Appendix Tumor Patient Perseveres
CancerWise - April 2007
By Lisa Garvin
Becky Bowden has a 14-inch scar as a souvenir of her encounter with a rare tumor of the appendix. “It’s my big red badge of courage, and I’m actually proud of it,” she says. “It reminds me of what I’ve been through.”
Bowden, a 56-year-old medical technologist from Houston, is one of the estimated 1,000 people each year in the United States who are diagnosed with pseudomyxoma peritonei, or PMP, a condition caused by a tumor originating from the appendix. PMP is commonly misdiagnosed, mistreated and misunderstood by physicians.
Part of the confusion, even among oncologists, stems from the unique behavior of the tumor cells responsible for PMP, says Laura Lambert, M.D., an assistant professor in M. D. Anderson’s Department of Surgical Oncology.
PMP comes from mucin-producing tumor cells originating in the appendix. Unlike other cancers, PMP tumor cells rarely spread to other parts of the body. Instead, they break free of the appendix and spread around the abdomen where they grow and produce a gelatinous material – PMP. If left untreated, the tumor cells and PMP eventually lead to life-threatening bowel obstruction.
PMP affects entire abdomen
The appendix is lined with cells that normally produce mucin, a substance that is released into the colon and mixes with stool. If the opening from the appendix to the colon is blocked by a tumor, the mucin builds up within the appendix.
“Eventually, the wall of the appendix gets thin like an overstretched balloon, and the mucin and mucin-producing cells escape into the abdomen,” Lambert says. “The cells stick to almost every surface within the abdomen, including the liver, gallbladder, spleen, diaphragm, and the ovaries and uterus in women.”
The resulting tumor and mucin (which looks like thick jelly) can weigh more than 40 pounds.
PMP mimics other diseases, is frequently misdiagnosed
Like most patients with PMP, Bowden’s quest for an accurate diagnosis was frustrating. “In 2004, I found a small lump on my abdomen that I thought was an ovarian cyst,” she says. “By the time I went to the doctor and had an ultrasound, the tumor was six pounds. I looked pregnant.”
Cat scans before the surgery showed that the tumor might be ovarian in origin, but during surgery the primary source was found to be the appendix.
Bowden was luckier than most because she was diagnosed with PMP quickly. Many patients undergo multiple surgeries and even chemotherapy before receiving the correct diagnosis. Surgeons removed tumors from both ovaries and the tip of her appendix. Later, she would learn that not all of the tumor cells were found and removed during her operation.
After the surgery, Bowden and her husband Robert did some research on the disease. “We had never heard of PMP, and my surgeon had never seen anything like it in 40 years of experience,” she says. “We didn’t find much information, but we knew that it was rare and the treatment unique.”
It was recommended that following surgery she undergo systemic chemotherapy (chemotherapy administered through the bloodstream, affecting the entire body). Her research showed that this type of traditional chemotherapy is ineffective in treating PMP.
Bowden then referred herself to M. D. Anderson to be treated by Paul Mansfield, M.D., a professor in the Department of Surgical Oncology, who she heard was an expert in treating PMP.
Treatment, recovery is demanding
PMP patients have coined a term for their treatment – MOAS, the Mother of All Surgeries. “It’s a hard surgery, and you have to get yourself ready for the run of your life,” says Bowden, who spent 47 days in the hospital on a feeding tube.
The procedure, which usually takes 8-12 hours, requires an incision from the tip of the sternum to the pubic bone. Surgeons must peel the tumor off the internal organs or remove them all together.
Then comes the part that Bowden likes to call “shake and bake.” The abdominal cavity is filled with mitomycin C, a chemotherapy drug heated to 41 degrees Celsius (106º F). For 90 minutes, surgeons rock the patient back and forth by hand to ensure that the drug coats the entire abdomen and destroys any lingering tumor cells.
Recovering from PMP surgery is tough on both patient and caregiver. “Dr. Mansfield warned me that I wouldn’t feel human for at least three months,” Bowden says. She required home health care and had to be fed intravenously by her husband, who she calls “nurse number one.”
“God’s going to give him stars in heaven for the way he took care of me,” she says. “I think I had an easier time than he did!”
Bowden returned to work fulltime five months after surgery, but still suffered from bouts of extreme fatigue for several more months. Full recovery can take up to a year.
From patient to crusader
The couple’s experience has turned them into passionate crusaders for increased awareness of PMP. After hearing horror stories from patients they met in the hospital and on the Internet, “we realized how many people weren’t getting to the right doctor, and we wanted to change that,” Bowden says.
The couple got involved with online groups like PMP Pals and the PMP Belly Button Club and will talk about PMP to anyone who will listen.
“If we can get patients and doctors to be more aware of this disease and the unique treatment it requires, we can get them help.”
CancerWise - April 2007
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- Q&A: Treatment for Rare Appendix Tumor
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