Infrequent intrahepatic cystic neoplasm: dilemmas in diagnosis and management

Abstract

Biliary mucinous cystic neoplasm (BMCN) is a rare intrahepatic neoplasm comprising approximately 5% of cystic liver lesions. It can cause diagnostic dilemmas with most common differentials being complex hepatic cyst, hydatid cyst and intraductal papillary neoplasm of the bile duct. Affecting middle-aged female population, BMCN presents variedly ranging from vague abdominal symptoms to obstructive jaundice. Preoperative diagnosis is difficult. Preoperative CT scan with intravenous contrast and carbohydrate antigen 19.9 levels may give a clue towards the diagnosis. Intraoperative frozen section or cyst fluid aspiration cytology might help confirm the diagnosis. Fine needle aspirations should not be performed if BMCN is suspected, and intraoperative cyst spillage should be avoided to prevent tumour dissemination. We present here a case with a very atypical presentation of BMCN and review its present literature in brief.

Benign biliary neoplasms and biliary tumor precursors

“…MCN shows either low grade or high grade epithelial dysplasia, and may be associated with an invasive carcinoma 4 , 7 . Most MCNs are solitary intrahepatic lesions, rarely seen in the extrahepatic bile ducts and in gallbladder 26 , 27 .…”

“…Patients typically show symptoms, which include chronic non-specific abdominal pain or discomfort and swelling, a palpable mass, and obstructive jaundice. In old patients, acute symptoms may suggest an invasive component 7 , 27 . Preoperative diagnosis may be challenging and requires a high degree of suspicion.…”

“…Preoperative diagnosis may be challenging and requires a high degree of suspicion. Serum CA19-9 levels may be increased, particularly in patients with an associated invasive component, while intracystic CA19-9 and carcinoembryonic antigen (CEA) levels may be of help in differential diagnosis with non-neoplastic lesions, since they are higher in MCN 27 , 30 , 31 . Abdominal imaging shows large multilocular cystic lesions, with cyst-in-cyst appearance, without any communication with the biliary tree, even though unilocular cystic lesions may be seen in about 10% of patients.…”

“…Abdominal imaging shows large multilocular cystic lesions, with cyst-in-cyst appearance, without any communication with the biliary tree, even though unilocular cystic lesions may be seen in about 10% of patients. Irregular thickness of the cystic wall, enhancing internal septa with mural nodules, and papillary projections suggest an invasive component 27 , 32 . Etiology of MCN remains unclear, even if its prevalence in middle-aged women point toward a hormonal influence 30 .…”

“…FNA samples usually contain aggregates of bland cuboidal to columnar epithelial cells, with occasional papillary arrangement. Different degrees of nuclear atypia might be observed, if a high-grade dysplastic or carcinomatous component is present 27 , 37 . The ovarian-type stromal component is typically not seen.…”