Giant biliary cystadenoma complicated with polycystic liver:A case report

Biliary cystadenoma(BCA)is a rare hepatic neoplasm.Although considered a benign cystic tumor of the liver,BCA has a high risk of recurrence with incomplete excision and a potential risk for malignant degeneration.Correct diagnosis and complete tumor excision with negative margins are the mainstay of treatment.Unfortunately,due to the lack of presenting symptoms,and normal laboratory results in most patients,BCA is hard to distinguish from other cystic lesions of the liver such as biliary cystadenocarcinoma,hepatic cyst,hydatid cyst,Caroli disease,undifferentiated sarcoma,intraductal papillary mucinous tumor,and hepatocellular carcinoma.Ultrasound(US),computed tomography(CT)and magnetic resonance imaging(MRI)may be necessary.They demonstrate intrahepatic cystic lesions with features such as mural nodules,varying wall thickness,papillary projections,and internal septations.Nevertheless,surgery is still the only means of accurate diagnosis.Definitive diagnosis requires histological examination following formal resection.We describe a57-year-old woman initially diagnosed with polycystic liver who was subsequently diagnosed with giant intra-hepatic BCA in the left hepatic lobe.This indicates that both US physicians and hepatobiliary specialists should attach importance to hepatic cysts,and CT or MRI should be performed for further examination when a diagnosis of BCA is suspected.     

Liver transplantation for symptomatic centrohepatic biliary cystadenoma

Biliary cystadenoma is a rare benign cystic tumor of the liver. The mainstay of treatment is complete resection, either by enucleation or by formal hepatectomy, since incomplete removal entails not only constant recurrence but also the risk of malignant transformation to cystadenocarcinoma. A case of symptomatic centrohepatic biliary cystadenoma involving the main vasculobiliary structures of the liver is reported. After an unsuccessful attempt at resection resulting in an intrahepatic biliary injury, relief of jaundice and radical excision were achieved by total hepatectomy and liver transplantation. The patient is now alive and well 4 years after transplant, disease-free, with normal liver and renal function while receiving everolimus monotherapy. This is the first report of liver transplantation performed for the treatment of this tumor. With the case on the background, diagnostic aspects and available therapeutic strategies for biliary cystadenoma are reviewed and discussed.     

Cystic tumors of the liver： A practical approach

Biliary cyst tumors （cystadenoma and cystadenocarcinoma） are an indication for liver resection. They account for only 5% of all solitary cystic lesions of the liver, but differential diagnosis with multiloculated or complicated biliary cysts, atypical hemangiomas, hamartomas and lymphangiomas may be difficult. The most frequent challenge is to differentiate biliary cyst tumors from hemorrhagic cysts. Computerized tomography （CT） and magnetic resonance imaging （MRI） are often not diagnostic and in these cases fine needle aspiration （FNA） is used to confirm the presence of atypical biliary cells. FNA, however, lacks adequate sensitivity and specificity and should always be used in conjunction with imaging. Pre-operative differentiation of cystadenoma from cystadenocarcinoma is impossible and surgery must be performed if a biliary cyst tumor is suspected. When multiple cystic lesions are observed throughout the liver parenchyma, it is important to exclude liver metastasis, of which colonic cancer is the most common primary site. Multiple biliary hamartomas （von Meyenburg complex） can appear as a mixture of solid and cystic lesions and can be confused with cystic metastasis. Strong and uniform T2 hyperintensity on MRI is usually diagnostic, but occasionally a percutaneous biopsy may be required.     

