“I will NOT miss dancing with my son at his wedding.” Though her son was only nine at the start of her journey, Denise Pires, 42, fixated on this mantra from the moment she received her 2012 diagnosis of PMP, a two-in-a-million variation of appendiceal cancer. Her fierce focus paid off: she has been disease-free for nearly five years and counting.
“My husband and our son were my heroes,” she reflects, acknowledging, “the care I received at Tufts Medical Center was nothing short of amazing. Dr. Goodman truly saved my life. PMP is such a sneaky disease and is often times misdiagnosed.”
Such was the case for Denise at the onset of her health odyssey. Sharp pains in her lower right side accompanied by nausea and digestion issues in April 2011 prompted her primary care physician to order an ultrasound, which uncovered a six-inch ovarian cyst. That October, the cyst was removed via laparoscopic surgery, but her symptoms continued.
Unearthing the root diagnosis
Over the next seven months, Denise wound along a twisting, turning medical path, inching ever-closer to the crux of her symptoms. Food allergies and Crohn’s disease were considered and ruled out. CT scans, blood work, and additional ultrasounds explored all conceivable options—revealing, in March 2012, the recurrence of the ovarian cyst, along with an appendiceal tumor.
In early May, Denise underwent an appendectomy and the removal of her left ovary and Fallopian tubes. During that procedure, surgeons discovered cyst perforations leaking mucus into the abdomen, and diagnosed Denise with PMP.
According to the National Organization for Rare Disorders, PMP, aka pseudomyxoma peritonei, aka mucinous adenocarcinoma (and a handful of other names), is an exceptionally uncommon malignant growth noted for its accrual of mucus-secreting (mucinous) pelvic and abdominal tumor cells. So thorny is this disease that for decades, leading experts and researchers have been exploring and debating its every detail from its classification to causes to best forms of treatment.
The best of the best
Luckily, Dr. Martin Goodman, Director of Tufts MC’s Peritoneal Surface Malignancy Program, is one of these leading experts, and has remained on the cutting edge of the most highly effective treatment of these complicated cancers for many years.
Denise and her family discovered as much when her unexpected PMP diagnosis led her to seek care from a top-shelf specialist. Internet research and a recommendation during an appointment with her appendectomy surgeon both pointed to Dr. Goodman. “I immediately went home and sent Dr. Goodman an email,” recalls Denise. “Within 30 minutes he had written me back. Within three days I had a VERY informative consult.”
Instantly confident that Dr. Goodman more than possessed the skill and experience to tackle her condition, Denise and family decided “the next adventure was going to be difficult but with positive thoughts and energy, we were ready for the challenge.”
Dr. Goodman’s game plan: abdominal cytoreductive or debulking surgery to remove any visible tumors, followed by hyperthermic intraperitoneal chemotherapy, or HIPEC treatment, which circulates heated chemotherapy through the abdominal cavity.
An innovative therapy
“We know cancer cells die at around 40 degrees Celsius and normal cells die at around 44 degrees Celsius,” says Dr. Goodman. Therefore, heating chemotherapy drugs to about 42 degrees Celsius (107.6 Farenheit) targets patients’ cancer while preserving healthy tissue. For the right patient under the right circumstances, he explains, HIPEC treatment can shift a diagnosis from incurable to curable—noting that some patients have gone on to live another 30 years.
“While HIPEC can be effective for many cancers in the abdominal cavity,” says Goodman, “where this treatment has been studied most is with appendiceal cancers, which are very rare, and very lethal.”
That last point was made abundantly clear through Denise’s research. “I eventually stopped googling because I felt like everything was so morbid,” she says. “As surgery drew near, I honestly just mentally and physically prepared myself.”
Her eight-hour procedure went off without a hitch. “We know what problems to expect and how to take care of the complications,” says Dr. Goodman, who successfully removed all of Denise’s cancerous tissue, and in doing so, had to remove her uterus and gall bladder, and scrape organs including the diaphragm, liver, and kidneys. Goodman, Boston’s most experienced HIPEC surgeon, has completed more than 450 such operations over the course of his career, and, along with his Tufts MC team—a chief resident, anesthesiologist, anesthesia resident, scrub tech, and nurse—performs what is considered a high volume at approximately 40 HIPEC procedures annually.
“Dr. Goodman is a GODSEND,” says Denise. “From day one, his confidence and expertise in handling cases like mine and more extreme than mine put me and my family at ease. We knew we were in good hands.”
The recovery team
Buoyed by five-star care at Tufts MC and an endless stream of visiting family and friends, Denise’s 15-day inpatient recovery flew by. “Everyone from housekeeping to the nursing staff to the medical team was WONDERFUL. I also had a special group of ‘cheerleaders’ that were at the hospital EVERY DAY. This is especially important during recovery,” she reflects. “A little bit of laughter is so good for the soul…although my 12-inch abdominal incision did not enjoy it!”
Dr. Goodman closely monitored Denise per his typical schedule—a post-operative appointment followed by tumor markers drawn every three months and CT scans every six months for the first two years after surgery. Clearing that hurdle with no recurrence, Denise’s prescribed visits scaled back to tumor markers every six months and annual CT scans until year five—a milestone she will officially reach in August 2017—at which point she will be down to annual tumor markers with plans for CT scans at years 7, 10, and 15.
“The further a patient is out from surgery without recurrence, the lower the likelihood of recurrence,” says Dr. Goodman. “Denise has been doing very well.”
“I am pretty optimistic in feeling that this journey is part of my past,” Denise concurs. She has shifted her focus to maintaining a healthy lifestyle, and—though it’s still far too early for gown shopping—today sets her sights ever more assuredly on one day dancing at her son’s wedding.
Posted March 2018
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