Vassiliou Ioannis, Kairi-Vassilatou Evi, Marinis Athanasios, Theodosopoulos Theodosios, Arkadopoulos Nikolaos, Smyrniotis Vassilios
2nd Department of Surgery, Areteion University Hospital, Athens Medical School, Athens, Greece.
World J Surg Oncol. 2006 Oct 7;4:71. doi: 10.1186/1477-7819-4-71.
Biliary papillomatosis (BP) is a rare disease entity with a strong malignant potential. It is characterized by multiple papillary adenomas involving both the intrahepatic and extrahepatic biliary tree. BP was considered in the past to be a disease with low malignant potential. However, a current review of the English literature revealed a high rate of malignant occurrence of approximately 41% and histological analysis along with the expression pattern of mucin core proteins (MUC) and mucin carbohydrate antigens suggests that BP is a borderline or low grade malignant neoplasm with a high malignant potential.
A 68 year-old male patient was referred to our hospital due to the presence of sudden right upper quadrant abdominal pain, nausea and dark urine. Imaging workup demonstrated dilatation of the left hepatic duct without the presence of a space-occupying lesion. A left hepatectomy and cholecystectomy were carried out and histological analysis revealed a moderately to poorly differentiated carcinoma of the left hepatic duct in the background of biliary papillomatosis. Postoperative course was uneventful. Unfortunately, two years after initial diagnosis the patient rapidly deteriorated and died from multiple pulmonary secondary deposits.
BP should not be considered to be a benign disease. The clinical behavior, the high recurrence rate and the even higher malignant transformation occurrence, as well as the presence of carcinogenetic indicators (K-ras mutation, overexpression of p53, MUC and Tn antigens) strongly support that BP is a low-grade neoplasm with high malignant potential.
胆管乳头状瘤病（BP）是一种罕见疾病，具有很强的恶变潜能。其特征是肝内和肝外胆管树均出现多个乳头状腺瘤。BP过去被认为是一种恶变潜能较低的疾病。然而，近期对英文文献的综述显示，其恶变发生率高达约41%，组织学分析以及黏蛋白核心蛋白（MUC）和黏蛋白碳水化合物抗原的表达模式表明，BP是一种具有高恶变潜能的交界性或低级别恶性肿瘤。
一名68岁男性患者因突发右上腹疼痛、恶心和深色尿被转诊至我院。影像学检查显示左肝管扩张，未见占位性病变。实施了左肝切除术和胆囊切除术，组织学分析显示在胆管乳头状瘤病背景下左肝管存在中分化至低分化癌。术后过程顺利。不幸的是，初诊两年后患者病情迅速恶化，死于多处肺转移瘤。
不应将BP视为良性疾病。其临床行为、高复发率以及更高的恶变发生率，还有致癌指标（K-ras突变、p53过表达、MUC和Tn抗原）的存在，有力地支持了BP是一种具有高恶变潜能的低级别肿瘤。
