Biliary Cystic Tumours with Mesenchymal Stroma

Abstract

Biliary Cystic Tumours with Mesenchymal Stroma Biliary cystadenoma and cystadenocarcinoma are rare cystic liver tumours arising from the bile ducts proximal to the hilum of the liver. The tumours have unique structure with ovarian-type mesenchymal stroma, leading to challenging considerations about their pathogenesis. The differential diagnosis is wide and further complicated by the lack of awareness about these neoplastic processes due to the rarity of the disease. Radical surgical treatment can be recommended whenever possible as it can result in prolonged survival.

A rare case of extrahepatic biliary mucinous cystic neoplasm in a middle‑aged woman: A case report

“…Additionally, the morphology of embryonic peritoneum in this specific location is characterized by activated cells that might reveal an evidence of relationship between gonads and developing liver, biliary tree and pancreas (16). These ectopic ovarian deposits might develop the tumors known as BMCNs by stimulating the proliferation of adjacent biliary or pancreatic ducts (18).…”

“…Only few cases of patients with BMCNs who were treated with chemoradiotherapy, have been described (15,24). In 2009, Vanags et al (18) reported the beneficial impact of systemic 5-fluoruracil-based chemotherapy in a patient diagnosed with recurrent and metastatic disease 41 months after the surgical resection of an invasive BMCN. In another case, as the patient was not candidate for major hepatectomy, hepatic arterial infusion of cisplatin was used (28).…”

Mucinous Cystic Neoplasms of the Liver and Extrahepatic Biliary Tract

“…"Central dot" sign is observed by CT. Bridges across the cavities are evident by MRI. Both these findings represent branches of portal vein embedded in connective tissue strands adjacent to and surrounded by dilated bile ducts [28].…”

“…Topics in the Surgery of the Biliary Tree gland and retroperitoneal space showing similar stroma [27]. During embryogenesis, ectopic ovarian rests might develop in the liver, along biliary tree, in the pancreas or retroperitoneal tissues and stimulate the proliferation of adjacent biliary or pancreatic ducts by synthesis of growth factors [9,28]. Indeed, during embryonic development, gonads initially are located directly under the diaphragm, dorsally to the liver and pancreatic tail, and only later they descend to the typical anatomic location seen in adults.…”

“…Radiological investigation is the mainstay of preoperative diagnostics in order to discriminate between simple liver cyst and mucinous cystic neoplasm. Estimates of the biological potential (benign versus malignant) and differential diagnostics with other cystic lesions, e.g., parasites, abscesses or cystic/necrotic metastases, represent other important tasks [28].…”