MSK Experts Develop New Guidelines to Treat Rare Form of Sarcoma: Epithelioid Hemangioendothelioma

William Tap, Chief of MSK’s Sarcoma Medical Oncology Service, sits at his desk

William Tap, Chief of MSK’s Sarcoma Medical Oncology Service, is studying better ways to treat ultrarare sarcomas, including epithelioid hemangioendothelioma.

Memorial Sloan Kettering has been a leader in the care of sarcoma patients for decades, treating about 70% of the cases in the New York City tri-state area. A disease largely affecting young people, sarcoma is a rare cancer of the bones and soft tissues with more than 50 subtypes. The Sarcoma Center at Memorial Sloan Kettering studies both rare and ultrarare forms of the disease, focusing on its molecular changes.

In June 2021, two of the Sarcoma Center’s leaders — MSK medical oncologist William Tap and pathologist Cristina Antonescu — were co-authors of a paper published in ESMO Open that established guidelines for caring for patients with an especially rare sarcoma called epithelioid hemangioendothelioma (EHE), which is a kind of tumor in the blood vessels.

“EHE is a very complicated disease,” says Dr. Tap, Chief of MSK’s Sarcoma Medical Oncology Service. “Some people can have very slow-growing disease and can go for long periods without treatment. Or they may do very well with surgery to remove the tumor and nothing else. But for others, it can be very aggressive and dangerous.” MSK researchers are trying to learn why the disease behaves differently in different people.

Teaming Up to Improve Patient Care

Because there are no drugs approved specifically for EHE, the European Society for Medical Oncology (ESMO) convened an international team to establish standards for the best way to treat the disease. The scientists seek to predict which patients are likely to do well with less-aggressive treatment and which need more-aggressive treatment. They also want to develop clinical trials for EHE and set standard treatment paradigms.

Learning more about EHE is an ongoing mission of MSK’s sarcoma research. Thanks to funding from the EHE Foundation, researchers at MSK are strengthening the EHE research program. The effort is led by Dr. Tap and Dr. Antonescu, Director for Bone and Soft Tissue Pathology. Medical oncologist Evan Rosenbaum is also involved.

The goals include:

  • creating an EHE clinical database that includes genomic information as well as samples of tissue and blood
  • instituting a multidisciplinary patient care team focused on diagnosis and treatment
  • launching a comprehensive research program, including various genetic projects
  • developing immunotherapy approaches
  • developing lab models of EHE and using them to study potential new drugs
  • developing a central repository of both data and tissue samples that can be shared with other researchers studying EHE

A major objective is bringing EHE patients into clinical trials for a broader range of sarcomas. “Clinical trials allow us to offer people with EHE cutting-edge therapies that are aligned with the biology and nuances of their disease,” Dr. Tap says. “They also allow us to collect prospective data about EHE and the best ways to treat people who have it.”

[Clinical trials] allow us to collect prospective data about EHE and the best ways to treat people who have it.
William D. Tap medical oncologist

Dr. Antonescu is also taking a deeper look at the different variations of EHE disease.

MSK’s Leadership in Sarcoma Research

MSK’s expertise in treating sarcomas has been recognized with a Specialized Programs of Research Excellence (SPORE) grant from the National Institutes of Health. MSK is the only institution in the country to receive a SPORE specifically for sarcoma. Surgeon-scientist Samuel Singer, who heads the Sarcoma Center and is the SPORE grant principal investigator, leads many research efforts across the range of sarcoma types.

Philanthropy has driven advances in MSK’s sarcoma research for many years, including longtime support from the Kristen Ann Carr Fund.

“The purpose of the Sarcoma Center is to really organize our research and patient care efforts,” Dr. Tap says. “EHE is just one example of a disease that requires us to work together across multiple areas of specialization and collaboratively among international institutions. Because this disease largely affects younger people, there is also an important role for our Adolescent and Young Adult Program to play in caring for these patients.”


Key Takeaways
  • MSK’s Sarcoma Center is a nationally recognized leader, and MSK treats the majority of sarcoma cases in the New York metropolitan region.
  • MSK researchers are developing new guidelines to treat a rare sarcoma, EHE.
  • Caring for sarcoma patients, who are typically young, requires experts from many disciplines and the MSK Adolescent and Young Adult Program.