Krisha Howell, MD

 

When addressing recurrent sarcoma, it takes a multidisciplinary team approach.

Each Tuesday, a group of Fox Chase Cancer Center doctors and specialists meet to discuss these complex sarcoma cases at their weekly Sarcoma Tumor Board meeting.

It’s a lively discussion among experienced specialists who’ve devoted their careers to tackling this rare and varied disease. The team includes medical oncologists, surgical oncologists, radiation oncologists, pathologists, radiologists, advanced practice clinicians, social workers, database managers, nurse navigators, and trainees.

Together, they assess each case, analyzing and reviewing pathology slides for each specific kind of sarcoma and strategizing about what surgical, radiation or chemotherapy approaches—alone or in combination—will lead to the best outcomes. They also determine what order the therapies should be delivered in and whether one of Fox Chase’s clinical trials might be appropriate.

Sarcoma is a rare and varied connective tissue cancer that affects about 12,000 people each year and falls into three main categories: soft tissue/extremity sarcomas, retroperitoneal sarcomas, and bone sarcomas (including osteosarcoma), which are typically diagnosed in young people. In addition to being a rare disease, sarcoma often recurs, particularly in cases where the original tumor has an aggressive biology or is discovered at advanced stages.

With so many different kinds of sarcoma that can be easily misdiagnosed in settings where they aren’t frequently seen, Fox Chase’s expertise in sarcoma care and its multidisciplinary team approach provide patients with more treatment options. This leads to better outcomes for all stages of presentation.

“When we see a patient with recurrent sarcoma, we think about how to best treat them as a team,” said Margaret von Mehren, MD, Chief of the Division of Sarcoma Medical Oncology at Fox Chase. “Our program includes specialists in sarcoma medical oncology, surgical oncology, orthopedic surgical oncology, radiation oncology, radiology and pathology who review the biopsies and imaging to determine the best possible treatment for each case.”

The members of the Sarcoma Tumor Board also review patients’ cases as their treatment timelines progress, allowing them to discuss new developments in each patient’s case and provide additional therapies (or change the treatment course) as needed.

The Riddle of Recurrent Sarcoma

There are over 70 different types of sarcoma, and each one is unique.

“You have to know the nuances of each type, such as whether it’s best to treat first with chemotherapy, radiation, or surgery and if you need additional biopsies or other staging studies before proceeding with a definitive plan,” said Jeffrey M. Farma, MD, FACS, Professor of Surgical Oncology and Chief of Fox Chase’s Division of General Surgery.

Sarcoma also has a high rate of being misdiagnosed and improperly categorized as the wrong subtype.

“We’ve had many patients whose diagnoses changed after our multidisciplinary review, which affected their disease management plans,” said Krisha J. Howell, MD, Assistant Professor in Fox Chase’s Department of Radiation Oncology.

As a designated National Comprehensive Cancer Center, Fox Chase has a variety of resources available for patients facing sarcoma and other rare cancers.

“We offer a strong cancer epidemiology program and top-notch pathology and radiology services,” said John A. Abraham, MD, FACS, a Fox Chase orthopedic oncologic surgeon with particular expertise in sarcomas of the bone (such as osteosarcoma). “When it comes to recurrent aggressive disease, every one of those offerings becomes important.”

Innovations

Fox Chase offers innovative treatments that improve on past approaches and employ novel tools and techniques. These include:

Systemic therapy: Because of Fox Chase’s robust clinical trials, specialized understanding of sarcoma biology, and extensive expertise with recurrent sarcoma, the team is able to sort out which patients need which type of systemic therapy, rather than just lumping all sarcomas together. “Our patients have greater access to treatment options that go beyond the standard of care that is available elsewhere,” said von Mehren. “For example, in one of our trials, we do genomic profiling on each patient’s tumor and can match that profile to specific targeted agents.”

CivaSheet®: Fox Chase is one of few medical centers now offering this unique brachytherapy approach, which is a flexible silicone sheet that delivers unidirectional low-dose radiation (LDR) to a customized, targeted area. After resection, the bioabsorbable sheet is implanted and sutured into areas of concern for positive or close margins. It’s used for patients who cannot receive further external beam radiation or attend daily treatments. “This targeted brachytherapy minimizes the dose falloff to normal organs, causing less damage to normal tissue that would have previously been exposed to radiation therapy,” said Howell.

Hypofractionated radiation therapy: An option for patients who cannot attend long-term treatments, Howell said this approach is well tolerated (no toxicities Grade 3 or higher), which is similar to standard radiotherapy but involves less time.

Isolated limb infusion: For multiple recurrent sarcomas on the extremity that are unresectable, Fox Chase’s surgeons use a bypass circuit to deliver heated chemotherapy drugs to the extremity through catheters for about two hours, delivering higher doses of chemotherapy regionally to the extremity than can be given systemically to the whole body.

Surgical implants and custom-designed cutting guides: When preparing for a resection of osteosarcomas on the extremities, Abraham and his team work closely with a number of medical fabricators that specialize in custom-designed cutting guides and bone replacement implants.

Plastic and reconstructive surgery: Fox Chase’s plastic and reconstructive surgeons work closely with surgical oncologists and orthopedic surgeons to determine how to provide structure that aids in a wound’s healing and improves the patient’s movement following surgery. “Meeting with our plastic and reconstructive team before procedures allows me to plan the best resection for each patient,” said Abraham, who noted that he and his colleagues use every resource possible to preserve the limb and avoid amputation. “I don’t have to worry about how the wound is going to be closed afterwards, and I don’t have to skimp on the resection because the plastic and reconstructive surgeon and I are already on the same page.”

Complex surgical challenges: Fox Chase surgeons have performed many redo operations for recurrent sarcoma and, according to Abraham, they have vast expertise with anatomy that has been distorted due to prior surgeries. The team also knows firsthand how important an aggressive approach can be for retroperitoneal sarcomas that have spread to nearby areas or organs and can often operate on GI tumors laparoscopically or robotically (using minimally invasive approaches that lead to easier recovery and shorter hospital stays).

Research: Patients at Fox Chase have the option of enrolling in various clinical trials, which often expand their treatment options. Also, all sarcoma patients at Fox Chase are offered enrollment in the organization’s sarcoma database, allowing for retrospective and prospective studies by Fox Chase researchers as well as national and international collaborative research projects.

Comprehensive Sarcoma Care

While recurrent sarcoma presents various challenges for physicians and patients, the expertise of a multidisciplinary cancer center offers the best chance for treatment success.

“Sarcoma recurrence is a marker for aggressive biology, which means we need to take an all-inclusive approach for the best chance of a successful outcome,” said Abraham. “At Fox Chase, we have the full breadth of expertise and services available to tackle this disease.”

Things to Know About Recurrent Sarcoma Treatment

• It’s not uncommon for sarcoma to recur, especially in cases where the original tumor has an aggressive biology or is discovered at advanced stages.
• Sarcoma also has a high rate of being misdiagnosed and improperly categorized as the wrong subtype, making a multidisciplinary team essential for proper identification and treatment.
• Fox Chase’s team of specialists offer innovative treatments for recurrent sarcoma, including various systemic therapy, radiation, and surgical options.