PITTSBURGH — The operation is so terrifying some call it MOAS: the Mother of All Surgeries. It can take 16 hours. The risk of complications is high. And after 30 years of research, doctors are still arguing about how well it works.
But as Stephen Phillips shimmied himself onto the operating table one recent morning, he was almost relieved. He’d spent five months desperately trying to arrange this surgery in the hope that it would beat back his rare cancer of the appendix.
Now, as the surgeon scrubbed in, Phillips was ready — or as ready as one can be — to have his innards scraped with electrified wires and sluiced with hot poison.
“It’s been referred to as barbaric. It’s been referred to as having up to 10 abdominal surgeries at once. It’s not for the faint of heart,” he’d said a few weeks earlier. He was nervous, but also eager. “It’s like gearing up for the Stanley Cup championship and the Super Bowl, all rolled into one,” he said. “Minus the hotdogs and the beer.”
STAT followed Phillips for three months on a winding odyssey that started at his home in Springfield, Mass. He consulted nine medical teams in seven states, fought with his insurance company, and dragged himself to appointments in distant cities when he could hardly walk.
To appendix cancer patients, Phillips’s frenzied medical crusade is familiar. Most are told in no uncertain terms that their condition is fatal. But the Internet tells them otherwise. A Google search brings them to descriptions of this marathon surgery — cytoreduction combined with hyperthermic intraperitoneal chemotherapy, or HIPEC, for short — and yields a list of institutions that offer it as a treatment for appendix cancer and other tumors of the abdominal cavity, including colorectal and ovarian cancer.
Fifteen years ago, that list included just 10 or so American hospitals. Now, the number is closer to 100 — but many patients are reluctant to trust doctors who are newer to this procedure, and experts say they’re right to be wary.
For the patient to survive, gloved hands need to feel out and remove every last tumor in the patient’s belly — no mean feat when the malignancies hide among loops of intestine and nestle into the deepest, darkest corners of the peritoneum. The protocol then calls for hot chemotherapy to be pumped into the belly to take care of any remaining cancer. Yet the drugs can only kill off microscopic bits.
If the surgeon has missed even a single tumor nodule, the cancer could grow back again even before the patient has recovered from surgery.
And so, often with little guidance, patients hunt for a surgeon they can trust.
“People will go to six or eight different surgeons. They’ll do a West Coast tour and an East Coast tour, and really struggle to figure out who the best fit is for them,” said Dr. Laura Lambert, a surgical oncologist at UMass Memorial Medical Center in Worcester.
Phillips knew what he wanted in a surgeon: “The best hands.”
So he set out to find them.
Phillips’s saga, like that of many appendix cancer patients, began with a misdiagnosis.
Late last September, just after a dinner to celebrate his 31st wedding anniversary, a sharp pain flashed through his lower right abdomen. The next day he was rushed into surgery for appendicitis: a routine operation, the surgeon told him, no more than 20 minutes.
He knew something wasn’t right when he woke up to a room packed with his wife, Zita, his adult kids, his friends, and his in-laws. Even a client from his legal practice showed up: an Orthodox priest who led a special prayer circle. There were hardly enough surfaces to hold all the flowers and teddy bears and cards.
Instead of 20 minutes, the surgery had lasted hours, because Phillips had a lot more than appendicitis. He had appendix cancer, and it had metastasized: The cells that lubricate the intestinal tract with a mucous-like jelly had mutated out of control, making their way from inside his appendix out into his abdominal cavity. There, they had begun to form tumors. They clung to his intestines and spread webs of white on the lining of his abdominal cavity.
The prognosis wasn’t good. If left there, the tumors would grow and grow, pushing up against his organs, blocking his digestion, and probably starving him to death.
Phillips, 58, was no stranger to terminal cancer. For over a decade, he’d volunteered his legal services to hospice patients, drafting wills and powers-of-attorney, driving patients to appointments, watching Patriots games by hospital bedsides. He’d tracked down one patient’s estranged son. He’d brought fake champagne into a hospital room for another patient, a man with liver cancer who wanted “one last drink” before he died.
Phillips knew that this kind of diagnosis meant he had to act fast. “Sometimes I get in there, and it’s too late, the patient’s out of it, can’t even sign his own name. Or he’s passed away,” he said. “Time is everything.”
But he couldn’t take charge of his own medical case. He could hardly get out of bed.
It was his younger brother Joe who, the night of the diagnosis, began to Google appendix cancer. He searched on his phone while at the hospital; he searched from his desk at the insurance company he runs; he searched at home. And the term that kept coming up was HIPEC.
