Maria Voermans, the friendly face behind the counter of the Small Stones Wellness Center, regularly assists patients, helping them to look and feel better as they face life-altering illness or disease. Maria is knowledgeable about the center’s wellness products for a reason: she is a survivor herself of a cancer so rare, her doctors referred to it as the “Maria Cancer.” Initially, Maria thought the pain in her upper thigh was just a pulled muscle. It was March 2008, and Maria was a busy single mom.

“I was giving my then 4-year-old daughter an “airplane ride,” where you lay on your back and lift up the child with your legs with your feet under their belly. I remember thinking, “Carly, you’re getting big!” My right leg hurt after that for a few days which turned into weeks, then into two months.” Eventually, Maria’s pain became so bad that when she put weight on her leg, it would buckle. Then, she began to feel her pulse in her leg. Maria’s primary care doctors originally told her she may have a common sports injury, but when the pain did not go away, Maria began to suspect something worse. An MRI revealed the problem: a five centimeter soft tissue mass was pressing on her femoral artery and nerve. Her doctors referred her to orthopaedic surgeon David King, MD, at the Clinical Cancer Center at Froedtert Hospital.

“Because of the location of her tumor, the mass was pressing on her femoral artery and was pushing it out, making it closer to her skin,” Dr. King said. “The mass was also pressing on her femoral nerve, so she had a lot of nerve pain.” On Sept. 5, 2008, Maria’s daughter Mallory’s birthday, Dr. King gave Maria the news that her biopsy revealed a very rare form of aggressive soft tissue, a stage III proximal type of epithelioid sarcoma. Sarcomas require a biopsy for diagnosis, something Dr. King said Froedtert Hospital oncologists prefer to do themselves because if done incorrectly, a physician with less experience could unwittingly spread the cancerous cells. While sarcomas are uncommon already, Dr. King said, Maria’s sarcoma is among the rarest.

“Sarcoma itself is already very rare, with only about 10,000 new cases per year, and proximal epithelioid cancers represent 1 or 2 percent of those. Because of this, very few people have expertise in this, and it is difficult to diagnose … I have only ever seen around five other patients with this in my career,” he said. While scientists do not understand the cause of soft tissue sarcomas like Maria’s, DNA mutations that are acquired during life, rather than inheritance at birth, are common in individuals with sarcomas. Epithelioid sarcomas are commonly characterized by a genetic mutation inactivation of the SMARCB1 gene. SMARCB1 is an active tumor suppressor gene and controls cell division. When it fails, unregulated cell growth occurs, and cancerous cells spread that may result in cancerous tumors forming. In most sarcomas, these mutations occur for reasons that are not apparent.

Because Maria’s tumor was already stage III, was large and deep in her leg, Dr. King and oncologist John Charlson, MD, suggested aggressive chemotherapy treatment to try to shrink her tumor because sarcomas require wide margins for the best chance for successful surgery. Dr. King enrolled Maria in a Radiation Therapy Oncology Group clinical trial that sought to shorten the timeframe before surgery by interdigitating chemotherapy and radiation pre-surgery.

“The goal was to perform both chemotherapy and radiation simultaneously before surgery. We hoped to improve survival rates of people with large, high-grade sarcomas,” Dr. King said.

“These thoughts are running through my mind as I began to cry, like ‘I thought I just hurt my leg! What is happening here? Stage III?’ And then Dr. King looked right at me and said, ‘You have beautiful hair, but you’re going to lose it.’ Open the floodgates,” Maria said. “It may sound silly that realizing I was going to lose my hair hit me so hard. Anyone who knows me and especially knew me in high school knows about my long, thick, curly, dark hair. My hair has always been an attention-getter. In my mind, it was the one thing that would stare me in the mirror every day, reminding me of the monster that had invaded my body, and the poison with which we were trying to fight it.”

The “poison,” was Maria’s aggressive chemotherapy regimen. She had a port in place and, unlike a more typical chemotherapy regimen for a patient who comes in for a few hours per day a couple of times a week or month, Maria came in for inpatient care for 24-hour stretches, four or five days per week. Her rounds meant one week on, two weeks off, for three rounds. In October, she began radiation for six weeks. 14 days into her chemotherapy treatments, Maria did start to lose her hair. But the chemotherapy and radiation successfully shrunk her tumor enough to operate; Dr. King successfully removed her tumor.

“The trial showed us that this protocol was an option. Now, here at Froedtert, we believe we see a survival benefit using the protocol of chemotherapy and radiation, then surgery and chemotherapy afterwards. This isn’t common practice everywhere since many places choose to do surgery first, so it’s not universally accepted. But we have a local recurrence rate of less than 5 percent here,” he said. National statistics show an average rate of recurrence around 77 percent, most often when tumorous sarcoma cells travel through the bloodstream and metastasize in other organs, often within two or three years.

Seven years post-surgery, Maria is still cancer-free, a fact which Dr. King said is very fortunate but not yet fully understood by physicians.

“In Maria’s case, we were very fortunate. We are hopeful that the chemotherapy killed anything that might have spread,” Dr. King said. Participating in soft tissue tumor clinical trials is vitally important to understanding what Dr. King calls “orphan diseases,” or diseases that have very little public attention, and thus, few research opportunities or funding.

“Most of the funding for these types of diseases comes from private donors who have personal experience with the orphan disease,” Dr. King said. “So it was important for us to participate in this trial. It wasn’t a home-run; however, it certainly has helped us develop ways that we treat patients with sarcoma.”

Maria does not attribute her outcome to luck.

“The biggest thing that I appreciated about my care at Froedtert Hospital was that I didn’t have to wait between diagnosis and treatment. For me, it was all very fast. I have heard horror stories about people finding out they have a disease and then having to wait for so long before they receive treatment, but that wasn’t the case for me. I had a whole team working with me right away,” Maria said. “Some may call it luck, but I think it has a lot more to do with my oncology team here at the Froedtert & the Medical College of Wisconsin Clinical Cancer Center,” she said.

In November 2009, Maria ran a 5k, something she never could have imagined accomplishing just two years before. Today, she is newly married, is busy raising two daughters, and enjoys the life she has been given. Four years ago, she began working in the Clinical Cancer Center at the Small Stones Wellness Center, providing support for patients and survivors.

“I had a good experience with the staff at the cancer center, from the nurses to the doctors to the schedulers. I wanted to be part of that, and providing care from the other side. I have a lot of empathy after my experience,” Maria said. “I mean, I'd be lying if I didn't think of my metastasis probability every day. I think about it, I don't dwell on it. I can't. I have two beautiful daughters who need their mom.”

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