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具有神经分泌特征及AA淀粉样蛋白全身沉积的纤维板层型肝肿瘤。

Fibrolamellar hepatic tumor with neurosecretory features and systemic deposition of AA amyloid.

作者信息

Lloreta J, Vadell C, Fabregat X, Serrano S

机构信息

Servei de Patologia, Hospital Universitari del Mar, Barcelona, Spain.

出版信息

Ultrastruct Pathol. 1994 Jan-Apr;18(1-2):287-92. doi: 10.3109/01913129409016302.

DOI:10.3109/01913129409016302
PMID:8191640
Abstract

A 28-year-old man presented with left cervical lymph node metastases and a 7-cm mass in the left lobe of the liver. Biopsy material from both sites revealed a fibrolamellar hepatocarcinoma (FLHC) with immunocytochemical and ultrastructural evidence of neurosecretory differentiation. The patient refused treatment. He died 6 years after the onset of symptoms with grade IV coma and bilateral bronchopneumonia. Postmortem examination disclosed persistent neoplastic disease in the liver and left lateral cervical lymph nodes as well as widespread deposition of AA amyloid in tumor stroma and in blood vessel walls of many tissues but mainly in the kidney, gastrointestinal tract, and heart. This appears to be the first report documenting the association of FLHC and amyloid deposition in the English literature.

摘要

一名28岁男性患者出现左颈部淋巴结转移及肝脏左叶7厘米肿物。两处活检材料均显示为纤维板层型肝癌(FLHC),免疫细胞化学及超微结构检查有神经分泌分化的证据。患者拒绝治疗。症状出现6年后,他死于IV级昏迷和双侧支气管肺炎。尸检发现肝脏和左侧颈外侧淋巴结有持续性肿瘤病变,且AA淀粉样蛋白广泛沉积于肿瘤基质及许多组织的血管壁,主要是肾脏、胃肠道和心脏。这似乎是英文文献中首篇记录FLHC与淀粉样蛋白沉积相关性的报道。

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Fibrolamellar hepatic tumor with neurosecretory features and systemic deposition of AA amyloid.具有神经分泌特征及AA淀粉样蛋白全身沉积的纤维板层型肝肿瘤。
Ultrastruct Pathol. 1994 Jan-Apr;18(1-2):287-92. doi: 10.3109/01913129409016302.
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Fatal Subacute Hepatic Failure in a Patient with AA-Type Amyloidosis: Case Report.1例AA型淀粉样变性患者发生致命性亚急性肝衰竭:病例报告
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