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ARTICLE IN PRESS Digestive and Liver Disease xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld
Correspondence Mucinous cystic neoplasm of common hepatic duct Dear Editor, A 44 year-old woman presented with abdominal pain, jaundice, anorexia and vomiting. Blood tests showed deranged liver function (raised alkaline phosphatase, alanine transaminase and bilirubin levels). An abdominal ultrasound demonstrated a 10 cm × 9 cm multiseptate mass possibly related to the biliary tree. An abdominal contrast-enhanced computerized tomography (CT) scan showed marked intrahepatic biliary and proximal common hepatic duct (CHD) dilatation secondary to a large multiseptate cystic mass in the region of the porta hepatis (Fig. 1a). The common bile duct was not visualized due to the presence of the mass. Magnetic Resonance Cholangio-Pancreatography (MRCP) (Fig. 1b) characterized the lesion as having thin septa with only one small nodular area, and present extrinsic to liver and pancreas. Gallbladder was seen to be separate with some extrinsic compression. It was not possible to determine whether there was communication with the biliary tree or whether the mass was just causing extrinsic compression. On-table cholangiography at the time of surgery demonstrated communication with the biliary tree and the patient underwent an excision of the cyst, the extrahepatic biliary tree and gallbladder. The CHD contained a 70 mm cystic, smooth-walled lesion, divided into multiple thin-walled locules by fibrous septa and without solid/papillary areas. The cyst contained thin fluid. Postoperative recovery was uneventful. Histopathological examination revealed multiloculated cyst lined by bland mucinous epithelium, resting on an ovarian-type stroma (Figure S1, panels a and b) that
was diffusely positive for oestrogen/progesterone receptors and focally positive for inhibin on immunohistochemistry (Figure S1, panels c and d). High grade dysplasia or malignant transformation was not seen. This lesion was diagnosed as an extrahepatic biliary mucinous cystadenoma and was completely excised. At 12 months’ follow-up liver function tests were normal with no evidence of disease. Biliary cystadenoma of the extrahepatic biliary tree is rarer compared to intrahepatic cystadenoma, and in literature is subject to small series [1,2]. Most of these mucinous cystadenoma arise in women, being similar to their pancreatic counterpart. In the biliary tree, the presenting symptoms include most commonly obstructive jaundice [3], but may include abdominal pain, fever, haemobilia and recurrent cholangitis [4]. Extrahepatic biliary cystadenoma can arise in the right or left hepatic ducts, the common hepatic duct, the common bile duct or the cystic duct. Imaging differential diagnosis for cystic lesions in this location include choledochal cysts, inclusion cysts, lesions that are more centred within the liver, such as liver abscesses and hydatid disease, those that are of pancreatic origin like pancreatic cystadenoma or pseudocysts and possibly also unusual duodenal diverticula. In this particular case, MRCP correctly predicted the pathological diagnosis because of the size and thin multi-septate nature of the lesion. It was reasonably clear that it did not arise in either liver or pancreas and there were no clinical features suggestive of an inflammatory process. Whilst a unilocular biliary cystadenoma might be considered to represent a choledochal cyst, the converse is not true with a multicystic lesion [5]. Histologically these lesions have a characteristic appearance, being composed of a multilocular cyst lined by bland mucinous epithelium resting on an ovarian type
Fig. 1. Panel a – An abdominal contrast-enhanced CT showing a large multiseptate cystic mass in the region of the porta hepatis. Panel b – MRCP demonstrated a lesion extrinsic to liver and pancreas with thin septa and only one small area of nodularity. http://dx.doi.org/10.1016/j.dld.2014.03.019 1590-8658/© 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Silvanto A, et al. Mucinous cystic neoplasm of common hepatic duct. Dig Liver Dis (2014), http://dx.doi.org/10.1016/j.dld.2014.03.019
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ARTICLE IN PRESS
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Correspondence / Digestive and Liver Disease xxx (2014) xxx–xxx
stroma [2], and which is oestrogen/progesterone receptors and inhibin positive. Whilst most of these lesions are benign, as in the case of pancreatic mucinous cystic neoplasms, a risk of malignant transformation does exist. Wheeler et al. reported 4/17 cases with malignant changes [2] and Davies et al. identified one case with foci of invasive adenocarcinoma, highlighting the malignant potential of these neoplasms. This combined with a 50% local recurrence rate in cases treated by local excision in their series indicates that complete excision with negative margins is the recommended treatment [1]. Conflict of interest None declared. Appendix A. Supplementary data Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.dld.2014.03.019. References [1] Davies W, Chow M, Nagorney D. Extrahepatic biliary cystadenomas and cystadenocarcinoma. Annals of Surgery 1995;222:619–25. [2] Wheeler DA, Edmondson HA. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. Cancer 1985;56:1434–5. [3] Kimura H, Kagawa K, Deguchi T, et al. Extrahepatic biliary cystadenocarcinoma arising from the left hepatic duct. Journal of Gastroenterology 1998;33:895–8.
