References | Clinical variables | Description |
---|---|---|
1–3 6–8 | Symptoms | Jaundice, nausea, vomiting, weight loss, intolerance to fatty food, cholangitis |
9 | Haematological tests | Elevated total bilirubin, AST, ALT, ALP |
9 | Serum tumour markers | Occasional elevated Ca 19.9 and or CEA |
1 2 4 11 12 | Endoluminal tumour markers | Levels of Ca 19.9 and CEA might be elevated in the fluid aspirated from the lumen of the cysts |
4 | Radiological findings: CT | Multi-loculated, multi-septated intrabiliary neoplasms. Average size at the time of diagnosis=15 cm. On CT scan, the content of the cyst is usually hypoattenuating. Calcifications within the cyst walls and septation have been described. Possible irregular wall enhancement |
10 | Radiological findings: MRI | T1 and T2 signal intensity of the intracystic fluid can be variable depending on the protein concentration and presence or absence of blood |
6 | Histology | Presence of cysts lined with mucinous cuboidal or columnar epithelium. Ovarian-type stroma present in 85% of cases, exclusively in females |
16 | Differential diagnosis | Hydatid cyst, biliary cystoadenocarcinoma, intraductal papillary mucinous tumour (IPMT) |
2–4 6 9–11 18 | Natural history | Malignant transformation into cystoadenocarcinoma or sarcoma has been described. Cystoadenomas with ovarian-type stroma have better prognosis |
ALP, alkaline phosphatase; ALT, alanine aminotransferase; AST, aspartate amino transferase; CA 19.9, carbohydrate antigen 19.9; CEA, carcinoembryonic antigen; GGT, γ-glutamyl transpeptidase.