Mucinous biliary cystadenoma with mesenchymal stroma: Expressions of CA 19-9 and carcinoembryonic antigen in serum and cystic fluid

a case of mucinous biliary cystadenoma with mesenchymal stroma (CMS tumor) in a 64-year-old woman is reported. The patient presented with acute abdominal pain and a palpable mass in the upper abdomen. Computed tomography and abdominal sonography showed characteristic multilocular cysts in the left lobe of the liver. Serum CA 19-9 was elevated to 108 U/ml with normal carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) levels. The levels of CA 19-9 and CEA in the cystic fluid were high at 7430 U/ml and 576ng/ml, respectively. The serum CA 19-9 returned to 35 U/ml 4 weeks after tumor resection. These corresponding findings of both tumor markers in the serum and cystic fluid imply that (1) CA 19-9 and CEA both exist in the epithelial component of CMS tumors as evidenced by immunohistochemical stain, (2) serum CA 19-9 is a valuable marker in the diagnosis and monitoring of CMS, and (3) in cystic fluid, there are more significantly high levels of CA 19-9 in CMS compared with levels in simple cyst and polycystic liver disease. Therefore, measurement of CA 19-9 in cystic fluid and serum may be helpful in the differential diagnosis of hepatic cystic lesions.     

Large mucinous biliary cystadenoma with ＂ovarian-like＂ stroma： A case report

Hepatobiliary cystadenomas are rare tumors that are difficult to diagnose preoperatively. They can reach large sizes that make them real intraoperative "surprises". A 63-year-old woman, presented with a symptomatic huge abdominal cystic mass, underwent complete resection of the mass with safety margins. Histopathological report revealed mucinous hepatic cystadenoma with "ovarianlike" stroma and areas of sclerohyalinization. The differential diagnosis of the large cystic tumors which occupy the right hemiabdomen must include the biliary cystadenoma; the complete resection of the tumor with safety margins avoids local recurrence, and therefore represents the optimal therapy because of the malignant potential of the disease. The postoperative follow-up includes abdominal ultrasound or CT scan and CA 19-9 measurement.     

Cystic Lesions of the Pancreas: A Diagnostic and Management Dilemma

Background/Aims: Due to enhanced imaging modalities, pancreatic cysts are being increasingly detected, often as an incidental finding. They comprise a wide range of differing underlying pathologies from completely benign through premalignant to frankly malignant. The exact diagnostic and management pathway of these cysts remains problematic and this review attempts to provide an overview of the pathology underlying pancreatic cystic lesions and suggests appropriate methods of management. Methods: A search was undertaken with a Pubmed database to identify all English articles using the keywords ‘pancreatic cysts’, ‘serous cystadenoma’, ‘intraductal papillary mucinous tumour’, ‘pseudocysts’, ‘mucinous cystic neoplasm’ and ‘solid pseudopapillary tumour’. Results: The mainstay of assessment of pancreatic cysts is cross-sectional imaging incorporating CT and MRI. Fine-needle aspiration (FNA) (often with endoscopic ultrasound) may provide valuable additional information but can lack sensitivity. Symptomatic cysts, increasing age and multilocular cysts (with a solid component and thick walls) are predictors of malignancy. A raised cyst aspirate CEA, CA 19-9 and mucin content (including abnormal cytology), if present, can accurately distinguish premalignant and malignant cysts from benign ones. Conclusion: In summary, all patients with pancreatic cystic lesions, whether asymptomatic or symptomatic, must be thoroughly investigated to ascertain the underlying nature of the cyst. Small asymptomatic cysts (<3 cm) with no suspicious features on imaging or FNA may be safely followed up. Follow-up should continue for at least 4 years, with a repeat FNA if needed. An algorithm for the management of pancreatic cystic tumours is also suggested.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Hemorrhagic hepatic cysts mimicking biliary cystadenoma

The hemorrhagic simple hepatic cyst is extremely rare and can sometimes be confused with biliary cystadenoma or cystadenocarcinoma. Here we present two cases of huge hemorrhagic simple hepatic cysts. Case 1 was a 43-year-old man with a cystic lesion measuring 13 cm× 12 cm in the right hepatic lobe. Ultrasound and computed tomography showed several mural nodules on the irregularly thickened wall and high-density straps inside the cyst. Case 2 was a 60-year-old woman with a huge cyst measuring 15 cm × 14 cm in the central liver. Ultrasound and magnetic resonance imaging showed the cystic wall was unevenly thickened and there were some flame-like prominences on the wall. The iconographic representations of the two cases mimicked biliary cystadenoma. Cystectomy and left hepatectomy were performed for the two patients, respectively. Both patients recovered quickly after their operations and showed no recurrence.     

