Intrahepatic bile duct cystadenoma D13.4

Intrahepatic bile duct cystadenoma and cystadenocarcinoma are very rare epithelial tumors of the liver.

Unsolved. Development from an "abnormal primitive hepatobiliary system". Thus, a congenital origin is assumed as one of the main theories (Davies W et al. 1995).

The cystadenomas basically occurred at any age. Mostly middle-aged women are affected (Kawashima et al. 1991). w:m=4: 1 The patient age of cystadenomas varies between 17 and 86 years (average age: 51 years). Only very rare occurrence in children and adolescents (Tran S et al. 2013).

The hepatobiliary cystic tumors often grow very slowly and have usually already reached enormous proportions before they become symptomatic. The appearance of symptoms and typical signs requires a cyst size of > 5 cm in diameter. Since the clinical signs are usually relatively mild and unspecific, a diagnosis is very difficult. In some cases patients complain of symptoms in the right upper abdomen. These are caused by a rapid increase in the size of the tumour or intracystic haemorrhage, which causes capsule tension in the liver. Most frequently, patients presented with a palpable tumor at the initial examination.

About 25% of intrahepatic localized cystic tumors are found in the right lobe of the liver, 50% in the left part of the liver and 25% in both liver lobes.

In about 20% of the patients increased laboratory parameters like direct and indirect bilirubin, alkaline phosphatase, GPT, GOT and glutamylpyruvate transferase (GPT). Individual patients show elevated tumor markers (CA 19-9), occasional positive carcinoembryonic antigen(CEA); AFP is negative.

Sonography is considered the method of first choice in the diagnosis of lesions of the liver, other parenchymatous organs and adjacent structures. The sonographic imaging allows a good morphological assessment of the liver lesion with intracystic septations, wall thickening and nodular mucosal growths. The echogenicity of the cyst content allows certain conclusions about the composition. Calcifications may be occasionally visible.

Endoscopic retrograde cholangiopancreatography (ERCP) can be used for anatomical imaging of the bile duct system. It is diagnostically useful in patients with biliary obstruction.

Cystic metastases, which can originate from adenocarcinomas of the ovary, small cell bronchial carcinomas, stomach carcinomas, chorionic and endometrial carcinomas or from sarcomas

Radical tumour resection is now considered the therapy of choice (Mantke et al. 2001). The "conservative" procedures, such as cyst drainage, fenestration or aspiration, marsupialisation or partial cyst resection are more likely to be considered palliative. There is a risk of tumour progression and the actual benign tumour has a tendency to relapse or possibly degenerate into a malignant cystadenocarcinoma (Asahara et al. 1999)

Cystadenomas tend to recur postoperatively, especially in cases of insufficient resection. The malignant potential of primarily benign tumors seems to be secured, but has so far only been documented by means of individual case descriptions. Development of a cystadenocarcinoma, even after latency periods of > 20 years possible.

1. Asahara T et al (1999) A case of biliary cystadenocarcinoma of the liver. Hiroshima J Med Sci 48:45-48.
2. Davies W et al (1995) Extrahepatic biliary cystadenomas and cystadenocarcinoma. Report of seven cases and review of the literature. Ann Surg 222: 619-625.
3. Filippi MM et al (1999) Biliary cystadenocarcinoma: sonographic and cytologic findings. J Clin Ultrasound 27: 210-212.
4. Kawashima A et al (1991) Biliary cystadenoma with intratumoral bleeding: radiologic-pathologic correlation. J Comput Assist Tomogr 15:1035-1038.
5. Mantke R et al (2001) Hepatobiliary cystadenoma. Surgeon 72: 277-280.
6. Tran S et al (2013) Hepatobiliary cystadenoma: a rare pediatric tumor. Pediatric Surgery Int 29:841-845.