Q J Med 2003; 96: 657-662
© 2003 Association of Physicians
Abdominal malignancies in patients with Wilson’s disease
From the 1Department of Neurology, The Middlesex Hospital, London, UK, 2Department of Internal Medicine, University Hospital, Uppsala, Sweden, 3Department of Histopathology, Norfolk and Norwich Hospital, Norwich, UK, and 4Department of Pathology, Sahlgrenska University Hospital, Göteborg, Sweden
Received 20 February 2003 and in revised form 25 June 2003
Background: Wilson’s 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 Wilson’s disease on long-term follow-up.
Design: Retrospective study in two specialist Wilson’s disease clinics: Cambridge/London and Uppsala.
Methods: We reviewed the case records of 363 patients seen at three centres: Addenbrooke’s Hospital, Cambridge, 1955–1987; the Middlesex Hospital, London, 1987–2000; and the University Hospital, Uppsala, Sweden, 1966–2002. Patients were grouped by length of follow-up: 10–19 years; 20–29 years; 30–39 years; and 40 years or more.
Results: No cancers were seen in patients followed for <10 years. For patients in the 10–19 years group, the frequency was 4.2%; at 20–29 years, it was 5.3%; and at 30–39 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 Wilson’s 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.
Address correspondence to Dr J.M. Walshe, 58 High Street, Hemingford Grey, Huntingdon PE28 9BN. e-mail: penicillamine{at}waitrose.com
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