Quigley Brian, Reid Michelle D, Pehlivanoglu Burcin, Squires Malcolm H, Maithel Shishir, Xue Yue, Hyejeong Choi, Akkas Gizem, Muraki Takashi, Kooby David A, Sarmiento Juan M, Cardona Ken, Sekhar Aarti, Krasinskas Alyssa, Adsay Volkan
Departments of Pathology.
Surgery.
Am J Surg Pathol. 2018 Jan;42(1):95-102. doi: 10.1097/PAS.0000000000000963.
The literature is highly conflicting on hepatobiliary mucinous cystic neoplasms (MCNs), aka "hepatobiliary cystadenoma/cystadenocarcinoma," largely because ovarian stroma (OS) was not a requirement until WHO-2010 and is not widely applied even today. In this study, MCNs (with OS) accounted for 24 of 229 (11%) resected hepatic cysts in one institution. Eight of the 32 (25%) cysts that had been originally designated as hepatobiliary cystadenoma/cystadenocarcinoma at the time of diagnosis proved not to have an OS during this review and were thus re-classified as non-MCN. In total, 36 MCNs (with OS) were analyzed-24 from the institutional files and 12 consultation cases. All were women. Mean age was 51 (28 to 76 y). Mean size was 11 cm (5 to 23 cm). Most (91%) were intrahepatic and in the left lobe (72%). Preoperative imaging mentioned "neoplasm" in 14 (47%) and carcinoma was a differential in 6 (19%) but only 2 proved to have carcinoma. Microscopically, only 47% demonstrated diffuse OS (>75% of the cyst wall/lining); OS was often focal. The cyst lining was often composed of non-mucinous biliary epithelium, and this was predominant in 50% of the cases. Degenerative changes of variable amount were seen in most cases. In situ and invasive carcinoma was seen in only 2 cases (6%), both with small invasion (7 and 8 mm). Five cases had persistence/recurrence, 2 confirmed operatively (at 7 mo and 15 y). Of the 2 cases with carcinoma, one had "residual cyst or hematoma" by radiology at 4 months, and the other was without disease at 3 years. In conclusion, many cysts (25%) previously reported as hepatobiliary cystadenoma/cystadenocarcinoma are not MCNs. True MCNs are uncommon among resected hepatic cysts (11%), occur exclusively in females, are large, mostly intrahepatic and in the left lobe (72%). Invasive carcinomas are small and uncommon (6%) compared with their pancreatic counterpart (16%). Recurrences are not uncommon following incomplete excision.
关于肝胆黏液性囊性肿瘤（MCNs），又称“肝胆囊腺瘤/囊腺癌”，文献中的观点存在很大冲突，这主要是因为直到2010年世界卫生组织（WHO）才将卵巢间质（OS）作为该肿瘤的必要诊断条件，且即便在今天这一标准也未得到广泛应用。在本研究中，某机构切除的229个肝囊肿中有24个（11%）为MCNs（伴有OS）。在此次回顾性研究中，最初诊断为肝胆囊腺瘤/囊腺癌的32个囊肿中有8个（25%）被证实没有OS，因此被重新归类为非MCN。总共分析了36个MCNs（伴有OS），其中24个来自该机构的病例档案，12个为会诊病例。所有病例均为女性。平均年龄为51岁（28至76岁）。平均大小为11厘米（5至23厘米）。大多数（91%）为肝内囊肿，且位于左叶（72%）。术前影像学检查显示14个（47%）有“肿瘤”，6个（19%）鉴别诊断为癌，但最终仅2个被证实为癌。显微镜下，仅47%显示弥漫性OS（>囊肿壁/内衬的75%）；OS通常为局灶性。囊肿内衬通常由非黏液性胆管上皮组成，在50%的病例中占主导。大多数病例可见不同程度的退行性改变。仅2例（6%）发现原位癌和浸润性癌，两者浸润范围均较小（7毫米和8毫米）。5例出现持续存在/复发，2例经手术证实（分别在7个月和15年）。2例癌患者中，1例在4个月时影像学检查显示有“残留囊肿或血肿”，另1例在3年时无疾病。总之，许多先前报告为肝胆囊腺瘤/囊腺癌的囊肿并非MCNs。真正的MCNs在切除的肝囊肿中并不常见（11%），仅发生于女性，囊肿较大，大多为肝内囊肿且位于左叶（72%）。与胰腺MCNs（16%）相比，浸润性癌较小且不常见（6%）。不完全切除后复发并不罕见。
