Saving Limbs and Lives

 

Sue and William Wall enjoy a day in the park with their son Jack.

 

 

Austin attorney Sue Wall dismissed the little lump above her right knee as a result of too much lifting and ladder climbing while moving into a new house. When the pain worsened, she went to a doctor, had diagnostic tests that found a bone tumor and was sent to a surgeon.

 

Her surgeon scheduled an amputation.

 

"I was absolutely terrified," she says, shuddering as if the experience had been last week instead of nine years ago. At the time, she was 27, recently married and getting established in her legal career.

 

The physician she consulted for a second opinion advised Wall to go to M. D. Anderson and then arranged for her to see Dr. Robert S. Benjamin, chairman of the Department of Melanoma and Sarcoma. Imaging studies and other tests indicated the cancer, an osteosarcoma, had not spread beyond its primary site. Dr. Benjamin told her she was a good candidate for a three-part therapy that included limb-sparing surgery.

 

Wall had four courses of intensive combination chemotherapy followed by surgery, in which the tumor-bearing bone was removed and replaced with a metal prosthesis. Then she received more chemotherapy designed to destroy any stray cancer cells that might have escaped and metastasized to distant organs. Her prognosis for long-term survival was positive.

 

"After awhile, I felt so good that I asked Dr. Benjamin about trying to have a baby. He never said I wouldn't be able to get pregnant, but I could tell he wasn't optimistic," she says.

 

Meanwhile, Wall and her husband, William, who teaches fourth and fifth graders, were enjoying life. They walked, rode bikes, swam and traveled as much as possible. Her prosthesis stood the test of sight-seeing trips to Mexico and Portugal as well as strenuous hiking and horseback riding in Costa Rica. She was happy specializing in property law. As the years passed, she gradually gave up hope for having a baby.

 

"I was thrilled when I got pregnant in the summer of 1996. My pregnancy was quite normal, and my right leg held up fine even after I gained weight . . . William and I were absolutely elated when Jack was born on March 20, 1997 . . . Today, I am bursting with gratitude for the blessings of good health and for our wonderful family," Wall says.

 

 

"Our foremost priority always is saving patients' lives. Through limb-salvage therapy, we also strive to provide an optimal quality of life by avoiding amputation." - Dr. Robert S. Benjamin

 

 

In retrospect, Dr. Benjamin says he was doubtful about Wall having a baby because the powerful chemotherapy could have impaired her ability to get pregnant. For a few years after she finished treatment, he was concerned about her long-term survival. He and her M. D. Anderson surgeon, who has since retired, followed her frequently for early signs of metastasis and/or problems with the prosthesis.

 

"Our foremost priority always is saving patients' lives. Through limb-salvage therapy, we also strive to provide an optimal quality of life by avoiding amputation of arms and legs for patients with primary bone cancers and various other sarcomas. We want all patients to attain their pre-cancer goals whenever possible. In Sue Wall's case, I tried to help balance her expectations because I didn't want her to be too disappointed if she couldn't have her own child," Dr. Benjamin says.

 

Hearing from Wall that Jack had arrived and that both mother and baby were quite well was one of the happiest phone calls Dr. Benjamin has received.

 

Limb-salvage therapy, which largely has been developed at M. D. Anderson, was in a state of tremendous evolution when Wall started treatment in 1988. Today, she is among a group of almost 100 osteosarcoma patients who have survived disease-free from five to nearly 20 years and are considered cured. Bone cancer is relatively rare so it takes time to follow a sufficient number of patients and analyze their treatment results.

 

"In the mid-1970s, we began combining continuous intravenous infusion of Adriamycin with intra-arterial infusion of cisplatin to destroy as much of the cancer as possible prior to limb-sparing surgery, and then we gave post-operative chemotherapy in an attempt to kill metastatic cells. By the late '80s, we had added ifosfamide and high-dose methotrexate to our chemotherapy regimen. Over the past decade, we have refined our mix of drugs and intensified some of the doses both before and after surgery," Dr. Benjamin explains.

 

Many limb-salvage strategies also apply to patients with soft-tissue sarcomas, which are newly diagnosed in about 6,600 Americans each year. Osteosarcomas and other bone tumors account for about 2,500 new cancer cases annually. The vast majority of these tumors occur in the extremities. Amputation of an affected arm or leg has been the exception for patients at M. D. Anderson for many years.

 

Dr. Alan W. Yasko, chief of the Section of Orthopaedic Oncology, says the increasingly successful management of sarcomas can be attributed to the optimal integration of medical skills. From a patient's initial appointment, multidisciplinary team members assess diagnostic test results, discuss treatment options, plan and then evaluate each phase of therapy, and talk about rehabilitation and quality of life issues.

 

Opening a dedicated Sarcoma Center last year has allowed team members to interact even more effectively, he says. The sarcoma treatment team usually consists of a medical oncologist, general and orthopaedic surgical oncologists, a plastic surgeon as needed, radiation oncologist, pathologists, two types of radiologists, a rehabilitation medicine specialist and a psychiatrist, nurses and physical and occupational therapists.

 

"Our challenge in preparing for and performing limb-sparing surgery is to preserve as much normal tissue as possible and replace what we take out with a durable prosthesis or suitable natural materials to give patients the best opportunity for maintaining function," Dr. Yasko observes.

 

Better constructed metal prostheses that withstand considerable "wear and tear" now are available but a lifetime guarantee remains elusive. Such prostheses are used in about 90 percent of limb-sparing operations for patients from about 15 years and older. Expandable prostheses allow a greater percentage of younger patients to be considered for limb-salvage.

 

"The expandable prostheses are much better designed but still require open surgery to lengthen them as the youngsters grow. My youngest sarcoma patient undergoing limb-sparing surgery of the leg was seven. Another child who was five had a prosthesis in his upper arm," he says.

 

Donor bones called allografts are used to replace tumor-bearing bones in about 10 percent of osteosarcoma patients. Dr. Yasko says a combination of donor bone and metal prosthesis is another alternative that may increase the survivorship of the reconstruction. He cited a couple of patients who have the combined materials from hip to knee.

 

Improved imaging studies, notably magnetic resonance and computed tomography, along with precisely performed needle biopsies are critical to preoperative planning for any limb-sparing procedure. Plastic surgeons now participate in some operations to help transfer tissue for use in the reconstructed sites and/or to perform other techniques aimed at preventing wound complications.

 

"For the present, our goal is a cured and intact patient who can resume as near normal function as possible for the duration of his or her lifetime," Dr. Yasko says. "Ongoing research efforts are focused on improving the quality of life with the promise of less debilitating therapies and customized tissue-engineered biologic reconstructions in the future."

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