Systemic cancer and the FAMMM syndrome
1990

Systemic Cancer and the FAMMM Syndrome

Sample size: 200 publication Evidence: moderate

Author Information

Author(s): W. Bergman, P. Watson, J. de Jong, H.T. Lynch, R.M. Fusaro

Primary Institution: University Medical Centre Leiden and Creighton University School of Medicine

Hypothesis

Is there an increased susceptibility to non-melanoma cancers in families with the FAMMM syndrome?

Conclusion

The study found evidence of increased susceptibility to systemic cancer, particularly digestive tract cancer, in a subset of FAMMM families.

Supporting Evidence

  • 43 extracutaneous cancers were reported among the 200 high risk family members studied.
  • Significant excess of digestive system cancer, especially pancreatic cancer, was found in the nine families.
  • Three of the nine families had a marked excess in numbers of systemic cancers.

Takeaway

Some families with a genetic condition called FAMMM are more likely to get certain types of cancer, especially in the digestive system.

Methodology

Clinical and genetic studies were performed on well-documented FAMMM families, including interviews and examinations for cancer occurrences.

Potential Biases

Potential bias in family selection and reporting of cancer diagnoses.

Limitations

The study may have biases due to family selection and the reliance on self-reported cancer diagnoses.

Participant Demographics

Families ascertained in a pigmented lesions clinic in the Netherlands.

Statistical Information

P-Value

p<0.001

Statistical Significance

p<0.001

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