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- Title
Abdominal malignancies in patients with Wilsons disease.
- Authors
J.M. Walshe; E. Waldenstrm; V. Sams; H. Nordlinder; K. Westermark
- Abstract
Background: Wilsons disease is associated with heavy copper overload, primarily in the liver. Copper is a toxic metal, and might be expected to be associated with cancer induction, as iron is in haemochromatosis. However, liver cancer is currently believed to be extremely rare in this disease, and other intra-abdominal malignancies have not been reported.Aim: To assess the frequency of abdominal malignant disease in patients with Wilsons disease on long-term follow-up.Design: Retrospective study in two specialist Wilsons disease clinics: Cambridge/London and Uppsala.Methods: We reviewed the case records of 363 patients seen at three centres: Addenbrookes Hospital, Cambridge, 19551987; the Middlesex Hospital, London, 19872000; and the University Hospital, Uppsala, Sweden, 19662002. Patients were grouped by length of follow-up: 1019 years; 2029 years; 3039 years; and 40 years or more.Results: No cancers were seen in patients followed for <10 years. For patients in the 1019 years group, the frequency was 4.2%; at 2029 years, it was 5.3%; and at 3039 years, 15%. No cancers were seen in the 40+ years follow-up group. The cancers consisted of hepatomas, cholangiocarcinomas, and poorly differentiated adenocarcinomas of undetermined primary site.Discussion: Patients with Wilsons disease appear to be vulnerable to the formation of aggressive malignant intra-abdominal tumours during long-term follow-up, irrespective of treatment. Ultrasound scanning of the abdomen seems to be a useful screening procedure.
- Subjects
CANCER patients; ABDOMINAL cancer; HEPATOLENTICULAR degeneration; LIVER cancer
- Publication
QJM: An International Journal of Medicine, 2003, Vol 96, Issue 9, p657
- ISSN
1460-2725
- Publication type
Article
- DOI
10.1093/qjmed/hcg114