World-Class Sarcoma Treatments by Multidisciplinary Team at Dana-Farber Brigham Cancer Center

Multidisciplinary cancer care is crucial for uncommon and complex diseases like sarcomas, and very few cancer centers can provide the level of expert care that Dana-Farber Brigham Cancer Center can. That is because Dana-Farber Brigham has invested heavily in its clinical and research infrastructure, building a team of specialized physician-scientists and other sarcoma-focused clinicians who work closely together to provide outstanding care and drive life-changing research advances.

“Dana-Farber Brigham has embedded some of the world’s best science in a highly experienced clinical program that provides top-notch care to patients with complex needs,” says George Demetri, MD, director of the Sarcoma Center at Dana-Farber Brigham. “That approach is rarely seen, even in the best cancer centers, and it allows us to take advantage of scientific opportunities as they arise.”

Unusual cancers like sarcoma can provide a springboard for developing new therapeutics based on scientific discoveries. Dr. Demetri encourages everyone on his team to capitalize on new discoveries to target vulnerabilities in cancer cells and apply them to relevant subtypes of sarcomas. This approach helps accelerate clinical testing, minimizing the time from scientific discovery to practice-changing clinical application. As a result, Dana-Farber Cancer Institute and Brigham and Women’s Hospital have their fingerprints on most of the approved sarcoma drugs on the market today.

A Skilled Sarcoma Treatment Team

Correctly diagnosing a patient’s sarcoma is crucial to proper treatment, and Dana-Farber Brigham’s sarcoma pathologists are among the best in the world, according to Elizabeth Baldini, MD, MPH, radiation oncologist in the Department of Radiation Oncology at Dana-Farber Brigham Cancer Center.

Sarcomas make up less than 1% of all cancers. Furthermore, they can occur anywhere in the body, and there are more than 150 different subtypes.

“Our pathologists see a very high volume of these rare tumors and are highly experienced in recognizing the various types of sarcoma,” Dr. Baldini says. “They change the diagnosis about 25% of the time for patients referred from outside hospitals. This leads to clinically significant changes in our management recommendations for more than one out of seven patients that we see here. We feel very fortunate to work with such expert colleagues, who enable us to deliver truly optimal personalized care.”

In the clinic, Dr. Baldini and her radiation oncology colleagues see each patient side-by-side with their surgical, medical, and other specialists. This allows the physicians to discuss patient-management approaches and techniques in real time as one integrated team. Further collaborative discussions take place in weekly tumor boards, in which the multidisciplinary team reviews pathology and radiology reports for new and existing patients.

This type of multidisciplinary care from an expert sarcoma center leads to better patient outcomes, Dr. Baldini said. For example, while the local recurrence rate for patients with extremity sarcoma is generally around 15%, the rate at Dana-Farber Brigham is about 5%.

Bringing the Latest Sarcoma Treatments to Patient Care

Customized radiation treatment plans at Dana-Farber Brigham include innovative technologies such as stereotactic body radiation therapy, which delivers multiple unique beams precisely shaped to deliver high doses of radiation to tumors.

In addition, Dana-Farber Brigham is the first cancer center in New England and one of the few in the country to offer the magnetic resonance treatment planning system and MRI-guided radiation therapy treatment. MRI-RT allows radiation oncologists to target tumors more accurately and adapt the radiation plan based on where the patient’s cancer and organs are moving each day.

“Radiation-therapy planning technology is typically guided by CT scans,” Dr. Baldini says. “However, CT scans are not ideal for imaging soft tissue tumors, including sarcomas of the extremity and trunk. Our MRI planning approach enables us to obtain MRI planning images with the patient immobilized in the radiation treatment device. This results in optimal visualization of the tumor and more precise target contouring than is possible with CT-based planning.”

