Division of Gastroenterology and Hepatology, MOB 602, Department of Medicine, William Beaumont Hospital, 3535 West Thirteen Mile Road, Royal Oak, MI, 48073, USA.
Dig Dis Sci. 2014 Apr;59(4):724-36. doi: 10.1007/s10620-013-2943-z. Epub 2013 Dec 27.
To characterize syndrome of acute liver failure (ALF) from metastatic breast cancer to promote premortem diagnosis. Up to now, only 25 % of the reported 32 cases of this syndrome were diagnosed premortem.
Cases identified by computerized literature review and review of files maintained by senior investigator.
Among 32 cases, average age at presentation was 47.9 ± 9.9 years. Common signs include jaundice, hepatomegaly, shifting dullness, and bilateral leg edema. Mean serum level of AST was 296.4 ± 204.0 U/L, ALT, 183.2 ± 198.9 U/L; alkaline phosphatase, 641.5 ± 610.1; and total bilirubin, 8.6 ± 8.3 mg/dL. Twenty-seven patients (84 %) have known prior breast cancer (mean diagnosis = 4.1 + 4.8 years earlier). Abdominal ultrasound findings (N = 10) include hepatomegaly in three cases, heterogeneous/multifocal hepatic lesions in three, ascites in three, and other in two. Abdominal CT findings (N = 16) include heterogeneous/multifocal hepatic lesions in six cases, ascites in five, hepatomegaly in three, cirrhosis in three, fatty liver in two, other in two. Hepatic metastases may not be suspected when abdominal CT shows no hepatic lesions. The diagnosis is made postmortem in 24 cases and antemortem in eight, with a statistically significant trend of increasing premortem diagnosis since 2000 (0 % before 2000 vs. 50 % after 2000; p = .001, 95 %--ORCI ≥ 2.86, Fisher's exact test). A new case of ALF from breast cancer is reported with notable features: abdominal CT revealed no discrete hepatic lesions despite widespread hepatic metastases demonstrated by liver biopsy; hepatic metastases occurred 21 years after original breast primary; and original diagnosis of lobular breast cancer in primary lesion was corrected to mixed ductal and lobular carcinoma, based on immunohistochemistry, performed 21 years afterward.
This review characterizes the clinical presentation and natural history of this syndrome to promote liver biopsy for premortem diagnosis and appropriate therapy.
对转移性乳腺癌所致急性肝衰竭（ALF）的综合征进行特征描述，以促进生前诊断。到目前为止，该综合征仅报道的 32 例中有 25%被生前诊断。
通过计算机文献检索和高级研究员档案回顾识别病例。
32 例患者中，平均起病年龄为 47.9 ± 9.9 岁。常见体征包括黄疸、肝肿大、移动性浊音和双侧下肢水肿。平均血清天冬氨酸转氨酶（AST）水平为 296.4 ± 204.0 U/L，丙氨酸转氨酶（ALT）为 183.2 ± 198.9 U/L；碱性磷酸酶为 641.5 ± 610.1 U/L，总胆红素为 8.6 ± 8.3 mg/dL。27 例（84%）患者有已知的既往乳腺癌病史（平均诊断时间为 4.1 ± 4.8 年前）。10 例（31%）患者行腹部超声检查，3 例有肝肿大，3 例有肝内多发病灶，3 例有腹水，2 例有其他表现。16 例（47%）患者行腹部 CT 检查，6 例有肝内多发病灶，5 例有腹水，3 例有肝肿大，3 例有肝硬化，2 例有脂肪肝，2 例有其他表现。当腹部 CT 无肝内病变时，可能会漏诊肝转移。24 例为尸检诊断，8 例为生前诊断，自 2000 年以来，生前诊断的比例呈上升趋势（2000 年前为 0%，2000 年后为 50%；p =.001，95%可信区间下限≥2.86，Fisher 确切概率法）。本文报道了 1 例新的乳腺癌所致 ALF 病例，其具有显著特征：尽管肝脏活检显示广泛肝转移，但腹部 CT 未显示离散肝病变；肝转移发生于原发性乳腺癌 21 年后；基于免疫组化，术后 21 年修正原发性病变的乳腺小叶癌为混合性导管和小叶癌。
本综述描述了该综合征的临床表现和自然病程，以促进生前诊断和适当治疗的肝活检。
