Mucinous cystic neoplasms of the liver: presence of biliary communication (original) (raw)
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Mucinous cystic neoplasm of the liver (MCN-L): a rare presentation and review of the literature
Medicine and Pharmacy Reports
Mucinous cystic neoplasms of liver (MCN-L; previously referred to as cystadenomas or cystadenocarcinoma) are rare cystic tumors that occur within the liver parenchyma, or less frequently, in the extrahepatic bile ducts. It is reported to account for <5% of all liver cysts. The differential diagnosis of MCN-L includes intraductal papillary neoplasm of the bile duct (IPNB), intrahepatic cholangiocarcinoma with cystic change, echinococcal cyst, and a simple cyst. Invasive MCNs can only be differentiated from non-invasive MCNs by microscopic evaluation for the presence of ovarian-type stroma. Intraoperative biopsy and frozen section(s) are essential to differentiate MCNs from other cystic liver lesions. The treatment of choice is complete excision and can result in excellent survival with initial correct diagnosis. But because of its rare presentation and insufficient recognition frequently leads to an incorrect initial or delayed diagnosis or often misdiagnosed.
Mucinous cystic neoplasm of the liver
Clinical & Biomedical Research, 2023
A 55-year-old woman was investigated for occasional epigastric pain and weight loss. T2-weighted abdominal magnetic resonance imaging and magnetic resonance cholangiography revealed a multilocular cyst with multiple septa and a solid component in the liver, measuring 6.1 × 4.8 × 6.5 cm. Given the patient's symptoms and malignant potential, a laparoscopic segmentectomy with partial resection of segments IV B and V was performed to completely remove the cystic lesion, associated with cholecystectomy. Histopathology demonstrated a cyst lined by columnar mucinous epithelium. Therefore, the diagnosis was mucinous cystic neoplasm of the liver. This article presents a case report and literature review of this entity.
Modern Pathology, 2011
Mucinous cystic neoplasm of the liver has been a controversial entity, in particular, regarding differentiation from intraductal papillary neoplasm of the bile duct. In this study, we compared the characteristics of hepatic mucinous cystic neoplasms with ovarian-like stroma (n ¼ 29) to those of cyst-forming intraductal papillary neoplasms of the bile duct (n ¼ 12). Radiological or macroscopic appearance, histological grade of malignancy, and postoperative clinical course were recorded. Immunohistochemistry for biliary or gastrointestinal markers was performed to characterize cell phenotypes. The patients with hepatic mucinous cystic neoplasm were all female and ranged in age from 21 to 67 years, which was significantly younger than that in the patients with biliary intraductal papillary neoplasm. Eighteen mucinous cystic neoplasms (76%) were located in the left lobe, with 13 (54%) in segment IV. Mucinous cystic neoplasms were significantly larger than intraductal papillary neoplasms (median diameter: 110 vs 50 mm, P ¼ 0.008). In contrast to intraductal papillary neoplasms that were all histologically malignant, 26 mucinous cystic neoplasms (90%) were adenomas, 2 (7%) were borderline malignant, and 1 (3%) was a carcinoma in situ. Benign mucinous cystadenomas had the pure biliary immunophenotype, whereas gastrointestinal markers including cytokeratin 20 and mucin core proteins 2, 5AC, and 6 were more frequently expressed in borderline or malignant mucinous cystic neoplasms and biliary intraductal papillary neoplasms. There was no mortality in the patients with mucinous cystic neoplasm, whereas one patient with intraductal papillary neoplasm died of cancer. In conclusion, hepatic mucinous cystic neoplasms and biliary intraductal papillary neoplasms have different clinicopathological characteristics as evidenced by differences in the age and gender of patients, macroscopic appearance, immunophenotypes, and grades of malignancy.
Invasive biliary mucinous cystic neoplasm: a review
HPB, 2012
Objectives: Biliary mucinous cystic neoplasms (BMCNs) are recently redefined rare liver tumours in which insufficient recognition frequently leads to an incorrect initial or delayed diagnosis. A concise review of the subtle, sometimes non-specific, clinical, serologic and radiographic features will allow for a heightened awareness and more comprehensive understanding of these entities. Methods: Literature relating to the presentation, diagnosis, treatment, pathology and outcomes of BMCNs and published prior to March 2012 was reviewed. Results: Biliary mucinous cystic neoplasms most commonly occur in females (Ն60%) in the fifth decade of life. Clinical symptoms, serologic markers and imaging modalities are unreliable for diagnosis of BMCNs, which leads to misdiagnosis in 55-100% of patients. Perioperative cyst aspiration is not recommended as invasive BMCNs can only be differentiated from non-invasive BMCNs by microscopic evaluation for the presence of ovarian-type stroma. Intraoperative biopsy and frozen section(s) are essential to differentiate BMCNs from other cystic liver lesions. The treatment of choice is complete excision and can result in excellent survival with initial correct diagnosis. Conclusions: A low threshold for considering BMCN in the differential diagnosis of cystic liver lesions and increased attentiveness to its subtle diagnostic characteristics are imperative. The complete surgical resection of BMCNs and the use of appropriate nomenclature are necessary to improve outcomes and accurately define prognosis.