Researchers at Dana-Farber Cancer Institute also were responsible for improving the standard of care for an ultra-rare subtype called perivascular epithelioid cell sarcoma (PEComa), which occurs in fewer than 85 patients per year in the United States and previously had no approved therapies. Andrew J. Wagner, MD, PhD, led a small but influential prospective clinical trial of a novel drug called nab-sirolimus which led to FDA approval of the new treatment targeting a specific molecular mutation in the PEComa cells.

Revolutionizing Treatments for GIST

The Sarcoma Center has revolutionized treatment for gastrointestinal stromal tumors (GISTs), a rare sarcoma found in the wall of the digestive system, with the first approved targeted therapy over two decades ago. Over the years, Dana-Farber Cancer Institute has developed all five FDA-approved drugs for the disease and is actively testing several more.

The Sarcoma Center’s Jiping Wang, MD, PhD, research director in the Brigham’s Division of Surgical Oncology, recently pioneered a state-of-the-art surgical technique for sarcoma called the transabdominal inferior retroperitoneal approach (TIRA).

TIRA was developed specifically for treating GISTs arising along a challenging stretch of the duodenum and other small retroperitoneal sarcomas near the aorta and vena cava in circumstances when a wide margin of normal tissue around the tumor is not typically required due to tumor behavior.

This groundbreaking approach employs robotic precision to remove GISTs with remarkable accuracy and minimal invasiveness, allowing for superior precision and articulation during surgery. Through strategically placed, dime-sized incisions, this procedure provides a viable alternative for duodenal GISTs to the more complex Whipple surgery, which often requires the removal of a wide margin of tissue. By eliminating the need for extensive incisions or organ repositioning and reducing the risk of substantial blood loss, TIRA helps patients recover faster, with reduced complications.

It should be noted that TIRA is not suitable for other periampullary tumors such as pancreatic cancer. The specificity of TIRA for duodenal GISTs lies in the fact that only a small margin of surrounding tissue is needed for effective treatment. By preserving surrounding organs, TIRA minimizes life-changing side effects associated with extensive organ removal.

Leadership in Surgical Treatments

Another example of Dana-Farber Brigham’s leadership in surgical treatments for sarcoma comes from Chandrajit P. Raut, MD, MS, chief of Surgical Oncology, who has led studies on the use of enhanced recovery after surgery (ERAS) pathways to decrease the rate of wound complications in patients with soft tissue sarcoma.

Dr. Raut also was instrumental in a recent multinational phase 3 study to evaluate the impact of preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma. The study was the first large, international, randomized trial in primary, localized retroperitoneal sarcoma to be successfully completed. With 43 months of follow-up, the trial was negative, showing similar abdominal recurrence-free survival in patients receiving surgery alone and in those receiving preoperative radiotherapy plus surgery. Similar overall survival was also observed in the two groups.

“We knew going into the study that radiation therapy reduces the risk of local recurrence in extremity soft tissue sarcomas, but we didn’t have evidence of efficacy for retroperitoneal sarcoma and therefore would have to extrapolate to justify its use,” Dr. Raut says. “We now suspect that radiation may not be as effective for all retroperitoneal sarcomas as it is for extremity sarcomas. There is more to study, but the potential role for radiation may be limited to only a subset of patients with retroperitoneal sarcoma.”

Collaboration Leads to Innovation

Dr. Baldini says that development of targeted therapies and other innovative sarcoma treatments are a testament to the way in which Dana-Farber Brigham’s physicians, clinical researchers, and basic science researchers collaborate. They work closely together, allowing them to brainstorm study ideas internally and with international researchers, as they did in a recent international trial of preoperative radiotherapy in patients with primary retroperitoneal sarcoma.

“From a therapeutics standpoint, we lead an impressive number of clinical trials, giving patients early access to drugs developed for specific types of sarcomas as well as drugs developed for other forms of cancer which might be useful in sarcomas,” Dr. Demetri says. “And while we’re proud of our collaboration with the biopharmaceutical industry, we’re even more gratified that we can provide a truly differentiated service for our patients, many of whom come from across the country and from around the world for treatments that will make a difference in their lives.”