Steve Phillips’s life depended on a procedure almost as contentious as it is invasive.
The package — cytoreduction and HIPEC — had been dreamed up as a kind of super-treatment for stubborn tumors that had metastasized into the abdominal cavity and were mostly unreachable by intravenous chemotherapy.
The idea: start the patient on IV chemo, just in case. Then open him up and scrape away all the tumors. Then heat chemo to 107 degrees Fahrenheit, because tumor cells are thought to be more susceptible to high temperatures than normal cells, and pump it straight into the patient’s belly. Slosh it around for almost two hours. Suction it out.
In the 1980s, when American surgeons first borrowed the technique from Japan, it was a gamble.
Surgeons have since gained experience with the procedure, and mortality rates have fallen. But it remains controversial.
“There are some institutions that don’t do this type of procedure, and say that we don’t have enough data to support doing it,” said Dr. David Bartlett, a surgical oncologist at the University of Pittsburgh Medical Center, who estimates that he has performed the operation more than 1,000 times. “And then you have the other end of the spectrum, the zealots …”
The zealots, he explained, believe it’s unethical to do clinical trials in which some patients won’t get the heated chemo. In their view, that’s depriving them of the full treatment.
Yet to Dr. David Ryan, chief of hematology and oncology at the Massachusetts General Hospital Cancer Center, that’s exactly the kind of research that’s needed. He knows that if a surgeon can cut out all the tumors, the patient has a better chance at survival. He just isn’t convinced that the heated chemotherapy has any therapeutic effect.
Reams and reams of research have been published about the operation. In 2012, one paper reported that 63 percent of patients with less aggressive forms of appendix cancer survive at least 10 years if they have IV chemo, cytoreduction, and HIPEC all together.
“We know that the whole package works,” said Dr. Victor Verwaal, a colorectal surgeon and HIPEC researcher at Aarhus University Hospital in Denmark.
But many experts, including those who regularly perform the procedure, take issue with these findings, as well as much of the literature about cytoreduction and HIPEC. They say that researchers have been too selective about which patients to include and that the protocols weren’t rigorous enough to really understand the benefit of the heated chemo.
More exacting studies — notably a randomized controlled trial in France — are finally on their way.
Tumors don’t wait for the latest research, though. And the Phillips brothers weren’t about to, either.
Steve Phillips could hardly walk. He’d just had two abdominal surgeries in two weeks — one to take out his cancerous appendix, and the other to repair leaky sutures. But on the October morning after he was discharged, he woke at dawn and dragged himself out to the car, where Joe was waiting to drive him the 90 miles to Boston to meet two different teams of HIPEC specialists.
The trip wasn’t just about Phillips choosing a surgeon. The surgeon, they’d been told, would also have to choose him. If the tumors had spread too much, or if he were too weak, most doctors wouldn’t operate.
Walking from his house to the car had Phillips doubled over in agony. “I was very, very nauseous, in a lot of pain. It was rough,” he said.
But he had to project strength to the surgeons.
“I’m ready to get in a wheelchair,” he said. “And my brother and my son say, ‘No, you can’t get in a wheelchair. You’ve got to frickin’ walk in there and show these guys that you’re ready.’ And so that’s what I did.”
In spite of his condition, the two medical teams they met with in Boston seemed more than happy to take on his case. “Every meeting is almost like a sales pitch,” said Joe Phillips.
But Steve wasn’t yet ready to pick which hands he’d trust: He isn’t the type to be reeled in by anyone. He’d spent most his adult life fighting legal cases. He’s 6’5”, drives a huge black SUV, and favors dark suits with a stars-and-stripes lapel pin. He’s a guy who likes to know exactly what he’s getting himself into.
And so, over the following weeks, Steve, Joe, and Zita drove south to New York and east to Worcester, Mass., to consult more experts. They flew to Pittsburgh. They sent scans of Phillips’s insides to Maryland, New Jersey, Texas, and Washington, D.C.
They read scientific papers and old news articles, picked apart statistics from clinical trials, reached out to patient advocacy groups for advice. They waged — and won — a war against Steve’s insurance company’s initial decision not to cover the procedure.
All the while, Steve was getting intraveneous chemo. He often felt nauseated. But he slogged on.
When interviewing surgeons, he and Zita and Joe had a standard question: How many times have you done this procedure?
But they also asked: If you needed this surgery and couldn’t go to your own hospital, where would you go?
