Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. May 27, 2021; 13(5): 611-619
Published online May 27, 2021. doi: 10.4254/wjh.v13.i5.611
Acquired hepatocerebral degeneration in a metastatic neuroendocrine tumor long-term survivor — an update on neuroendocrine neoplasm’s treatment: A case report
Oriol Mirallas, Nadia Saoudi, Diego Gómez-Puerto, Mar Riveiro-Barciela, Xavier Merino, Cristina Auger, Stefania Landolfi, Laia Blanco, Amparo Garcia-Burillo, Xavier Molero, Maria Teresa Salcedo-Allende, Jaume Capdevila
Oriol Mirallas, Nadia Saoudi, Diego Gómez-Puerto, Jaume Capdevila, Medical Oncology Department, Vall d'Hebron University Hospital, Vall Hebron Institute of Oncology, Barcelona 08035, Catalunya, Spain
Mar Riveiro-Barciela, Liver Unit, Department of Internal Medicine, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona 08035, Catalunya, Spain
Xavier Merino, Cristina Auger, Radiodiagnostic Department, Vall d’Hebron University Hospital, Barcelona 08035, Catalunya, Spain
Stefania Landolfi, Maria Teresa Salcedo-Allende, Pathology Department, Vall d'Hebron University Hospital, Barcelona 08035, Catalunya, Spain
Laia Blanco, Xavier Molero, Hepatobiliopancreatic Surgery and Transplantation Department, Vall d'Hebron University Hospital, Barcelona 08034, Catalunya, Spain
Amparo Garcia-Burillo, Nuclear Medicine Department, Vall d'Hebron University Hospital, Barcelona 08035, Catalunya, Spain
Author contributions: Mirallas O, Saoudi N and Gómez-Puerto D were responsible for collecting the data, imaging, and writing the main text; Riveiro-Barciela M critically reviewed the text and helped write the acquired hepatocerebral degeneration text; Merino X and Auger C helped collect and describe the optimal images; Blanco L, Molero X, Salcedo-Allende MT, Landolfi S, Garcia-Burillo A, and Capdevila J are members of the multidisciplinary team of neuroendocrine tumors at Vall d’Hebron University Hospital who are taking care of patients, and critically reviewed and approved the manuscript; All authors read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and accompanying images.
Conflict-of-interest statement: Mirallas O, Saoudi N and Gómez-Puerto D declare that they have no competing interests funding related to this work.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to it.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Oriol Mirallas, BSc, MD, Occupational Physician, Medical Oncology Department, Vall d'Hebron University Hospital, Vall Hebron Institute of Oncology, Passeig de la Vall d'Hebron, 119, Barcelona 08035, Catalunya, Spain.
Received: January 2, 2021
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.

Keywords: Neuroendocrine tumors, Hepatocerebral degeneration, Parkinsonism, Somatostatin analogues, Everolimus, Hepatic metastases, Peptide radionuclide receptor therapy, Encephalopathy, Paramagnetic deposits, Case report

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.