Kuo Chung-Mou, Changchien Chi-Sin, Wu Keng-Liang, Chuah Seng-Kee, Chiu King-Wah, Chiu Yi-Chun, Chou Yeh-Pin, Kuo Chung-Huang
Division of Gastroenterology, Department of Internal Medicine, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Taiwan.
Scand J Gastroenterol. 2005 Apr;40(4):455-9. doi: 10.1080/00365520510011551.
Mucin-producing cholangiocarcinoma (MPCC) is an uncommon biliary tract malignancy. This retrospective study was designed to investigate the clinical presentations, associated diseases, the value of tumor markers, and treatment and prognosis of MPCC.
The study included 24 MPCC cases (7 M, 17 F) from 132 histopathology-proven cholangiocarcinoma patients seen between 1988 and 2003. MPCC and non-mucin-producing cholangiocarcinoma (non-MPCC) were compared.
Common clinical symptoms and signs included epigastric or right upper quadrant pain, fever and chills, jaundice, and anemia. Seventeen patients had mucobilia in surgical, cholangiographic, or CT findings. Biliary stones were found in 71% of MPCC patients. In detecting MPCC, a serum CA-19-9 level > 120 U/ml had 73% sensitivity, 41% specificity, 25% positive predictive value, 85% negative predictive value, and 48% accuracy in the 132 cholangiocarcinoma patients. The 1-, 2-, 3-, 4-, and 5-year survival rates were 44.3%, 33.8%, 28.1%, 21.1%, and 21.1%, respectively. Univariate analysis showed that tumor type (MPCC) was the only significant factor predicting survival when compared with non-MPCC patients in 5-year cumulative survival rates (21.1% versus 5.4%). MPCC patients receiving surgical resection had a prolonged median survival time (13 months versus 6 months) than those without resection.
MPCC clinical presentations were similar to non-MPCC in Taiwan except for more jaundice, fever and chills. The major factor predisposing to MPCC was biliary stones. Using serum CA19-9 levels greater than 120 U/ml was a diagnostic aid to detecting MPCC patients. MPCC patients had a better prognosis than non-MPCC patients. The recommended treatment for MPCC is surgical resection.
产黏液性胆管癌（MPCC）是一种罕见的胆道恶性肿瘤。本回顾性研究旨在探讨MPCC的临床表现、相关疾病、肿瘤标志物的价值以及治疗和预后情况。
本研究纳入了1988年至2003年间132例经组织病理学证实的胆管癌患者中的24例MPCC病例（男性7例，女性17例）。对MPCC和非产黏液性胆管癌（非MPCC）进行了比较。
常见的临床症状和体征包括上腹部或右上腹疼痛、发热寒战、黄疸和贫血。17例患者在手术、胆管造影或CT检查中发现有黏液胆汁。71%的MPCC患者发现有胆结石。在132例胆管癌患者中，血清CA-19-9水平>120 U/ml检测MPCC时，敏感性为73%，特异性为41%，阳性预测值为25%，阴性预测值为85%，准确性为48%。1年、2年、3年、4年和5年生存率分别为44.3%、33.8%、28.1%、21.1%和21.1%。单因素分析显示，与非MPCC患者相比，在5年累积生存率方面（21.1%对5.4%），肿瘤类型（MPCC）是预测生存的唯一显著因素。接受手术切除的MPCC患者中位生存时间（13个月对6个月）比未切除的患者延长。
在台湾，MPCC的临床表现与非MPCC相似，只是黄疸、发热寒战更多见。MPCC的主要诱发因素是胆结石。血清CA19-9水平大于120 U/ml有助于检测MPCC患者。MPCC患者的预后比非MPCC患者好。MPCC推荐的治疗方法是手术切除。
