Show - rr I A30 The Salt Lake Tribune Sunday June 9 1991 11 A Tragic Family Affair: Researchers Probe Genetic Link to Cancer Continued From 75 percent of all cases A minority of tumors are familial cancers They might be due to a shared environmental factor or the interaction of a genetic predisposition and environment Or they might be the very rare hereditary cancers The average age of onset for most colorectal cancers is between age 61 and 63 and the tumors are considered sporadic Creighton's Lynch has identified about 50 American families that have a rare hereditary form of colon and endometrial cancers — known collectively as the Lynch syndromes I and II For these families colon cancer strikes on average at age A-2- ra 4 t'Ir' 14 t1 E George Weihe of Louisville Weihe had known cancer was frequent among his relatives his mother died of uterine cancer When his children were growing up he and his wife took care to inform physicians that there was a family history of cancer "Some would look at you like this has no relevance it's so rare why are you telling me this?" recalled his wife Joann Weihe Then at age 20 Weihe's oldest son became suddenly ill The diagnosis: colon cancer Weihe's son died seven months later Weihe's older brother died of colon cancer a few years after that and then Wcihe himself was stricken with cancer of the colon and urinary tract After Weihe's cousin was also diagnosed with cancer a doctor recalled an article he had read on Lynch syndrome which mentioned families suffering from colon urological and reproductive-systecancers The Weihes called Lynch who told the family that their cancer was definitely hereditary Joann Weihe has taken a survey of her husband's family Thirty of its 100 members have had cancer Creighton researchers say the Weihes are an extremely valuable resource because several members have survived the cancer their blood and live tissues can be combed for genetic clues and markers for the disease that could lead to better testing and treatment of others at risk Members of the Weihe family now undergo early screening tests including colonoscopies "We always live in fear Every time you look at one of your kids if they look pale or say they don't feel well you think is this it for him? 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'1i i T 411- I 'tit ' ' - iig I r factory model is ecogenetic in the direction of genes and the — admitting that we haNe-to learn about still lot ' cases in individual patients" ap- - - :' w "Now I think we've come 'to a middle ground that a more satis- 1114174 '41 ' - ' ' g' r- T I 2 ff--- -- 5 - fr't--- i ' i- - 'I--4- " ' e- - '- a' - - ---' x I genetic ly ' - if 4 4Ti f'AV - - 2' ' -- - - l it6 Avrtmolow- '"or'In' :5:::11 -7- ' i' - But the environmental ! dividuals At the level of the cell"" fe said "cancer is a genetic diseaseThe experimentalist sees how cancer cells pass on bad genetic information to daughter and son tens and thus regards cancer as entire- proach to cancer as Mulvihill points out fails to take into ac ' ' f fir g Ar 7' J: ''i ' -- i tri8u '1777T jit$ ' -0 'i t": 41 i- tt - 4 '''' - : ' i L 2 Y 1 1 it' 4 '" 1 ':41-4- 1 - - ' '''''7''''' jr-f E511 - - 4' 7to ' "1"i 11 7 I 55' 71r1 r i - 04- tg '' 4 - ' '5 -'- i - '"'t't - I' i 1 "' ''' - Genetics of course does not tell the whole story of cancer Mulvihill who has been focusing recently on pancreatic cancer and worked with former President Carter's family has done much to popularize the term ecogenetics — the interaction between environ 1:--- A :7:4 - --- --v it 1411 1 - 1 1 1 ri:tt4 -: '" - 1 4 r 4 414042: 1- - 1 - -- di t LI '1 I i v'tilo : 1 - r'' - of contracting cancer" said the sisters 11'041 center and e I 'a o14 - 24- it1 j 42 - 4' Joann consultation t ticiiit -- cancer-car- dants through four generations of her family ffp ri il 14 i r ha It 'VIA' k iI national a i count the genetic variability ot tr7-- ment and heredity Each cancer he says probably arises from a complex interaction of environmental exposures with varying susceptibilities on the part of the patient "The causes of any one person's cancer are usually uncertain "All of us" he said "are born with 100000 genes that interact with our peculiar life encounters with environment — lifestyle " drugs diet and radiations countered skepticism from doctors "She is 19 and I've had trouble getting her treated" she said "We've really had to go in armed to educate the physician" within 24 hours Wilkison was diagnosed as having advanced ovarian cancer After investigating census records she has turned up ovarian cancer in direct descen- - w- :74 1-- Among them is the family of - were so remote" A few months later in 1989 while vacationing in the state of Washington Wilkison was stricken with unbearable stomach pain The local hospital happened to be The story of Gilda Radners battle with ovarian cancer rang a bell with Roxanne Wilkison a medical-record- s manager in Milwaukee At 36 she went to her doctor with vague complaints of abdominal pain "I explained to him the history of my mother's ovarian cancer and I had an article out of the local newspaper on Gilda Radner and n Now she is trying to protect her daughter but has once again en- the chances of me getting cancer counselor told the sisters they probably each had a 50 percent chance of developing breast cancer at some time during their lives A 44 One of the very rare forms of hereditary cancer called the LiFraumeni cancer family syndrome is named for the two scientists — Frederick P Li and Joseph F Fraumeni Jr — who first described the phenomenon This syndrome also known as SBLA syndrome encompasses a range of cancers including sarcomas of the breast bone and brain tumors malignancies of the lung larynx and adrenal gland and leukemia Roughly 100 familleE worldwide have been identified with this rare hereditary cancer syndrome Women with syndrome are most likely to develop breast cancer before other tumors and they are providing scientists with important clues to the genetics of more common breast cancers Not all women are at the same risk of breast cancer a disease that has a tendency to run in families a fact first noted by the ancient Romans Researchers now recognize twyt) distinct diseases of breast cancer The common type affecting perhaps one in 14 American women over the course of a lifetime usually occurs some time after menopause and typically affects just one breast There is no distinct pattern in the family tree through two generations The type that is considered h e re dit a ry can strike before menopause and arise independently in both breasts A family pattern is evident often showing cases in "first degree" relatives: parents siblings or children This type of cancer accounts for only perhaps 7 percent of all breast tumors Yet breast cancer is so common — and the fear of the disease so widespread — that women tend to overestimate their familial risks of breast tumors The age at which cancer develops and the pathology of tumors are important in assessing an individuals risk Having a mother who developed breast cancer at age 65 does not automatically increase a daughter's risk Counselors at Johns Hopkins Oncology Center in Baltimore still talk about Jennifer Wilkins of Columbia Md and Kristen Mintlich of Baltimore two sisters chise in age who came to see them in 1988 after their mother bad sufferfA cancer in both breasts Before t hi he kind of laughed it off and said since" said Wilkins 9 1 |