Roberts Preston, Trout Andrew T, Dillman Jonathan R
Department of Radiology, Madigan Army Medical Center, Joint Base Lewis-McChord, Lakewood, WA, USA.
Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., MLC 5031, Cincinnati, OH, 45229, USA.
Pediatr Radiol. 2018 Jul;48(7):932-940. doi: 10.1007/s00247-018-4129-5. Epub 2018 Apr 19.
Published case series have described central hepatic macroregenerative nodules or masses as a common feature of Alagille syndrome. Our experience suggests this regenerative pattern can be seen more generally in cholangiopathic disorders.
To define the frequency of central regenerative tissue in Alagille syndrome and other cholangiopathic disorders and to describe the typical appearance of such regenerative tissue.
We conducted a retrospective study of CT and MR imaging performed in children and young adults with cholangiopathic disorders between January 2000 and June 2016. Two pediatric radiologists reviewed images in consensus for the presence and features of macroregenerative tissue. Tissue histopathology, when available, was retrieved from the medical record.
Of 226 patients with cholangiopathic disorders, 23% (52/226) had macroregenerative tissue, and this tissue was central in 96% (50/52). Tissue was well defined and mass-like in 38% (20/52). Regenerative tissue was most common among the subset of patients with Langerhans cell histiocytosis with hepatic involvement (71%, 5/7) and was identified in 43% (16/37) of patients with Alagille syndrome. Regenerative tissue was iso- to hyperintense on T1-weighted MR sequences in 96% (50/52) of cases and hypointense on T2-weighted MR imaging in 94% (48/51). Arterial phase hyperenhancement was present in only five patients (12% of 43), none of whom showed portal venous phase washout. Histopathology was available for 20 cases, all showing benign regenerative tissue.
Central mass-like regeneration appears to be a common regenerative pattern in cholangiopathic disorders and should not be mistaken for malignancy.
已发表的病例系列研究描述了肝中央大再生结节或肿块是阿拉吉耶综合征的常见特征。我们的经验表明，这种再生模式在胆管疾病中更为普遍。
确定阿拉吉耶综合征和其他胆管疾病中中央再生组织的发生率，并描述此类再生组织的典型表现。
我们对2000年1月至2016年6月期间患有胆管疾病的儿童和青年进行的CT和MR成像进行了回顾性研究。两名儿科放射科医生共同对图像进行了复查，以确定大再生组织的存在和特征。如有可用的组织病理报告，则从病历中获取。
在226例胆管疾病患者中，23%（52/226）有大再生组织，其中96%（50/52）的组织位于中央。38%（20/52）的组织边界清晰且呈肿块样。再生组织在肝受累的朗格汉斯细胞组织细胞增多症患者亚组中最为常见（71%，5/7），在43%（16/37）的阿拉吉耶综合征患者中也有发现。96%（50/52）的病例在T1加权MR序列上再生组织呈等信号至高信号，94%（48/51）在T2加权MR成像上呈低信号。仅5例患者（43例中的12%）动脉期有强化，且均无门静脉期廓清。有20例可获得组织病理学结果，均显示为良性再生组织。
中央肿块样再生似乎是胆管疾病中常见的再生模式，不应误诊为恶性肿瘤。