The name that kept coming up was Dr. David Bartlett, out in Pittsburgh. He had been performing this operation since the early 1990s.
Steve made up his mind. The surgery was set for Feb. 19.
“It’s in God’s hands,” Phillips said. “It’s in Bartlett’s hands.”
The night before the surgery, Steve sat with Joe, Zita, and three of his kids in the hotel bar across the street from the University of Pittsburgh Medical Center in the neighborhood of Shadyside.
With rounds of Bud Light, they toasted a successful surgery. They picked at quesadillas. Steve, allowed only clear liquids, took sips of yellow Gatorade. His kids helped him put on his hospital bracelet.
His daughter Katie, a bubbly 26-year old who works in insurance, looked at her dad. Usually, he was something of a ham, telling stories, repeating punch lines, and banging the table for emphasis. Now, he was holding a cup of Jell-O, half eating it, half jiggling it around with his spoon. He had hardly said a thing all day. He was due at the hospital at 5 a.m.
“You excited, Dad?”
“That’s not exactly the right adjective,” he said, shaking his head.
Eleven hours later, he was anesthetized, his torso shaved, a band placed across his forehead to monitor his brain activity.
Bartlett stood over him, inserting a camera into a little incision in his upper left abdomen. A second later, the inside of Phillips’s belly appeared on two screens. It looked arched, like the ceiling in a church, its pink surface overlaid with white.
Pop music was pulsing in the background.
“That white plaque, that looks like tumor,” Bartlett said.
If there were too many tumors, Bartlett would have to sew Phillips up and send him home. But what he saw looked manageable.
With a needle-like tool that cuts and burns at the same time, Bartlett began to make a slit in Phillips’s skin, from sternum to groin. A surgeon-in-training wiped away the blood as it appeared.
The hole was stretched wide and held in place with what looked like a giant Erector set. Inside, Phillips’s intestines were visible, the red loops rising and falling with every one of his machine-aided breaths.
Close to 9 a.m., Bartlett began to cut away the tumors. He started with a yellowish fold of fat called the omentum, which covers the abdominal organs. It jiggled as he ran a gloved finger over it. He could feel that it was studded with tumors.
He took another burning-and-cutting instrument. This one looked like a barbecue lighter, and it made a little click as it clamped down over bits of fat. “I call this The Claw,” Bartlett said. A few clicks later, the omentum was just a bloody lump of flesh in a blue container, ready to be sent to pathology.
Then he moved on to the intestines, which glistened like a string of raw bratwurst.
“With these small tumor nodules, they can hide in this stuff very easily,” said Bartlett. He began to cut with the needle-like tool again. The flesh sizzled and smoked.
The lower down on the intestine Bartlett’s fingers went, the more tumors he found. “We’re going to take out some of that intestine …” he said.
Bartlett snipped tumors out of the peritoneum and off the surface of the spleen. He sliced the gallbladder out completely.
It was hard to believe anyone could survive such a procedure. Phillips’s body seemed at once delicate and resilient beyond belief. One moment, the surgeon and his team were yanking at the sides of the wound with the force of construction workers; the next, they were sewing up a nick in Phillips’s intestines to stem a tiny jet of blood.
The cutting lasted hours.
The Black Eyed Peas came on. Then the Beatles. Simon and Garfunkel. Adele.
The operating room filled with the sour smell of disinfectant and singed flesh. Bloodied surgical sponges were scooped out of Phillips’s belly and handed over to a nurse, who kept careful count so that none would be left inside.
The atmosphere was one of camaraderie, like a theater troupe backstage. When they weren’t asking for instruments, the team talked about their families, teased each other. Everyone ganged up on the nurse who had switched the music to oldies, but they were scrubbed in and so couldn’t touch the sound system.
At 1:30 p.m., five and a half hours after the operation had begun, Bartlett straightened. It was almost time for the chemo.
But first, he took an enormous measuring cup of clear liquid and poured it straight into Phillips’s opened-up belly.
It was water, Bartlett explained: “For cancer cells that are floating, water does more than the chemo.” He reached inside and stirred Phillips’s innards.
The water was suctioned out, and then what looked like two clear garden hoses were stuck into Phillips’s belly, pumping him full of heated chemo. A resident stood there, using her whole upper body to rock Phillips back and forth, making sure that the poison got into every last crevice of his abdomen.
She and the surgical fellows took shifts. The rocking didn’t stop for 100 minutes.
