounsfield Units (Genkins et al., 1988; Choi et al., 1989). The variation of attenuation is due to the cysts containing serous fluid, mucin, pus or cholesterol (Genkins et al.,
1988). Internal septations are easily seen which, as in our case, may enhance with intravenous contrast (Frick and Feinberg, 1982; Itai et al., 1983). Computed tomography may detect calcification in these lesions. Various authors state that calcification, particularly when the pattern is coarse and associated with nodular projections, is commoner in cystadenocarcinoma than cystadenoma (Choi et al., 1989; Korobkin et al., 1989). Angiography of biliary cystadenoma rarely aids the diagnosis but helps to delineate vascular anatomy prior to operative resection. The reported features include hypovascularity, with stretching and displacement of surrounding vessels associated with clusters of fine abnormal vessels at the periphery and penetrating into the lesion corresponding to the septa and papillary ingrowths (Genkins et al., 1988; Lewis et al., 1988). The liver parenchyma adjacent to the lesion may show a dense stain of contrast corresponding to compressed normal liver tissue (Forrest et al., 1980). Other lesions to be considered in the differential diagnosis of cystic liver conditions include benign hepatic cysts, necrotic neoplasms, echinococcosis, Caroli's disease and cystic hamartomas. The radiological appearances of cystadenomas are varied and it is rare for the diagnosis to be made pre-operatively. The treatment for all these lesions should be surgery with complete excision. Inadequate excision of both cystadenomas and cystadenocarcinomas will lead to recurrence in all cases (Lewis et al., 1988).
212
CLINICAL RADIOLOGY
Acknowledgements: We wish to thank Mr G. Jacob, Senior Lecturer in Surgery, for allowing us to report on this patient.
REFERENCES Choi, BI, Lim, JE, Han, MC, Lee, DH, Kim, SH, Kim, YI et al. (1989). Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. Radiology, 171, 57-61_ Cuschieri, A, Giles, GR & Moossa, AR. (1982). Essential Surgical Practice. Wright, London. p. 1060. Forrest, ME, Cho, KJ, Shields, JJ, Wicks, JD, Silver, TM & McCormick, TLK (1980). Biliary cystadenomas: sonographic-angiographic-pathologic correlations. American Journal of Roentgenology, 135, 723 727. Frick, MP & Feinberg, SB (1982). Biliary cystadenoma. American Journal of Roentgenology, 139, 393 395. Genkins, SM, Tucker, JA, Seigler, HF & Dunnick, WR (1988). Case report--biliary cystadenoma with mesenchymal stroma: CT and angiographic appearances. Journal of Computer Assisted Tomography, 12, 527 529.
Ishak, KG, Willis, GW, Cummins, SD & Bullock, AA (1977). Biliary cystadenoma and cystadenocarcinoma--report of 14 cases and review of the literature. Cancer, 38, 322 328. Itai, Y, Araki, T, Furui, S, Yashiro, N, Ohtomo, K & Iio, M. (1983). Computed tomography of primary intrahepatic biliary malignancy. Radiology, 147, 485 490. Korobkin, M, Stephens, DH, Lee, JKT, Stanley, RJ, Fishman, EK, Francis, IR et al. (1989). Biliary cystadenoma and cystadenocarcinoma: CT and sonographic findings. American Journal of Roentgenology, 153, 507 511. Lewis, WD, Jenkins, RL, Munson, L, ReMine, SG, Cady, B e t al. (1988). Surgical treatment of biliary cystadenom~a report of 15 cases. Archives of Surgery, 123, 563-568. Stanley, J, Vujic, I, Schabel, SI, Gobien, RP & Reines, HD (1983). Evaluation of biliary cystadenoma and cystadenocarcinoma. Gastrointestinal Radiology, 8, 245 248. Wheeler, DA & Edmondson, HA (1985). Cystadenoma and mesenchymal stroma (CMS) in the liver and bile ducts. Cancer, 56, 1434 1445.
![[Adenomyomatosis of the gallbladder: A case report].](/assets/img/hold.png)
