Audrey Hepburn’s Rare Cancer

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Audrey HepburnAudrey Hepburn was a movie star, ballet dancer, model and humanitarian who suffered such extreme starvation as a child during the Nazi occupation of Holland that she came out of World War II weighing only 88 pounds in a 5'6" frame.  She was extremely thin all her life.  She died at age 63 of a very rare cancer of her appendix.
 
She is one of the few performers to have won Oscar, Tony, Emmy and Grammy Awards, and also received three BAFTA Awards for Best British Actress.  The American Film Institute named her the third "Greatest Female Star of All Time."  The US Postal Service issued a commemorative stamp honoring her as a Hollywood legend and humanitarian.  She was also awarded the U.S. Presidential Medal of Freedom and the Academy of Motion Picture Arts and Sciences' Jean Hersholt Humanitarian Award in recognition of her work at UNICEF to help thousands of children in Africa, South America and Asia. 
 
Childhood From Privilege to Trauma
Hepburn was born in Belgium to wealthy parents and lived in Belgium, England and the Netherlands where she learned to speak five languages: Dutch, English, French, Spanish and Italian.  Several biographers describe her as being a chubby child. Her father was a British subject who became a strong Nazi sympathizer, and her mother was a Dutch noblewoman.  When Hepburn was six, her father abandoned the family, which hurt her greatly.  During World War II, when she was 9-16 years old, she and her mother were stuck in Nazi-occupied Holland and in 1944, the Nazis caused a "winter of hunger" in which they tried to kill the local population by starving them to death.  All they had available to eat were tulip bulbs, nettles and boiled grass. Her mother told her to drink water to feel full.  She developed asthma, liver disease and anemia.  She saw innocent people lined up against a wall and shot by the Nazis.  Her uncle was killed and her half-brother was deported to Berlin to work in a German labor camp.  She saw Jewish families being packed into trains taking them to concentration camps.   At war's end, She was 5’ 6″ and weighed 88 pounds.  After the war, she studied ballet, moved from Amsterdam to  London and performed as a chorus girl in musical theater.  
 

Her Thinness Helped to Make Her a Star
She won her breakthrough leading role in the Broadway play, Gigi, partly because of her waif-like thinness.  The producer of the show recommended that she prepare for the show by repeating her lines over and over on a cruise from London to New York.  At the end of the 18-day cruise, she had eaten so much chocolate that she was 15 pounds heavier than when she started the cruise.  The producer told her of his immense displeasure with her weight gain, and she responded by losing all of weight she had gained and more.  She smoked up to three packs of cigarettes per day, which would suppress hunger and prevent most people from gaining weight. She spent the rest of her life being very thin, often dropping well below 100 pounds. 
 
Her Illness and Death from a Cancer Called Pseudomyxoma Peritonei
In 1992 at age 62, she developed belly pain and her doctors found a cancer in her appendix called pseudomyxoma peritonei.  This is an extremely rare cancer that starts as a small polyp in the appendix. The cancer cells produce large amounts of mucous, called mucin, that slowly accumulate in the appendix until it bursts.  The cancer cells and mucous then spread throughout everything inside the belly to cause pain, constipation or diarrhea, loss of appetite and extreme tiredness.  Hepburn returned to her home to Switzerland and spent her last days at home.  On January 20, 1993, she died in her sleep. 
 
Nobody knows what causes this horrible cancer, as there are no known genetic, familial or environmental factors.  We do not know if severe food deprivation in childhood increased Hepburn's risk for dying from cancer later in life.   The literature shows that long-term calorie restriction can help to prevent diabetes, nerve damage and cancer in animals (Nature, 2012;489:318–321) and humans (Trends Pharmacol Sci, Feb, 2010;31(2):89–98) and sometimes can help to treat cancers in humans (Cancer Metab, Mar 7, 2013;1(10);  Recent Results Cancer Res, 2016;207:241-66).   However, severe calorie restriction in childhood was found to be associated with a significantly increased risk for breast cancer in the female survivors of the 1944 Dutch "Hunger Winter," which Hepburn lived through (Cancer Epidemiol Biomarkers Prev, 2005;14:1981–1985), as well as of the Jewish Holocaust (J Natl Cancer Inst, 2009;101:1489–1500) and the Siege of Leningrad (Int J Cancer, 2009;124:1416–1421).  There is also data to show that women who survived these brutal caloric restrictions in childhood suffer increased colon cancer risk as adults (International Journal of Epidemiology, April 1, 2017;46(2):612–621).  Smoking is a strong known risk factor for many cancers, including colon cancer, and Hepburn was a life-long heavy smoker.  Her cancer, pseudomyxoma peritonei, is in the same family of cancers as colon cancer.  
 

Audrey Hepburn's Legacy
In 1989, Hepburn was appointed a Goodwill Ambassador of UNICEF. On her appointment, she said that she was grateful for receiving international aid after enduring the German occupation as a child, and wanted to show her gratitude to the organization.  She received 17 major awards for her humanitarian work in addition to her numerous awards for acting.  She also received the Lifetime Style Award from the Council of Fashion Designers of America, and was inducted into the International Best Dressed List's Hall of Fame.  Her son, Sean Ferrer, established the Audrey Hepburn Children's Fund after her death, and UNICEF's Audrey Hepburn Society has raised more than $100 million, recognizing her many years of service to the organization.   
 
May 4, 1929 – January 20, 1993