Cholangiocystic adenocarcinoma is a malignant neoplasm of the liver of epithelial origin originating from the intrahepatic bile ducts and secreting mucus. Because of the rarity of the disease, the lack of obvious early symptoms, and the lack of accurate clinical adjuvant examinations (imaging features are not obvious), it is easily misdiagnosed as liver cyst, intrahepatic bile duct cystadenoma, and so on. Even performing liver cyst puncture and drainage delays the diagnosis and treatment of the disease, and increases the risk of tumor metastasis.
The etiology of intrahepatic bile duct cyst adenocarcinoma is unknown, and the possible pathogenesis includes: 1) carcinoma of the intrahepatic bile duct; 2) carcinoma of the intrahepatic bile duct cyst adenoma; and 3) congenital development of intrahepatic bile duct malformations, such as hepatic cysts and congenital cystic dilatation of the intrahepatic bile duct. The disease starts insidiously and has no obvious clinical manifestations in the early stage; as the disease progresses, epigastric fullness, hidden pain and epigastric masses may appear. The enlargement of the mass can lead to compression symptoms, compression of the vena cava to lower limb edema, compression of the bile ducts can cause jaundice, and combined infection can lead to abdominal pain and fever.
In terms of ancillary tests, intrahepatic bile duct cyst adenocarcinoma has some diagnostic value in the presence of elevated CA-199 in serum or cyst fluid. On ultrasound, CT or MR examination, intrahepatic cystic tumors with multiple chambers, thickened cystic wall intervals, inward formation of papillary protrusions, uneven cystic wall thickness, or even calcified foci should be considered if they are found.
Surgical resection is the most effective treatment for intrahepatic biliary cystadenocarcinoma, but care should be taken intraoperatively to avoid cyst rupture and leakage of cyst fluid into the abdominal cavity leading to implantation and metastasis.