PMID: 9179058Jun 1, 1997Paper

Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population

Cancer
M E ZöllerL Angervall

Abstract

The development of malignant and benign tumors in patient with neurofibromatosis type 1 (NF1) was investigated in a long term follow-up study of 70 adult NF1 patients living in Göteborg, Sweden, on January 1, 1978. Their mean age at that time was 44 years (range, 20-81 years). The 70 NF1 patients had previously been investigated in a population-based study. The first part of this study involved a cancer registry study. The authors compared the number of tumors in the 70 NF1 patients reported to the Swedish Cancer Registry during the period 1978-1989 with the number of tumors expected in the general population by matching the incidence rates of the two populations specific to age, time of follow-up, and gender. The 95% confidence interval for the risk quotient between the risk to the patients and the risk to the general population was estimated. The second part of the study was a clinical pathologic follow-up study. All living patients were offered a clinical reexamination in 1990. All hospital records for all the NF1 patients were reviewed, and death certificates were also reviewed when available. Malignant tumors were reported to the Cancer Registry four times as often in the NF1 patient group as in the general population (95%...Continue Reading

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