Kiriyama Muneyasu, Ebata Tomoki, Yokoyama Yukihiro, Igami Tsuyoshi, Sugawara Gen, Mizuno Takashi, Yamaguchi Junpei, Nagino Masato
Division of Surgical Oncology, Department of Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, 466-8550, Japan.
Surg Case Rep. 2017 Dec;3(1):6. doi: 10.1186/s40792-016-0283-x. Epub 2017 Jan 4.
Mucin-producing cholangiocarcinoma (MPCC) is an uncommon tumour that is clinically characterized by mucin-hypersecretion. Because the initial symptoms of MPCC may be attributed to the viscus mucobilia, the primary tumour mass may potentially be unrecognizable. We report an interesting case of curatively resected occult MPCC in situ.
A 70-year-old man was referred to our hospital with increased levels of biliary enzymes. Multidetector row computed tomography (MDCT) demonstrated a diffuse dilatation of the entire biliary system without evidence of tumour mass. Additionally, there were numerous variably sized cysts throughout the liver. The cyst of S4 was the largest, followed by that of S1, which connected with the right hepatic duct. Endoscopic retrograde cholangiography showed intrabiliary mucus, predominantly in the left hepatic duct, but failed to show a communication of both cysts with the bile duct. We clinically suspected that minute MPCC was present within the S1 cyst and performed left hepatectomy, caudate lobectomy, and resection of the extrahepatic bile duct. Macroscopically, papillary adenocarcinoma in situ was present in the S1 cyst, and a final diagnosis of MPCC originating from the bile duct of the caudate lobe was made.
For MPCC, in practice, we should consider the possibility that this tumour can be occult. In this complicated setting, demonstrating the communication to the responsible dilated duct is a clue to the diagnosis. Multidirectional MDCT images succeeded in specifically demonstrating this communication, which is insensitive to the presence of excessive mucobilia.
产黏液胆管癌（MPCC）是一种罕见肿瘤，临床特征为黏液分泌过多。由于MPCC的初始症状可能归因于黏液性胆汁，原发性肿瘤肿块可能难以识别。我们报告一例原位隐匿性MPCC经根治性切除的有趣病例。
一名70岁男性因胆汁酶水平升高转诊至我院。多排螺旋计算机断层扫描（MDCT）显示整个胆道系统弥漫性扩张，未发现肿瘤肿块。此外，肝脏内有许多大小不一的囊肿。S4段的囊肿最大，其次是S1段的囊肿，其与右肝管相连。内镜逆行胆管造影显示胆管内有黏液，主要位于左肝管，但未显示两个囊肿与胆管相通。我们临床怀疑S1段囊肿内存在微小MPCC，并进行了左肝切除、尾状叶切除和肝外胆管切除。大体检查发现S1段囊肿内有原位乳头状腺癌，最终诊断为起源于尾状叶胆管的MPCC。
对于MPCC，在实际中我们应考虑到这种肿瘤可能是隐匿性的。在这种复杂情况下，显示与扩张胆管的相通是诊断的线索。多方向MDCT图像成功地特异性显示了这种相通，而对过多黏液性胆汁的存在不敏感。
