Alberti N, Bechade D, Dupuis F, Crombe A, Neuville A, Debled M, Palussiere J, Buy X, Perez J-T, Desjardin M, Frulio N, Kind M
Department of Radiology, Institut Bergonié, Comprehensive Cancer Center, 229, cours de l'Argonne, 33000 Bordeaux, France.
Department of Oncology, Institut Bergonié, Comprehensive Cancer Center, 229, cours de l'Argonne, 33000 Bordeaux, France.
Diagn Interv Imaging. 2015 Jan;96(1):73-8. doi: 10.1016/j.diii.2014.11.003. Epub 2014 Nov 25.
Hepar lobatum carcinomatosum (HLC) is an exceptional acquired hepatic distortion which consists in irregularly lobulated hepatic contours seen in patients with known liver metastases, usually from breast carcinoma. We aimed to describe and analyze five similar cases of HLC resulting from metastatic mammary carcinoma in the liver and associated with rapid hepatic failure.
Five cases of HLC were investigated. Medical (including blood liver tests), radiological and histological data (2 cases) were collected and retrospectively analyzed. All patients were followed up for metastatic invasive ductal carcinoma of the breast and had a common pattern of treatment with combination of targeted therapies (bevacizumab, AVASTIN) and chemotherapy (paclitaxel, TAXOL).
All the patients showed rapid hepatic failure after a mean of 9 courses of bevacizumab/paclitaxel. In all cases, liver imaging revealed liver capsule retraction and an irregular lobular margin. An apparent tumor regression of all liver metastases was showed in two cases. Biopsies were consistent with sinusoidal obstruction syndrome (SOS) and, surprisingly, no tumoral cells were found.
Although rare, such an unusual pattern of liver metastasis may mimick acute cirrhosis and cause rapid hepatic failure in patients, despite possible apparent tumor regression on imaging. The etiology of this pathology is unclear, and may involve multiple pathogenic factors. Direct or indirect vascular injury plays an important role in the development of HLC.
分叶状癌性肝(HLC)是一种罕见的后天性肝脏变形,表现为已知有肝转移(通常来自乳腺癌)的患者肝脏轮廓呈不规则分叶状。我们旨在描述和分析五例由肝转移性乳腺癌导致并伴有快速肝功能衰竭的类似HLC病例。
对五例HLC病例进行了研究。收集并回顾性分析了医学资料(包括肝脏血液检查)、放射学资料和组织学资料(2例)。所有患者均因乳腺浸润性导管癌转移接受随访,且都采用了靶向治疗(贝伐单抗,阿瓦斯汀)和化疗(紫杉醇,泰素)联合的常见治疗模式。
所有患者在平均接受9个疗程的贝伐单抗/紫杉醇治疗后均出现快速肝功能衰竭。所有病例的肝脏影像学检查均显示肝包膜回缩和不规则的小叶边缘。两例患者所有肝转移灶均出现明显的肿瘤退缩。活检结果符合窦性阻塞综合征(SOS),令人惊讶的是,未发现肿瘤细胞。
尽管罕见,但这种不寻常的肝转移模式可能会模仿急性肝硬化,并导致患者快速肝功能衰竭,尽管影像学上可能出现明显的肿瘤退缩。这种病理状况的病因尚不清楚,可能涉及多种致病因素。直接或间接的血管损伤在HLC的发生发展中起重要作用。