Bartlett took a lunch break. When he came back, the chemo had turned bright red with Phillips’s blood.
At 3:15 p.m., seven hours after Phillips was wheeled into surgery, and just before the team was about to close him up, Bartlett made one final pass through his patient’s belly.
He paused. His gloved fingers moved over the same spot again and again. He’d found another tumor, a hard white lump nestled into the pink flesh of Phillips’s intestine. Somehow, he had missed it before.
“I’m not sure why,” he said. “We ran the bowel twice and didn’t see that spot. That’s just the nature of these tiny little dots.”
He burned it off with a sizzle of smoke.
Bartlett checked the blood flow along Phillips’s intestinal tract, poured in a bit more water to wash out any leftover chemo. And then, with sutures and staples, the team closed Phillips up and wheeled him to the ICU.
Update: Not long after his surgery, Steve Phillips was back in the ICU — and then back in the operating room — due to a series of complications. He had an infection that led to pneumonia, and a leakage in his intestinal tract that needed to be surgically repaired. He was finally discharged a month after his surgery, and is now in recovery.
Correction: A previous version of this story misstated the full name of hyperthermic intraperitoneal chemotherapy.
Greetings! I want to ask if anyone has heard of neuroendocrine cancer? It’s supposed to be the cancer that killed Steve Jobs. He had it on the pancreas, while mine was on my appendix. Initially, I had bi-lateral renal cell carcinoma. The VA claimed it was caused by an exposure to radiation in the military back in the middle 1980’s. Since my last cancer surgery in 2008, the VA checks me every six months. A few years ago, the VA switched from CT Scans to ultrasounds. Last November, they saw something on the right kidney and referred me to Vanderbilt in July 2019. If I would have known they found something I would have acted faster. Vanderbilt conducted a CT Scan and determined there was nothing on the kidney, but thought they saw a banine mass on my appendix. I was given the option of an appendectomy or do nothing at all. The doctor told me he didn’t believe it was necessary to remove the appendix, but would remove it if I chose to. I wasn’t comfortable with anything on my appendix and opted to have it removed. On the Sunday night following the appendectomy, I got a call from the doctor explaining to me the mass was actually this super rare form of cancer called neuroendocrine cancer. The oncologist wants me to have a surgery to remove a quadrant of my colon and all the lymph nodes around the colon and the lymph nodes around my stomach. She referred me to an oncologist surgeon, who said surgery isn’t absolutely necessary, but he would do as I wished. I am having the surgery. My question is has anyone ever heard of neuroendocrine cancer? Would anyone have opted to do nothing?
Hi Everyone, I’m so sorry for everyone who is going thru this horrible cancer. My husband was diagnosed June 2019 at age 55. He has Mixed Goblet cell carcinoid adenocarcinoma. They found the cancer accidentally, thinking it was a bowel obstruction. They removed his appendix ( primary) 15 inches of small intestines, part of his large intestine, removed 23 lymph nodes and three of them had cancer and his omentum was loaded with cancer. We go this Wednesday for his 10th chemo and his last is Christmas. The dr’s have scheduled his Hipec surgery for January 15th, 2020. I would like to talk to anyone who is going thru this or had a loved one go thru this. This has knocked me down, like nothing else in my life. I love him so much. We have three beautiful kids and seeing their faces scared of the unknown hurts beyond belief. When they look at me I just die inside. Wondering what they are thinking bc we don’t talk about it. I personally think they look at me thinking mom you made us better, so now you need to make dad better. Which is just killing me. I am praying for you all as I pray for my family. If anyone would want to talk to me, I can be reached by email. [email protected]
I am thinking and praying for you all. Thank you for letting me vent. Good night.
There is an appendix cancer support group on facebook called PMP Appendix Cancer Support Group. It’s for both patients with appendix cancer and their caregivers. It is an amazing group and you gain knowledge and hope. I have my HIPEC on Feb 4, 2020 with Dr Choudry on Dr Bartlett’s team at UPMC Pittsburgh. The most important thing is to have an appendix cancer specialist! Good luck and God bless you on this journey! There is hope! 🎗
My diagnosis is secondary malignant neoplasm of genital organs, maglignant neoplasm of right ovary and appendex and secondary malignant neoplasms of retroperitoneum and peritoneum, and right and left ovary. Looking for any info I can get. I can’t seem to find any thing like this on internet.
