cancer cells

‘Hope is not lost’ Mom beats rare appendix cancer

The TikTok video featuring a joyous Jessica Diaz resembles so many others of the same style: two attractive women showing off their dance moves as a Beyoncé tune undulates in the background.
Violet Diaz
City of Hope patient Violet Diaz

Only when captions begin to appear do you realize you’re watching a daughter’s tribute to her mother, Violet Diaz, dancing alongside her.

“My mommy is a warrior! She survived the mother of all surgeries! They removed eight organs … to get rid of the Stage 4 cancer! She’s now into month two of a four-month recovery!”

To date, the video has been viewed over 4 million times.

“After posting it, I had so many comments with people praying for her,” said Jessica. “I believe the relatability of cancer is what made the video get so many views.”

In just a few short seconds, the inspirational footage trumpets a triumph by Diaz over a cancer she didn’t know she had, a cancer few have even heard of.

Back in May 2022, Diaz, a 53-year-old real estate appraiser and online reseller from Rancho Cucamonga, California, was flying to Chicago when she began experiencing severe abdominal pain.

“I’m a very positive person,” she explained. “I normally dismiss pain. At first, I thought this was just appendicitis.”

Once on the ground, Diaz shared her suspicions at a nearby emergency room. Doctors gave her morphine. All it did was upset her stomach. When they followed up with a computed tomography scan, it became clear that this was not appendicitis.

“The doctor said I had two masses, one near an ovary, another near my liver,” she recalled. “And then he said, ‘I think it’s cancer. Possibly ovarian.’

“My husband and I just looked at each other, flabbergasted.”

A biopsy showed Diaz did not have ovarian cancer, but a much rarer malignancy: Stage 4 cancer of the appendix that had spread to other parts of her abdomen.

Mustafa Raoof
Mustafa Raoof, M.D., M.S., assistant professor, Division of Surgical Oncology, Department of Surgery

“Our best estimate is that there are about 1,500 cases of appendiceal cancer a year in the U.S.,” said surgical oncologist Mustafa Raoof, M.D., M.S., assistant professor in the Division of Surgical Oncology, Department of Surgery, at City of Hope and a recognized authority on appendiceal cancer. “That’s less than one percent of all gastrointestinal cancers.”

No one is certain why people get appendiceal cancer. In about 10% of cases, there is a mutation in germline (inherited) DNA, but its implications are not clear. Raoof’s laboratory is examining the genomic makeup of appendiceal cancer cells. For now, though, “We think the majority of cases are environment-related,” he said. “But there’s no specific risk factor, like smoking, nor any proven risk factor that you can minimize.”

Appendix cancer is hard to diagnose in its early stages. Standard screening tests for colon cancer don’t reach far enough to detect most appendiceal malignancies. This cancer also differs molecularly from colon cancer, and it behaves differently as well. The rarest type, epithelial appendiceal cancer, which grows from the cells that make up the lining of the appendix, leads to a buildup of mucin — a gel-forming component of mucus — inside the appendix. Eventually, the appendix may rupture, not an abrupt “pop” as in appendicitis, but a slower, gradual process in which the mucin oozes out and spreads throughout the abdomen, filling the belly and blocking organ function, as well as spreading cancer cells. Patients may not even realize it’s happening. Nor may most doctors.

“There’s a general lack of awareness,” said Raoof. “Some of my patients were told to lose weight. Others were told they had a hernia. The symptoms can be so gradual that the patient normalizes them — they think it’s just the result of aging or a bad diet.”

After absorbing the initial shock of the diagnosis, Diaz made a quick decision.

“The moment cancer was mentioned, I wanted immediately to return to California and get to City of Hope,” she said. She’d been there before, accompanying a friend undergoing treatment for colon cancer. “He kept saying how incredible the place was, and I was impressed because the whole campus was focused on people with cancer, and everyone was super friendly.”

She also knew which doctor to see.

