Wilson’s disease is associated with heavy copper overload, primarily in the liver.
Copper is a toxic metal, and therefore might be expected to be associated with cancer induction, as iron is in haemochromatosis.
The frequency of intra-abdominal malignancies has not previously been reported in patients with Wilson’s disease.
Doctors in Sweden and the UK conducted a retrospective study of 363 patients with Wilson’s disease to assess the long-term risk of intra-abdominal malignancy.
The research team grouped the patients by length of follow-up: 10–19 years; 20–29 years; 30–39 years; and 40 years or more.
The frequency of intra-abdominal malignancies was 4.2% for patients followed up for 10–19 years.
This rose to 5.3% in patients followed up for 20-29 years and to 15.0% in patients followed for 30-39 years.
No cancers were seen in the very small number of patients followed for more than 40 years.
The reported cancers consisted of hepatomas, cholangiocarcinomas, and poorly differentiated adenocarcinomas of undetermined primary site.
The authors of the study conclude. “Patients with Wilson’s disease appear to be vulnerable to the formation of aggressive malignant intra-abdominal tumours during long-term follow-up”
They also note that. “Ultrasound scanning of the abdomen seems to be a useful screening procedure.”
|15% of patients with Wilson’s disease who were followed-up for 30-39 years developed intra-abdominal malignancies.|
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