© 1999 by Oxford University Press
Journal of the National Cancer Institute, Vol. 91, No. 18, 1590,
September 15, 1999
© 1999 Oxford University Press
CORRESPONDENCE |
Re: Maternal Inheritance Pattern of Hereditary Pancreatitis in Patients With Pancreatic Carcinoma
Affiliations of authors: A. B. Lowenfels, Department of Surgery, New York Medical College, Valhalla; P. Maisonneuve, Division of Epidemiology and Biostatistics, European Institute of Oncology, Milan, Italy.
Correspondence to: Albert Lowenfels, M.D., Department of Surgery, New York Medical College, Munger Pavilion, Valhalla, NY 10595.
Since our original report (1), we now have 412 patients in the International Hereditary Pancreatitis Registry. Sixteen patients have developed biopsy-proven pancreatic cancer, with a parent of origin known for 11 patients—all paternal. Adding the two patients in the recent correspondence by Lerch et al. (2) makes 13 patients with a known parent of origin, of which 11 parents are paternal and two parents are maternal. Against an expected male-to-female parental distribution of 50 : 50, this is still statistically significant (P = .02). However, in our expanded database, we note that before 1960, when most of the pancreatic cancer patients were born, the male-to-female parent of origin ratio is 60 : 40, rather than 50 : 50. This slight difference may possibly be related to reduced fertility in females with hereditary pancreatitis married to normal males, compared with normal females married to males with hereditary pancreatitis. Similar fertility differences have been noted in females with much milder disorders (3). The probability of 11 paternal parents of origin against two maternal, if a 60 : 40 split is assumed, is about 0.06. We agree with Lerch et al. that hereditary pancreatitis patients with either a male or a female inheritance pattern are at risk for pancreatic cancer, but the risk appears to be higher in patients with a paternal inheritance pattern.
REFERENCES
1
Lowenfels AB, Maisonneuve P, DiMagno EP, Elitsur Y, Gates
LK Jr, Perrault J, et al. Hereditary pancreatitis and the risk of pancreatic cancer. International
Hereditary Pancreatitis Study Group. J Natl Cancer Inst 1997;89:442-6.
2
Lerch MM, Ellis I, Whitcomb DC, Keim V, Simon P, Howes N,
et al. Maternal inheritance pattern of hereditary pancreatitis in patients with pancreatic carcinoma
[letter]. J Natl Cancer Inst 1999;91:723-4.
3
Skjaerven R, Wilcox AJ, Lie RT. A population-based study of
survival and childbearing among female subjects with birth defects and the risk of recurrence in
their children. N Engl J Med 1999;340:1057-62.
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