Cystic Neoplasms of the Liver: Biliary Cystadenoma and Cystadenocarcinoma (original) (raw)
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Biliary cystadenoma a case report
International Surgery Journal, 2021
Cystic diseases of liver occur in 5-10% of the population. Biliary cystadenoma (BCA) and cystadenocarcinoma (BCAC) are rare cystic neoplasms of the liver incidence 5 % of all the cystic lesions of the liver. Origin may be intra hepatic or extra hepatic biliary ducts (10%). Etiology is mainly unknown based on the mesenchymal stroma and the epithelium resembling endodermal (primitive hepatobiliary) cells that can be seen in BCT, Wheeler and Edmondson hypothesized that BCA arise from ectopic rests of embryonic bile ducts. 50% of BCAs found to have endocrine cells suggesting that the origin may be from intrahepatic peribiliary glands. BCAC is thought to originate from either denovo or acquired carcinogenesis in the pre-existing BCA. Intrahepatic BCA are more common and predominantly occurs in Women (90%) and usually asymptomatic. BCAC is more evenly distributed among men and women. Based on the location of origin symptoms may differ. These are first described by Heuter in 1887. BCA is b...
Rare biliary cystic tumors: a case series of biliary cystadenomas and cystadenocarcinoma
Annals of Hepatology, 2016
Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA.
Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver
Genetics and molecular research : GMR, 2014
Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC) are rare biliary duct neoplasms. This study investigated reasonable management strategies of cystic neoplasms in the liver. Charts of 39 BCA/BCAC patients (9 males, 30 female; median age 53.74 ± 14.50 years) who underwent surgery from January 1999 to December 2009 were reviewed retrospectively. Cyst fluid samples of 32 BCA/BCAC patients and 40 simple hepatic cyst patients were examined for the tumor markers carbohydrate associated antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA). The most frequent symptoms were abdominal pain (N = 10), abdominal mass (N = 7), abdominal distension (N = 4), jaundice (N = 2), and fever (N = 3); the remaining patients showed no clinical symptoms. Liver resection (N = 17) or enucleation (N = 22) was performed in the 39 patients. Ultimately, 35 patients were diagnosed with intrahepatic BCA and four patients were diagnosed with BCAC. The median CA19-9 level was significantly higher in ...
World Journal of Surgery, 2006
Introduction: Biliary cystadenomas are complicated cystic lesions of the liver. They are rare and pose considerable diagnostic and therapeutic challenges. Methods: We present our experience managing these lesions by performing a retrospective review of all patients with a preoperative diagnosis of or histologically proven biliary cystadenoma who underwent surgery between January 1995 and January 2005 at our institution. Altogether, 20 patients (16 women, 4 men) with a mean age of 58 years underwent a total of 22 operations. The diagnosis of biliary cystadenoma was based on exclusion of other pathologic entities and the presence of radiologic characteristics of biliary cystadenoma. Abdominal ultrasonography (US), computed tomography, or both were performed in all patients. US-guided fine-needle aspiration cytology was performed in seven patients and all of them were negative for malignancy. A preoperative diagnosis of biliary cystadenoma was made in 16 patients based on clinical and radiologic features and was correct in 6 of them. Diagnosis of biliary cystadenoma was not suspected in four patients. Results: The overall diagnostic accuracy was 30%. Enucleation was the most common surgical procedure and was performed in 10 patients. The mean follow-up period was 5.5 -2.8 years. No recurrence was detected in patients with confirmed biliary cystadenoma after adequate excision. Conclusions: The findings of this study highlight the difficulty with preoperative diagnosis of biliary cystadenoma, which has seldom been discussed in the literature. Preoperative differentiation by means of radiologic imaging is inaccurate (30%). Any therapy short of complete excision leads to local recurrence and risk of malignant transformation. Complete excision of any suspicious lesion remains the best method of diagnosis and treatment.
