Primary biliary cystadenocarcinoma perforating the duodenum and left intrahepatic biliary tree ‐mimicking a hydatid cyst

Abstract

We report the case of a 76-year-old woman with biliary cystadenocarcinoma perforating the left biliary tree and exhibiting intra-tumoral gas bubbles resulting from invasion of the duodenum. The clinical history included subfebrile temperatures of 3 months duration, and pains associated with an abdominal mass in the right upper quadrant. Blood tests showed leucocytosis, and radiological studies revealed the features of a partially calcified septated tumor with nodular components combined with multiple gas-fluid levels, mimicking an infected hydatid cyst. Intraoperative ultrasonography, cholangiography and frozen section histology were necessary to prove the malignant nature of this cystic tumor. Provided that complete resection with strict adherence to oncological precepts is possible, the prognosis of cystadenocarcinoma is better than in hepatocellular or cholangiocellular carcinoma.

Invasive biliary mucinous cystic neoplasm: a review

“…The invasive BMCN (then referred to as a ‘biliary cystadenocarcinoma’) was first described by Willis in 1943 and defined as an entity by Edmondson in 1958 as a multilocular lesion lined by columnar epithelium with an accompanying densely cellular (‘ovarian‐like’) stroma 11,12,17 . Over the 70 years since its definition, its exact incidence among malignant hepatic epithelial tumours has remained unknown, but has been reported to be as low as 0.41% 7,13,20–22 . This may actually be an underestimation as both non‐invasive and invasive BMCNs are being discovered with increasing frequency secondary to advances in abdominal imaging and as a result of growing awareness of the cysts themselves 10,15,23,24 .…”

“…This may actually be an underestimation as both non‐invasive and invasive BMCNs are being discovered with increasing frequency secondary to advances in abdominal imaging and as a result of growing awareness of the cysts themselves 10,15,23,24 . Of note, the incidence of simple hepatic cysts has been established by both computed tomography (CT) and autopsies to be 14–24% and to increase with age 20,23 …”

“…In addition to primary non‐invasive and invasive BMCNs, differential diagnosis should include hepatic abscess, haematoma, haemorrhagic cyst, simple congenital cyst, hydatid (echinococcal) cyst, polycystic disease, cystic hamartoma or post‐traumatic cysts, Caroli's disease, other neoplastic lesions such as undifferentiated embryonal sarcoma, cystic primary hepatocellular carcinoma, metastatic pancreatic or ovarian cystadenocarcinoma, intraductal papillary mucinous neoplasm biliary type (IPMN‐B) and hepatobiliary mesenchymal tumours (particularly biliary smooth muscle neoplasms), such as biliary leiomyoma, adenomyoma and primary hepatic leiomyosarcoma (Table 1). 4,13,20,24,25,31,32 …”

Management of Hepatobiliary Cystadenocarcinoma

“…The radiological appearance varies from unilocular to multilocular cystic masses with multiple satellite tumors, although the majority are single, multilocular and cystic [10, 11]. Furthermore, the septate cystic lesions with occasional calcifications, irregular walls and nodular components can often be confused for a hydatid cyst [12, 13]. For a practicing clinician, this is an important point that needs to be stressed especially in countries like India with a high incidence of echinococcal disease.…”

Primary hepatoma – guidelines for interdisciplinary treatment

“…Another rare form of hepatic malignancies is cystadenocarcinoma, which has to be suspected when hepatic cysts increase in size or show irregularities, thickening or papillary formations of their walls. If complete resection is possible, the prognosis is better than for hepatocellular or cholangiocellular carcinoma [19] Regional/local therapeutic procedures These techniques may decrease tumour mass or stop tumour growth. Further prospective clinical studies are needed to evaluate potential positive effects on survival.…”