Updates in diagnosis and management of pancreatic cysts

Incidental pancreatic cysts are commonly encountered with some cysts having malignant potential. The most common pancreatic cystic neoplasms include serous cystadenoma, mucinous cystic neoplasm and intraductal papillary mucinous neoplasm. Risk stratifying pancreatic cysts is important in deciding whether patients may benefit from endoscopic ultrasound (EUS) or surgical resection. Surgery should be reserved for patients with malignant cysts or cysts at high risk for developing malignancy as suggested by various risk features including solid mass, nodule and dilated main pancreatic duct. EUS may supplement magnetic resonance imaging findings for cysts that remain indeterminate or have concerning features on imaging. Various cyst fluid markers including carcinoembryonic antigen, glucose, amylase, cytology, and DNA markers help distinguish mucinous from nonmucinous cysts. This review will guide the practicing gastroenterologist in how to evaluate incidental pancreatic cysts and when to consider referral for EUS or surgery. For presumed low risk cysts, surveillance strategies will be discussed. Managing pancreatic cysts requires an individualized approach that is directed by the various guidelines.     

Biliary cystadenocarcinoma followed up as benign cystadenoma for 10 years

We describe a case of biliary cystadenocarcinoma that showed a longterm clinical course. A 69-year-old Japanese man was admitted to our hospital because of abdominal discomfort. The patient had been diagnosed with benign cystadenoma of the liver at another hospital in 1987 and had been followed up for 10 years. Abdominal ultrasonography (US) and computed tomography (CT) scan demonstrated a unilocular cystic lesion, which included multiple conspicuous papillary protrusions in the left hepatic lobe. Left lateral segmentectomy was performed, and intraoperative cholangiography revealed a communication between the cystic tumor and intrahepatic bile duct. The tumor contained clear mucinous fluid and enfolded multiple yellowish papillary projections on the cystic wall. Histological examination showed the tumor to be biliary cystadenocarcinoma. The patient is doing well 4 years after the operation.     

Significances of serum level and immunohistochemical stain of CA19-9 in simple hepatic cysts and intrahepatic biliary cystic neoplasms]

BACKGROUND/AIMS: In spite of various diagnostic modalities, biliary cystic neoplasms (biliary cystadenoma and cystadenocarcinoma) remain to be difficult to diagnose preoperatively. Recently, there are some reports that elevated CA19-9 level in serum and/or cystic fluid could be a useful finding in the differential diagnosis of biliary cystic neoplasm. This study aimed to evaluate the expression of CA19-9 and to elucidate its significances in intrahepatic biliary cystic neoplasms and simple hepatic cysts. METHODS: In 8 patients with biliary cystic neoplasms and 6 simple hepatic cysts, symptoms, radiologic and laboratory findings were reviewed retrospectively. In 5 biliary cystic neoplasms (4 biliary cystadenomas, 1 biliary cystadenocarcinoma) and 5 simple hepatic cysts, immunohistochemical stainings for CA19-9 were performed with formalin-fixed, paraffin-embedded tissues. RESULTS: In 8 biliary cystic neoplasms, two cases were suspected as biliary cystadenoma preoperatively and 6 cases could not be distinguished from simple cysts or cholangiocarcinoma preoperatively. In 6 simple hepatic cysts, 3 cases were diagnosed preoperatively and 3 cases could not be distinguished from biliary cystadenoma or pancreatic pseudocyst preoperatively. Expression of CA19-9 in simple hepatic cysts and biliary cystic neoplasms were 80% in both groups. Expression of CA19-9 is not related to the elevated level of CA19-9 in serum. CONCLUSIONS: Our data suggests that the elevated level of CA19-9 in serum may not be helpful in the preoprative diagnosis of biliary cystic neoplasm.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

Combination of cyst fluid CEA and CA 125 is an accurate diagnostic tool for differentiating mucinous cystic neoplasms from intraductal papillary mucinous neoplasms