Liver Mucinous Cystic Neoplasm With Obstructive Jaundice
Cureus, 2022
Biliary mucinous cystic neoplasms (BMCNs) are rare and slow-growing lesions that are usually discovered incidentally. They can imitate various other liver tumors. Here, we present a 31-year-old female patient who presented with complaints of abdominal pain, nausea, shortness of breath, and obstructive jaundice. Ultrasound showed a large, lobulated, cystic liver mass. Abdominal computed tomography (CT) scan showed features suggestive of a hydatid cyst or complicated liver cyst. A laparoscopic deroofing was performed and showed a liver cyst involving segments 2, 3, 4A, and 4B. Histopathology showed that the cyst wall was lined by columnar mucin-producing epithelium with multifocal areas of ovarian-like stroma, and the diagnosis of biliary mucinous cystic neoplasms was made. A one-year, follow-up radiological examination did not show any recurrence. BMCNs are quite rare. The nonspecific nature of the symptoms and radiological characteristics makes the diagnosis of BMCN challenging. Imaging modalities can aid in the diagnosis, but pathological examination is essential in confirming a definite diagnosis.
Cureus 9(11): e1863
Biliary mucinous cystic neoplasms of the liver are rare cystic tumors comprising less than 5% of the liver cystic neoplasms. These tumors demonstrate a female predominance and entail a risk of malignant transformation. We present a 56-year-old female patient with a multiloculated liver cystic lesion measuring 22 cm who underwent a cystectomy with en bloc resection of the liver segments II, III, and cholecystectomy. Serum cancer antigen 19.9 was 4,122.00 U/ml, supporting the diagnosis of a biliary cystic tumor. The cytology of the cystic fluid was negative for malignancy and intracystic fluid cancer antigen 19.9 level was measured over 12,000.00 U/l. The patient is free of recurrence at one-year follow up. Although a rare entity, the biliary mucinous cystic neoplasms should be considered in the differential diagnosis in the patients with liver cystic tumors. The appropriate management with complete surgical resection with negative margins is recommended given the risk of recurrence and malignant transformation.
BMC gastroenterology, 2015
Intrahepatic biliary mucinous cystic neoplasms are rare hepatic tumors and account for less than 5% of intrahepatic cystic lesions. Accurate preoperative diagnosis is difficult and the outcome differs among various treatment modalities.The aim of this study is to investigate the clinico-radiological characteristics of intrahepatic biliary mucinous cystic neoplasms and to establish eligible diagnostic and treatment suggestions. Nineteen patients with intrahepatic biliary cystadenomas and two patients with biliary cystadenocarcinomas were retrospectively reviewed. Their clinico-radiological variables and survival outcome were analyzed. Of the 19 patients with biliary cystadenoma, 16 (84.2 %) were female. 11 (57.9 %) patients had symptoms before operation with the most common presenting symptom being abdominal pain. Among the patients with available data, serum and cystic fluid CA 19-9 levels were invariably elevated and the CA 19-9 level in the cystic fluid was significantly higher th...
Biliary mucinous cystic neoplasm: a case report and review of the literature
2016
Hepatobiliary cystadenomas (HBC) is a rare neoplasm which comprising less than one percent of liver cystic neoplasms. Although it’s known as a benign tumor, but they have a potential for neoplastic transformation. Making a proper diagnosis and ruling out of other differential diagnosis is important because of different treatment. In the present study, we described a case of HBC manifested as idiopathic dominant biliary stricture in common hepatic duct (CHD), on the basis of spiral CT scan and MRI, and elevated CA19-9. With a probable diagnosis of malignant biliary stricture, she underwent ERCP and cholangioscopy that were non-diagnostic and final diagnosis was made surgically. HBCs usually found incicentally as a cystic lesion and biliary stricture without visible cyst in imaging like that seen in cholangiocarcinoma is very unlikely. In truth, this patient is an unusual manifestation of one rare disease.
Journal of Medical Case Reports
Background: Mucinous cystic neoplasm of the liver with extrahepatic growth is a rare benign epithelial neoplasm of the biliary system that presents with a mass effect or is incidentally found on imaging. The tumor affects mostly the common hepatic duct, which is difficult to diagnose preoperatively by radiology, endoscopy, or cystic fluid analysis. Endoscopic ultrasound is a noninvasive tool for the evaluation of features of a cystic lesion and the extent of disease. Optimal treatment is complete tumor resection. Case presentation: A 27-year-old Thai woman was referred to our hospital for investigation and treatment of clinical symptoms of obstructive jaundice and ascending cholangitis, as well as an unknown cause of obstruction. Multiple investigations were performed, including endoscopic retrograde cholangiography and magnetic resonance imaging. Endoscopic ultrasound showed a multiloculated cystic lesion with internal septations without communication to the bile duct, which helped to support a diagnosis of mucinous cystic neoplasm. Eventually, the pathological diagnosis made was mucinous cystic neoplasm of the bile duct. A follow-up clinical examination with imaging at 6 months revealed that the patient was asymptomatic and without recurrence. Conclusions: We report a rare case of a patient with a large mucinous cystic neoplasm of the liver with extrahepatic growth causing biliary obstruction, which was diagnosed on the basis of endoscopic ultrasound features. Following definitive diagnosis, treatment with complete surgical resection using a multidisciplinary approach was successful.