Cancer that struck Farrah Fawcett is rare but deadly

MIAMI — After a lifetime of Hollywood success, actress Farrah Fawcett had the misfortune to die from one of the rarest malignancies, anal cancer.

It's a cancer that struck 5,070 Americans in 2008, compared with 40,740 cases of rectal cancer, 108,070 cases of colon cancer, 184,450 cases of breast cancer and 215,020 cases of lung cancer.

It's so rare, doctors say, that many caregivers don't routinely screen for it and many patients don't notice it until it reaches advanced stages.

"Early on, the patient often doesn't feel anything or know anything," said Dr. Michael Hellinger, colorectal surgeon at Mt. Sinai Hospital in Miami Beach. "As it advances, with rectal bleeding, a little lump, people sometimes think it's hemorrhoids."

That's tragic, because when anal cancer is caught early, before it has spread to lymph nodes, liver or lungs, the five-year survival rate is 82 percent, according to the American Cancer Society.

The survival rate drops to 60 percent if it has spread to surrounding lymph nodes, 20 percent if it spreads to lungs, liver or other organs. The anus is the opening at the lower end of the digestive tract through which solid waste is excreted. Above it is the rectum, then the colon.

Among patients under 50, anal cancer is more common in men; after 50, it is more common in women. It occurs more often in smokers, people who have many sexual partners, have receptive anal intercourse or have a weakened immune system, the American Cancer Society says.

Some doctors believe that means it's more common among men who have sex with men and have anal intercourse, Hellinger says. "But that's never been statistically proven. Studies have never been done to look at that."

Another cause can be chronic infection with the human papilloma virus, in both men and women. HPV can be spread by either straight or gay sex, Hellinger said. And while it can be slowed among women who take the HPV vaccine to avoid cervical cancer, the vaccine never has been studied in men, and men are not routinely vaccinated.

When anal cancer is diagnosed, the standard care is chemotherapy and radiation. Until the 1970s the standard treatment was a "radical abdominal perineal resection," Hellinger said, in which the entire rectum and anal canal are removed, requiring the patient to wear an external colostomy bag.

In her TV program, "Farrah's Story," videotaped by Fawcett and her friends and broadcast on NBC on May 15, she made two statements that have led some to question her decisions about her own care.

First, she says her UCLA doctors wanted to do the radical surgery after the cancer had spread to her lymph nodes and liver. Instead, she traveled to Germany and found a surgeon willing to remove the tumor without the wider operation.

Second, until the final months, she insisted that any chemotherapy she was given involved drugs that would not cause her famous hair to fall out.

Hellinger declined to speculate on those decisions.

"It's impossible to judge without more information," he said.

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AP-NY-06-25-09 1335EDT