Background/objectivesDespite advances in imaging techniques, diagnosis and management of pancreatic cystic lesions still remains challenging. The objective of this study was to determine the utility of cyst fluid analysis (CEA, CA 19-9, CA 125, amylase, and cytology) in categorizing pancreatic cystic lesions, and in differentiating malignant from benign cystic lesions.MethodsA retrospective analysis of 68 patients with histologically and clinically confirmed cystic lesions was performed. Cyst fluid was obtained by surgical resection (n = 45) or endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) (n = 23). Cyst fluid tumor markers and amylase were measured and compared between the cyst types.ResultsReceiver operating characteristic (ROC) curve analysis of the tumor markers demonstrated that cyst fluid CEA provided the greatest area under ROC curve (AUC) (0.884) for differentiating mucinous versus non-mucinous cystic lesions. When a CEA cutoff value was set at 67.3 ng/ml, the sensitivity, specificity and accuracy for diagnosing mucinous cysts were 89.2%, 77.8%, and 84.4%, respectively. The combination of cyst fluid CEA content >67.3 ng/ml and cyst fluid CA 125 content >10.0 U/ml segregated 77.8% (14/18) of mucinous cystic neoplasms (MCNs) from other cyst subtypes. On the other hand, no fluid marker was useful for differentiating malignant versus benign cystic lesions. Although cytology (accuracy 83.3%) more accurately diagnosed malignant cysts than CEA (accuracy 65.6%), it lacked sensitivity (35.3%).ConclusionsOur results demonstrate that cyst fluid CEA can be a helpful marker in differentiating mucinous from non-mucinous, but not malignant from benign cystic lesions. A combined CEA and CA 125 approach may help segregate MCNs from IPMNs.     

Mucinous cystic tumour of the pancreas presenting with acute pancreatitis

BackgroundThe optimal therapy for mucinous neoplasms of the pancreas is surgical resection because these tumours are either premalignant (cystadenoma) or malignant.Case outlineA 44-year-old previously fit woman presented with sudden onset of epigastric pain. Clinical and laboratory findings were consistent with acute pancreatitis. Abdominal ultrasound scan demonstrated a mature 6-cm cyst in the tail of pancreas and no findings suggestive of cholelithiasis. These findings were confirmed by a CT scan, which also demonstrated splenic infarction and evidence of recent haemorrhage into the cyst. The patient''s abdominal pain persisted after amylase levels returned to normal.ResultsSplenic infarction, a mature cyst in the tail of the pancreas, and peripancreatic inflammation consistent with recent pancreatitis were found at laparotomy. Enbloc distal pancreatectomy and splenectomy were performed. Histological examination of the cyst wall demonstrated a focus of mucinous cystadenoma.DiscussionThis case demonstrates that acute pancreatitis may be the first presentation of a cystic neoplasm.     

Laparoscopic fenestration of multiple giant biliary mucinous cystadenomas of the liver

Biliary cystadenomas of the liver are rare, cystic neoplasms of the biliary ductal system usually occur in middle aged women. We report a case of synchronous multiple huge biliary mucinous cystadenomas with unique features. This is, according to our knowledge, the first report in the literature about three synchronously occurring hepatobiliary cystadenomas. Cystadenomas have a strong tendency to recur, particularly following incomplete excision, and a potential of malignant transformation. A therapeutic re-evaluation may be necessary when the diagnosis of hepatobiliary cystadenoma is made after the operation and an open liver resection should be considered.     

Lymphoepithelial cyst of the pancreas. No evidence for Epstein-Barr virus-related pathogenesis.

