Published online May 27, 2021. doi: 10.4254/wjh.v13.i5.611
Peer-review started: January 2, 2021
First decision: January 18, 2021
Revised: February 4, 2021
Accepted: April 9, 2021
Article in press: April 9, 2021
Published online: May 27, 2021
Metastatic small bowel low-grade neuroendocrine tumors (NETs) have a good prognosis. Surgery is the only curative treatment; however, this may induce advanced liver disease, particularly in long-term survivor patients. Acquired hepatocerebral degeneration or Parkinsonism in cirrhosis is characterized by rapidly progressive extrapyramidal symptoms in patients with advanced liver disease.
A 70-year-old man presented to the emergency department with diminished consciousness and disorientation, and was diagnosed with hepatic encephalopathy. The patient was diagnosed in 1993 with a metastatic small bowel NET, for which he twice underwent hepatic surgery, with metastatic resection in 1993 and a right hepatectomy in 2002 to remove two hepatic metastases. In 2003, the patient started first-line chemotherapy and in 2004 started the first of three consecutive biological treatments, followed by radio-molecular therapy, achieving stable disease for 14 years. Disease progression was identified and he underwent an endoscopic retrograde cholangiopancreatography. However, in 2019 advanced liver disease was identified. We diagnosed the development of acquired hepatocerebral degeneration, an unusual long-term side effect after multiple hepatic procedures.
The importance of regular and ongoing surveillance in long-term NET survivors who undergo hepatic procedures should be integrated into the therapeutic management plan, as some of these negative outcomes could be prevented.
Core Tip: To the best of our knowledge, this is the first case report of acquired hepatocerebral degeneration in a metastatic small bowel neuroendocrine tumor long-term survivor, an uncommon irreversible extrapyramidal neurodegenerative condition encountered in patients with cirrhotic chronic liver disease, and resulting in widespread cerebral, basal ganglia, and cerebellar damage.