By Jocelyn Selim
The Fairest of All
Audrey Hepburn died of appendix cancer at 63, leaving behind a legacy of style and good works
By Jocelyn Selim
After a round of routine tests at Cedars Sinai Hospital in Los Angeles revealed no cause for her pain, Hepburn’s physicians recommended laparoscopy—a procedure in which doctors insert a camera through a tiny incision in the abdomen. The exam found a tissue-paper-thin tumor encasing the last segment of her small intestine and the beginning of her large intestine; the medical team determined that the cancer had metastasized from her appendix.
A Diagnosis of Appendix Cancer
The appendix is a tiny, elongated sac about the width of a pencil that sits at the junction of the small and large intestines in the lower right abdomen. It has no confirmed function. Recent studies have suggested that it aids digestion, but biologists often accept it as the remnant of a much larger sac that allowed pre-human ancestors to digest the cell walls of plants.
Cancer of the appendix is extremely rare. “It’s sort of the poor orphan cousin of colon cancer,” says Edward Levine, a surgical oncologist at Wake Forest University School of Medicine in Winston-Salem, N.C. While colorectal cancer is diagnosed in roughly 150,000 people a year, cancers that start in the appendix are much less frequently diagnosed, with estimates ranging from a few hundred to 2,500 cases each year. Of those cases, one half or so are mostly harmless carcinoid tumors—cancers of the neuroendocrine, or hormone-producing, cells—that are found by chance when pathologists look at appendixes removed during routine appendectomies or other surgeries.
More serious are the minority of appendix cancers—roughly 30 percent, according to the American Society of Clinical Oncology—that are classified as adenocarcinomas. These start in cells that typically line the inside of organs, where they produce mucus and other fluids. Although scarce among appendix cancers, adenocarcinomas make up the vast majority, or about 95 percent, of colorectal cancers. Surprisingly, while they arise from the same type of cell as colon adenocarcinomas, appendix adenocarcinomas often behave dramatically differently from colon adenocarcinomas, for reasons oncologists still don’t understand.
Hepburn’s cancer was an adenocarcinoma. The tumor was strangling her intestines, creating an obstruction that was causing the intense pain she felt. There is no official staging system to describe the spread of most appendix cancers, but because the tumor had invaded Hepburn’s abdomen and peritoneum—the thin covering that lines the organs and separates the abdominal cavity from the rest of the body—her cancer would have been considered very advanced by any standard.
Adenocarcinomas that start in the appendix are much more likely than typical colon cancers to have what doctors call a “mucinous histology”—meaning that when the cells lining the intestine turn cancerous, they make excess amounts of the jellylike secretions they normally produce, says Jesus Esquivel, a surgical oncologist at St. Agnes Hospital in Baltimore. “A doctor will maybe see one of these cases over the entire course of his career,” he says. “I hear about cases where doctors open up a patient, take one look and think, ‘I really don’t know what this is,’ and then close the patient back up.”
Hepburn called the disease “abominable”—rather than “abdominal”—cancer, recalls Ferrer. On Nov. 1, 1992, just days after entering the hospital, his mother underwent surgery. The doctors removed about a foot of her intestine. A week later, she was started on intravenous fluorouracil (5-FU) and leucovorin, a drug combination that is still widely used to treat colon cancer today.