The objective of this study was to present our experience with intrahepatic biliary cystadenomas and cystadenocarcinomas in 10 patients surgically managed in our department. Intrahepatic biliary cystadenomas and cystadenocarcinomas are rare cystic tumors that are often misdiagnosed preoperatively as simple cysts or hydatid cysts. They recur after incomplete resection and entail a risk of malignant transformation to cystadenocarcinoma. A retrospective review was conducted of patients with histolog-ically confirmed intrahepatic biliary cystadenomas and cystadenocarcinomas between August 2004 and February 2013 who were surgically managed in our department. A total of 10 patients, 9 female and 1 male (mean age, 50 years), with cystic liver were reviewed. The size of the cysts ranged between 3.5 and 16 cm (mean, 10.6). Five patients had undergone previous interventions elsewhere and presented with recurrences. Liver resections included 6 hepatectomies, 2 bisegmentectomies, 1 extended right hepatec-tomy, and 1 enucleation due to the central position and the large size of the lesion. Pathology reports confirmed R0 resections in all cases. All patients were alive after a median follow-up of 6 years (range, 1–10 years), and no recurrence was detected. Intrahepatic biliary cystadenoma and cystadenocarcinoma should be considered in differential diagnosis in patients with liver cystic tumors. Because of the high recurrence rate and difficult accurate preoperative diagnosis, formal liver resection is
Intrahepatic biliary cystadenoma: a need for radical resection
European Journal of Gastroenterology & Hepatology, 2008
Background Intrahepatic biliary cystadenoma (IBC) is a rare liver tumour, which has strong tendency to recur and malignant potential as it can progress to cystadenocarcinoma (IBCa). Methods From June 2003 to December 2006, four patients diagnosed with hepatic cystadenoma were operated on our Liver Surgical Unit. All patients were females with median age of 51 years (range 45-63 years). Liver resections included three left and one right hepatectomies. In two patients, IBC was diagnosed by abdominal imaging and serum tumour markers but the rest of the patients were initially misdiagnosed as simple cysts, treated by laparoscopic fenestration and referred to our unit after cyst recurrence. Results In all cases, the pathology report was consistent with liver cystadenomas. The postoperative course was uneventful and the median hospital stay was 8 days (range 5-12 days). In a median 18-month follow-up (range 2-40 months), all patients are alive and free of recurrence. Conclusion Liver cystadenomas can be easily misdiagnosed with other hepatic cystic lesions. An aggressive surgical approach is recommended, due to their malignant potential and high recurrence rate after fenestration.
Management of Biliary Cystic Tumors
Annals of Surgery, 2014
Objective: To characterize clinical and radiological features associated with biliary cystic tumors (BCTs) of the liver, and to define recurrence-free and overall survival. Background: Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC) are rare tumors that arise in the liver. Methods: Between 1984 and 2013, 248 patients who underwent surgical resection of BCA or BCAC were identified. Clinical and outcome data were analyzed. Results: Median total bilirubin, CA19-9, and carcinoembryonic antigen (CEA) levels were 0.6 mg/dL, 15.0 U/mL, and 2.7 ng/mL, respectively. Preoperative imaging included computed tomography only (62.5%), magnetic resonance imaging only (6.9%), or CT + MRI (18.5%). Features on cross-sectional imaging included multiloculation (56.9%), mural nodularity (16.5%), and biliary ductal dilatation (17.7%). The presence of these factors did not reliably predict BCAC versus BCA (sensitivity, 81%; specificity, 21%). Median biliary cyst size was 10.0 cm (interquartile range, 7-13 cm). Operative interventions included unroofing/partial excision of the lesion (14.1%), less than hemihepatectomy (48.8%), or hemi-/extended hepatectomy (36.3%). On pathology most lesions were BCA (89.1%), whereas 27 (10.9%) were BCAC. At last follow-up, there were 46 (18.3%) recurrences; 2 patients who initially had BCA recurred with BCAC. Median overall survival was 18.1 years; 1year, 3-year, and 5-year survival was 95.0%, 86.8%, and 84.2%, respectively. Long-term outcomes were associated with BCAC versus BCA, as well as the presence of spindle cell/ovarian stroma (both P < 0.05). Conclusions: Among patients undergoing surgery for BCT, associated malignancy was uncommon (10%) and no preoperative findings reliably predicted underlying BCAC. After excision of BCA, long-term outcomes were good; however, patients with BCAC had a worse long-term prognosis.
Biliary cystadenoma: short- and long-term outcome after radical hepatic resection
Updates in Surgery, 2011
The intrahepatic biliary cystadenoma is a rare benign tumor of the liver, originating from an intrahepatic bile duct: it becomes symptomatic only when it causes obstruction of the bile duct itself. Regardless of the various diagnostic modalities available, it is difficult to distinguish preoperatively the cystadenoma both from a simple liver cyst, and from a cystic carcinoma of the bile duct. An incomplete surgical removal of the cyst often results in a higher risk of size increase and recurrence, even considering that the lesion may degenerate into a cystadenocarcinoma. Between January 2004 and May 2011, 1,173 liver resections were carried out at the Hepatobiliary Surgery Unit of San Raffaele Hospital: 12 of these were performed for cystadenoma. Forty-six patients underwent laparoscopic liver cysts deroofing: definitive histological examination in six of these patients revealed instead the diagnosis of cystadenoma. In 50% of cases, the diagnosis of cystadenoma was therefore acquired as a result of an incidental finding. The patients were all female, median age 45 years.