Compared to pseudocyst formation after prior pancreatitis, true cysts of the pancreas are rare. Pancreatic cysts with irregular wall components or a mucinous content raise the suspicion for the presence of a cystic neoplasm, and surgical resection is recommended. A case of a patient with a history of prostate cancer is described in whom a cyst of the pancreatic tail was discovered incidentally. Based on the radiographic features, which did not support the presence of a serous cystadenoma, a spleen-preserving distal pancreatectomy was performed. Histologic features were characteristic for a lymphoepithelial cyst (LEC) of the pancreas, lined with thinned squamous epithelium surrounded by benign lymphoid tissue. Since LECs of the parotid gland, which are associated with acquired human immunodeficiency, are frequently related to Epstein-Barr virus (EBV) infection, EBV in situ hybridization was performed and did not reveal evidence for EBV. Twenty-eight instances of pancreatic LECs have been reported, primarily affecting adult males, without evidence of increased numbers of EBV-positive cells. The pathogenesis, differential diagnosis, and clinical implications of lymphoepithelial pancreatic cysts are discussed.     

Cystadenoma of the liver with high levels of ACE and CA 19-9 in the cyst

The development of a cystadenocarcinoma from previously benign cystadenoma is controversial. Neither clinical nor biological factors have been described to explain this transformation. High levels of serum and cystic CEA and CA 19-9 seem to help in the diagnosis of cystadenoma but not cystadenocarcinoma. Definitive histological evaluation is the only means to discriminate malignant from benign cysts. We report a case of cystadenoma of the liver with very high cystic levels and normal serum levels of CEA and CA 19-9.     

Giant benign true cyst of the spleen with high serum level of CA 19-9.

We report the case of a 21-year-old woman with a giant (20 cm) benign mesothelial splenic cyst and a high CA 19-9 serum level (1240 U/ml). The patient underwent resection of the cyst and splenectomy. True non-parasitic splenic cysts are very rare. Only 10 cases of benign true splenic cysts, with a high CA 19-9 serum level, have ever been published in the medical literature written in English. These are reviewed in this paper. We believe that the inner cellular lining of the cyst wall produces CA 19-9, which causes the high CA 19-9 serum level. This is supported by the facts that (1) the inner cellular lining is strongly positive for immunohistochemistry with CA 19-9, and (2) the CA 19-9 serum level returned to normal after resection of the cyst.     

Cystic diseases of the liver and biliary tract (except for hydatid cyst). Role of surgery

The role of surgery in cystic diseases of the liver and biliary tract depends upon the kind of cysts. When they are symptomatic, solitary cysts of the liver may require laparoscopic unroofing. Mucinous cystadenoma should be resected since there is a risk of cystadenocarcinoma. Polycystic liver disease may require surgery when massive hepatomegaly results in pain or a worsening of the patient's general condition. Laparoscopic fenestration and partial hepatectomy are only indicated in a small number of selected patients with large or localized cysts. Orthotopic liver transplantation may be recommended in symptomatic cases with massive hepatomegaly even if there is no renal failure and no need for renal transplantation. Caroli's syndrome localized in one lobe or one segment should be resected since it leads to cholangiocarcinoma in more than 10% of cases. When cystic dilatations are diffuse, liver transplantation may be required. Choledochal cysts should be completely resected since cancer may arise in non resected parts. Complete resection may be associated with major hepatectomy.     

Surgical Management of Pancreatic Cysts: A Shifting Paradigm Toward Selective Resection

Due to the widespread use of high-quality cross-sectional imaging, pancreatic cystic neoplasms are being diagnosed with increasing frequency. Clinicians are therefore asked to counsel a growing number of patients with pancreatic cysts diagnosed incidentally at an early, asymptomatic stage. Over the last two decades, accumulating knowledge on the biologic behavior of these neoplasms along with improved diagnostics through imaging and endoscopic cyst fluid analysis have allowed for a selective therapeutic approach toward these neoplasms. On one end of the management spectrum, observation is recommended for typically benign lesions (serous cystadenoma), and on the other end, upfront resection is recommended for likely malignant lesions (main duct IPMN, mucinous cystadenoma, solid pseudopapillary tumor, and cystic pancreatic neuroendocrine tumors). In between, management of premalignant lesions (branch duct IPMN) is dictated by the presence of high-risk features. In general, resection should be considered whenever the risk of malignancy is higher than the risk of the operation. This review aims to describe the evolution and current status of evidence guiding the selection of patients with pancreatic cystic neoplasms for surgical resection, along with a specific discussion on the type of resection required and expected outcomes.