“I went on a website for appendix cancer, and Dr. Raoof’s name came up as No. 1.” (ACPMP, the Appendix Cancer Research Foundation, lists Raoof as first among a dozen physicians in California.).

Raoof remembers their first meeting well.

“She was anxious, scared, distraught at the thought of having Stage 4 cancer,” he recalled. “She had lots of questions.”

He responded with a formula he uses with all his patients: a combination of compassion and clear, easy-to-understand information.

“In that situation,” he explained, “the patient isn’t thinking logically. You need to establish a rapport, make them feel comfortable, de-escalate, and explain that we have resources to try.

“The greatest thing that empowers patients is knowledge.”

Diaz describes that meeting as “fantastic. He walked into the room like we were friends. We connected immediately. He takes out a sheet of paper that describes the cancer and provided information in such a wonderful way. It couldn’t have been better explained.”

Working with pathologist and appendix cancer expert Rifat Mannan, M.D., Raoof determined that Diaz’s cancer — which may have been growing inside her for as long as seven or eight years — was the low-grade variety, a positive in her favor. Low-grade tumors grow less rapidly and are easier to treat.

If the appendix hasn’t ruptured, removing it surgically (along with some lymph nodes and possibly the right side of the colon) may be all the treatment that’s needed. But when mucin has spread into the abdominal cavity, more extensive surgery is required. So extensive, in fact, that the procedure has a nickname: MOAS — The Mother of All Surgeries — in which every organ affected by the cancer is taken out. In Diaz’s case, this included her appendix, ovaries, fallopian tubes, spleen and sections of her intestines. Raoof and gynecologic oncologist Stephen J. Lee, M.D., would work together in the operating room with an eye toward clearing out all the cancer while saving as much of the anatomy as possible.

But that’s only the first step. With the patient still on the table, after all areas of cancer are removed, heated chemotherapy drugs are pumped into the abdomen to eradicate any remaining cancer cells. “HIPEC [hyperthermic intraperitoneal chemotherapy] is the long-established standard of care for low-grade appendiceal cancer to treat the microscopic cells,” Raoof explained. The benefit of HIPEC is that chemotherapy can be targeted to the abdominal cavity, an area difficult to reach for standard chemotherapy. Plus, a much more powerful dosage can be given, without systemic side effects. The chemotherapy drugs are drained out of the body at the end of the procedure.

All told, the surgery plus HIPEC procedure can last as long as 12 hours. Diaz went through it in August 2022. Raoof describes it as “pretty uneventful. I was very happy with the quality of the operation.”

Violet Diaz
Violet Diaz

As for Diaz, she was nervous, but never in doubt. “I knew I was where I was supposed to be. And I knew I’d be OK.”

Recovery, however, took longer than expected. Diaz developed stomach acid problems that made it difficult to take the typical pain medications administered after surgery. Pain management specialists from City of Hope’s Department of Supportive Care Medicine tried a variety of options before finding success with a combination of Tylenol and Mylanta.

Diaz went home after two weeks, but still needed six rounds of systemic chemotherapy to target any lingering cells. Since then, she’s had a few ups and downs, including some nerve pain and, most recently, a bowel blockage. But most important, coming up on one year after surgery, her scans show NED: no evidence of disease.

Is she cured?

“With solid tumors, there’s always a risk of recurrence,” said Raoof. “But I believe she’s in the 70% who will live 10 years or longer. And even if it does come back, it’s likely to be very slow growing.”

He sees a lesson in this case that everyone should take to heart.

“If you have vague, persistent abdominal symptoms, don’t ignore them! And if it is appendix cancer, find an expert. With such a rare cancer, general oncologists may not be up to speed on the best treatments.”

Diaz learned a few lessons, too.

“Hope is not lost!” she said. “Don’t ever give up. Cancer is scary, but with City of Hope you can survive it. They’ll help you, and they’ll help your family. They are awesome.”