[4] Ray S, Khamrui S, Mrida AR, et al. Extrahepatic biliary cystadenoma: an unusual cause of recurrent cholangitis. American Journal of Surgery 2010;199:e3–4. [5] Park J, Lee DH, Kim HJ, et al. Unilocular extrahepatic biliary cystadenoma mimicking choledochal cyst: a case report. Korean Journal of Radiology 2004;5: 287–90.
Anna Silvanto Department of Histopathology, Royal Surrey County Hospital, Guildford, UK Fuad Hussain Department of Radiology, Royal Surrey County Hospital, Guildford, UK Angela Riga Department of General Surgery, Royal Surrey County Hospital, Guildford, UK Izhar N. Bagwan ∗ Department of Histopathology, Royal Surrey County Hospital, Guildford, UK ∗ Corresponding author at: Department of Histopathology, The Royal Surrey County Hospital, Egerton Road, Guildford GU2 7XX, UK. Tel.: +44 1483571122. E-mail addresses: [email protected], [email protected] (I.N. Bagwan)
Please cite this article in press as: Silvanto A, et al. Mucinous cystic neoplasm of common hepatic duct. Dig Liver Dis (2014), http://dx.doi.org/10.1016/j.dld.2014.03.019
ARTICLE IN PRESS Digestive and Liver Disease xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld
Correspondence Mucinous cystic neoplasm of common hepatic duct Dear Editor, A 44 year-old woman presented with abdominal pain, jaundice, anorexia and vomiting. Blood tests showed deranged liver function (raised alkaline phosphatase, alanine transaminase and bilirubin levels). An abdominal ultrasound demonstrated a 10 cm × 9 cm multiseptate mass possibly related to the biliary tree. An abdominal contrast-enhanced computerized tomography (CT) scan showed marked intrahepatic biliary and proximal common hepatic duct (CHD) dilatation secondary to a large multiseptate cystic mass in the region of the porta hepatis (Fig. 1a). The common bile duct was not visualized due to the presence of the mass. Magnetic Resonance Cholangio-Pancreatography (MRCP) (Fig. 1b) characterized the lesion as having thin septa with only one small nodular area, and present extrinsic to liver and pancreas. Gallbladder was seen to be separate with some extrinsic compression. It was not possible to determine whether there was communication with the biliary tree or whether the mass was just causing extrinsic compression. On-table cholangiography at the time of surgery demonstrated communication with the biliary tree and the patient underwent an excision of the cyst, the extrahepatic biliary tree and gallbladder. The CHD contained a 70 mm cystic, smooth-walled lesion, divided into multiple thin-walled locules by fibrous septa and without solid/papillary areas. The cyst contained thin fluid. Postoperative recovery was uneventful. Histopathological examination revealed multiloculated cyst lined by bland mucinous epithelium, resting on an ovarian-type stroma (Figure S1, panels a and b) that
was diffusely positive for oestrogen/progesterone receptors and focally positive for inhibin on immunohistochemistry (Figure S1, panels c and d). High grade dysplasia or malignant transformation was not seen. This lesion was diagnosed as an extrahepatic biliary mucinous cystadenoma and was completely excised. At 12 months’ follow-up liver function tests were normal with no evidence of disease. Biliary cystadenoma of the extrahepatic biliary tree is rarer compared to intrahepatic cystadenoma, and in literature is subject to small series [1,2]. Most of these mucinous cystadenoma arise in women, being similar to their pancreatic counterpart. In the biliary tree, the presenting symptoms include most commonly obstructive jaundice [3], but may include abdominal pain, fever, haemobilia and recurrent cholangitis [4]. Extrahepatic biliary cystadenoma can arise in the right or left hepatic ducts, the common hepatic duct, the common bile duct or the cystic