Sammy, I am so very sorry you are having to deal with crazy disease. There is a very informative support group on Facebook. Search for PMP Appendix Cancer Support Group. Also, this link is another great source on Facebook. https://www.facebook.com/acpmpresearch/
I was told what the surgeon removed was a Appendiceal Mucinous Cystadenoma.
Since the doctors world wide cannot agree on a standardized ratings system for the classification of Appendiceal Mucoceles, it’s hard to know exactly what my surgeon removed when he took out my appendix. My surgeon called it an Appendiceal Mucocinous Cystademona, while my onocologist referred to it as a LAMN.
So, I am left “up-in-the-air as to what exactly was found.
This becomes even more confusing since it appears it might also be called a Low-Grade Appendiceal Neoplasm [a neoplasm defined as a tumor associated with neoplastic adenomatous growth – either adenoma or andeocarcinoma], which I gather is an appendiceal neoplasms of uncertain malignant potential or even a enigmatic & confusing low grade malignancy.
Although in September 2017, the medical literature I read simply told me I would be all right or I was going to be in the fight of my life.
Since this condition is so rare, my surgeon referred me to Dr. Kodali as a “safety” precaution. (I think they ended up playing “hot potato” because she referred me bact to Dr. Gade.
However, every six months she has me get an enhanced CT scan & then meet with her to discuss what was or wasn’t found. This is just fine with me because further research has indicated there is a real possibility that I may develop pseudomyxoma peritonei even though there was no indication my appendix had ruptured, my appendix was removed intact using an endobag.
All the medical literature from when I initially conducted my research through the present has indicated that the best treatment for pseudomyxoma pertionei is to debulk (remove) every scrap of the mucin as possible and then after rinsing out the abdomen flood the abdomen with a heated liquid chemo-therapy solution that remains inside the abdomen for “x” amount of time before removing the solution, thoroughly rinsing the abdomen out & then closing the abdomen. The medical articles I read stated this may need to be done more than once.
So, although at the present time, I am more fortunate than many others, I still live with the fear I’m going to develop pseudomyxoma peritonei.
Good luck to everyone seeking hospitals where this procedure is performed.
My 69 year old mother has stage 4 Appendix cancer and told an operation is not possible! It’s chemo with a bleak prognosis. Can you help. We are in Sydney Australia.
Fred, most people diagnosed with appendix cancer are stage 4 as was I. If you are on Facebook please join the group PMP Appendix Cancer Support Group! I don’t know if your mom would qualify but there is a clinical trial in Australia conducted by a Prof. Morris. I’m in Texas and I don’t know the specifics but many people in the Facebook group can help with the information. Praying you find the help you’re looking for.
SOS – My nephew has been fighting stage IV PMP for three years. First he had chemo, then surgery with HIPEC. He had a second emergency surgery for bowel obstruction. He has been on chemo since his first surgery. His abdomen is now full of mucin which is pressing against his bowel etc. Surgeons say he is inoperable and no one seems to know what to do. Has anybody had any kind of procedure(s) for recurrent PMP other than surgery? He is in dire straights unless there are doctors out there doing something for his type of condition.
My 46 year old daughter was diagnosed with this same cancer 5 1/2 years ago, and has had the HIPEC surgery twice. She is now being treated by MD Anderson in Houston. She has appointment next month to check on a spot that seems to be growing. We pray that it isn’t, but she still works and has a great outlook, and has live 3 years longer than the oncologist here in San Angelo told her. We were devastated at the diagnosis, but feel blessed that she is fighting this awful disease.
Never give up. My dad wanted his story told so he could shed light on this terrible disease. Always stay positive and god bless all of you.
Janet I would love so much to speak with you. I was the same age when I got diagnosed I would love to hear more about your daughter and her survival rate. My doctors had given me 10-15 years. I’m at 5 yrs & 4 mis out of surgery & 3 spots appeared. I’m desperate to find out more info. My children’s father passed away unexpectedly a few years back, it was devastating. I can’t hard the thought of not being here for my boys. Please contact me so we can talk if that is acceptable. I pray that your daughter continues to do well. Thank you so much & God bless! This disease us horrible…we need to stick together 💙
Hi Janet,I just read your post and was wondering how your daughter is doing.I am also 46 with stage 4 apendix cancer.I have done 12 sessions of chemo and having the HIPEC surgery while getting some organs in my stomach removed.I am very nervous about the surgery but know that it’s my only chance to see my beautiful children grow a few more years.Would your daughter be interested in connecting with me to talk about the surgery?I wish her to get through all this and to feel her best .
Thank you ,
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