Deadly cancer eludes diagnosis

As the cancer raged through her breast and invaded her body, every single doctor — six of them, all in Tucson — blew the diagnosis, threatening her survival.
It wasn't until Norma Greer, thoroughly terrified, left town that she finally found someone to confirm her disease and actually start treating it.
The delay could cost her her life, or at least years of it.
What's troubling about Greer's awful ordeal is that it is not all that unusual. What she has is a rare, aggressive and especially lethal form of breast cancer known as inflammatory breast cancer, or IBC.
There is no lump. It does not show up on a mammogram. Instead, it appears suddenly and grows rapidly, with obvious external symptoms marked by a swollen, red, often pocked breast that grows more inflamed by the day.
It mimics infection, making misdiagnosis tragically common.
Even so, it took Greer herself less than an hour to come up with a possible IBC diagnosis, after she plugged her symptoms into the Internet. Along with that possibility came the words "very aggressive" and "like wildfire" and "grim prognosis," sending her into shock. She — like so many — had never heard of this thing.
She carried that terror for six more weeks, while an internist, two obstetrician-gynecologists, a radiologist, a breast surgeon and an infectious disease specialist all got it wrong, treating her over and over for an infection she didn't have, despite symptoms that only worsened.
"I was freaking out because too much time had passed and I wasn't getting any answers. My breast just kept getting larger, it was very hot, and now I was getting stabbing pains," said Greer, 62, the Tucson mother of two grown daughters.
"I knew by now that if I had IBC, time was of the essence and aggressive treatment would need to start immediately. I knew I was getting nowhere."
But the frightening truth is what happened to Greer is typical for patients with this bizarre killer cancer. It is why she decided to sacrifice her privacy so other women — and their doctors — can be warned of a disease so badly misunderstood.
"Unfortunately, many women see multiple physicians before they get a correct diagnosis. I've seen many examples of cases like that," said Dr. Julie Lang, a surgical oncologist specializing in breast cancer at the University of Arizona Cancer Center.
"That is why we need to do a major education initiative here to get the facts out about this very different breast cancer — one that develops in weeks to months, not months to years. It has to be treated very aggressively, very quickly."
Although Greer asked that the doctors who misdiagnosed her not be named, all are part of the UA medical system. Lang arrived at UA this fall, almost a year after Greer's battle began.
Well aware that misdiagnosing a very rapidly growing cancer can have terrible consequences, doctors who deal with IBC nevertheless try to cut some slack to those who normally don't.
The inflamed IBC breast looks almost exactly like a breast afflicted with a far more common female complaint, the infection known as mastitis.
"Most often, IBC is missed by primary-care doctors or in the ER. They will diagnose the most common thing, an infection," said Dr. Barbara Pockaj, the surgical oncologist at the Mayo Clinic in Scottsdale who finally confirmed IBC in Greer, after she turned there for help.
"So it's not unreasonable to give a short course of antibiotics at first. But if the infection does not clear up, if the antibiotics are not working, that should be a signal something else is going on."
At no point during the six weeks of Greer's misdiagnosis did any of the four courses of increasingly powerful antibiotics have any effect. Her breast only grew more swollen, red, hot and painful.
Also, since mastitis overwhelmingly occurs to women who are lactating, Pockaj insists on considering IBC first in a non-lactating woman, such as Greer, with these symptoms.
With no lump and nothing showing up on a mammogram — IBC grows in "sheets" throughout the entire breast — the only real way to confirm it is through a biopsy of the skin and breast tissue.
Greer's biopsy, done at University Physicians Healthcare Hospital at Kino Campus, came back negative. But at least one physician who has treated her since then told her there is clear evidence of cancer on the biopsy. A biopsy at Mayo confirmed inflammatory breast cancer.
Though she had suspected this all along and was relieved to finally have an answer, this news put her in shock.
"I remember feeling flushed as the shock wave rolled through me, that I couldn't escape this and couldn't believe it was happening to me," she said.
"Cancer. I was trying to get around the thought of a rare, very deadly, aggressive cancer in my body as I sat there. I wanted to rip it out and run. I didn't know what to do, but wanted to stop crying before I called my girls to give them this incredible information."
By the time IBC symptoms appear, the entire breast and nearby lymph nodes are cancerous. But with a six-week delay, Greer had progressed to the next stage, with the cancer invading distant lymph nodes. Within several more weeks, she likely would have moved to the final stage, with cancer spreading to other organs.
"It's difficult to say how the delay has affected her prognosis," said Pockaj, at Mayo. "In the lifetime of a cancer, six weeks is not very long. But with this cancer, the sooner you get on it the better, no doubt. With this cancer, it's one day you're fine, the next day you're not.
"I really have to give credit to this patient, for her persistence, for pushing the system so hard for answers. That will make the difference for her."
Even if diagnosed immediately, the IBC prognosis is indeed grim with a 40 percent chance of surviving five years. In comparison, 65 percent of people with large-tumor classic breast cancer live five years or longer. If the disease is caught early, survival soars to 95 percent.
Greer well knows there is a better than even chance her cancer will recur, as do all women with IBC. She is now completing the extremely toxic throw-everything-at-you course of IBC treatment — high-dose chemotherapy, followed by full breast and lymph node surgical removal, then high-dose radiation and more chemotherapy.
After initial treatment at Mayo, Greer transferred last winter to the just-opened, first-ever IBC clinic in the country, at M.D. Anderson Cancer Center in Houston. She was placed in the care of that clinic's founder, Dr. Massimo Cristofanilli, one of the top — and few — experts on inflammatory breast cancer in the world.
"No one yet knows why this cancer very clearly does not look or behave like a breast cancer, but that is what we want to find out, and why we want as many IBC cases as possible to treat and to study," he said in an interview last week. His clinic has seen 100 IBC patients from all over the country In its first year, and is expecting 200 more through next year.
"One day soon, I am certain we will understand why this cancer is so different, and what to do about it."
Until then, and for the rest of her life — however long that may be — Greer will make it her job to get the word out about "this crud," as she calls it.
"Knowing the symptoms can save lives," she says simply. "I have heard far too many stories like mine. This is hard to diagnose, but if people know it exists, there is a chance to get a speedy diagnosis and begin treatment."
And now, she will get plenty of help from a new team of IBC specialists forming at the UA Cancer Center who share that vision.
"There have been no improvements in understanding or treating IBC in 30 years, and now there are signs it is becoming more prevalent," said Lang, a prime mover and shaker on this team, who trained under Cristofanilli at M.D. Anderson.
For decades, experts have estimated that IBC amounts to less than 5 percent of all breast cancer. Today, that figure is approaching 8 percent, she said.
If a grant from the Susan G. Komen Foundation comes through, Lang will launch an IBC educational campaign aimed at primary-care doctors — "those likely to see this first" — and hospitals, clinics and medical offices throughout Southern Arizona.
In the meantime, Lang is organizing an IBC clinic and an IBC team within the Arizona Cancer Center, and wants it up and running within a year.
"If we can get these women diagnosed quickly, then treated urgently and aggressively at a specialty center with significant multidisciplinary experience, then those 30 years of no good news will be over," she said.
On StarNet: Learn the basics of breast cancer and read about screening and prevention methods at go.azstarnet.com/breastcancer.