duct. Imaging differential diagnosis for cystic lesions in this location include choledochal cysts, inclusion cysts, lesions that are more centred within the liver, such as liver abscesses and hydatid disease, those that are of pancreatic origin like pancreatic cystadenoma or pseudocysts and possibly also unusual duodenal diverticula. In this particular case, MRCP correctly predicted the pathological diagnosis because of the size and thin multi-septate nature of the lesion. It was reasonably clear that it did not arise in either liver or pancreas and there were no clinical features suggestive of an inflammatory process. Whilst a unilocular biliary cystadenoma might be considered to represent a choledochal cyst, the converse is not true with a multicystic lesion [5]. Histologically these lesions have a characteristic appearance, being composed of a multilocular cyst lined by bland mucinous epithelium resting on an ovarian type
Fig. 1. Panel a – An abdominal contrast-enhanced CT showing a large multiseptate cystic mass in the region of the porta hepatis. Panel b – MRCP demonstrated a lesion extrinsic to liver and pancreas with thin septa and only one small area of nodularity. http://dx.doi.org/10.1016/j.dld.2014.03.019 1590-8658/© 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Silvanto A, et al. Mucinous cystic neoplasm of common hepatic duct. Dig Liver Dis (2014), http://dx.doi.org/10.1016/j.dld.2014.03.019
G Model YDLD-2620; No. of Pages 2
ARTICLE IN PRESS
2
Correspondence / Digestive and Liver Disease xxx (2014) xxx–xxx
stroma [2], and which is oestrogen/progesterone receptors and inhibin positive. Whilst most of these lesions are benign, as in the case of pancreatic mucinous cystic neoplasms, a risk of malignant transformation does exist. Wheeler et al. reported 4/17 cases with malignant changes [2] and Davies et al. identified one case with foci of invasive adenocarcinoma, highlighting the malignant potential of these neoplasms. This combined with a 50% local recurrence rate in cases treated by local excision in their series indicates that complete excision with negative margins is the recommended treatment [1]. Conflict of interest None declared. Appendix A. Supplementary data Supplementary material related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.dld.2014.03.019. References [1] Davies W, Chow M, Nagorney D. Extrahepatic biliary cystadenomas and cystadenocarcinoma. Annals of Surgery 1995;222:619–25. [2] Wheeler DA, Edmondson HA. Cystadenoma with mesenchymal stroma (CMS) in the liver and bile ducts. Cancer 1985;56:1434–5. [3] Kimura H, Kagawa K, Deguchi T, et al. Extrahepatic biliary cystadenocarcinoma arising from the left hepatic duct. Journal of Gastroenterology 1998;33:895–8.
[4] Ray S, Khamrui S, Mrida AR, et al. Extrahepatic biliary cystadenoma: an unusual cause of recurrent cholangitis. American Journal of Surgery 2010;199:e3–4. [5] Park J, Lee DH, Kim HJ, et al. Unilocular extrahepatic biliary cystadenoma mimicking choledochal cyst: a case report. Korean Journal of Radiology 2004;5: 287–90.
Anna Silvanto Department of Histopathology, Royal Surrey County Hospital, Guildford, UK Fuad Hussain Department of Radiology, Royal Surrey County Hospital, Guildford, UK Angela Riga Department of General Surgery, Royal Surrey County Hospital, Guildford, UK Izhar N. Bagwan ∗ Department of Histopathology, Royal Surrey County Hospital, Guildford, UK ∗ Corresponding author at: Department of Histopathology, The Royal Surrey County Hospital, Egerton Road, Guildford GU2 7XX, UK. Tel.: +44 1483571122. E-mail addresses: [email protected], [email protected] (I.N. Bagwan)
Please cite this article in press as: Silvanto A, et al. Mucinous cystic neoplasm of common hepatic duct. Dig Liver Dis (2014), http://dx.doi.org/10.1016/j.dld.2014.03.019
