Fibrolamellar Hepatic Tumor with Neurosecretory Features and Systemic Deposition of AA Amyloid

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 hepato-carcinoma (FLHC) with immunocytochemical and ultrastruc-tural 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.

Key Words:

• amyloidosis
• fibrolamellar hepatocarcinoma
• neuroendocrine carcinoma