Sarcoma is a relatively rare type of cancer, accounting for only about 1% of all malignancies in the U.S. While the term sarcoma may suggest a single disease, there are actually more than 70 subtypes of the disease that fall into two broad groups: soft tissue and bone. Around 80% of sarcomas arise from soft tissues, such as fat, muscles, blood vessels, nerves, deep skin tissues and fibrous tissues. Bone sarcomas, which can also affect children and young adults, form in the cells that make hard bone tissue.
Dr. John Mullinax, an assistant member in Moffitt’s Sarcoma Department, says over half of soft tissue sarcomas start in an arm or leg. A lump may grow over several weeks or months, and a patient may not experience pain or other symptoms until the tumor is quite large. This is even more true if a sarcoma is growing in the abdomen where symptoms can occur infrequently. As the sarcoma grows, it can press on blood vessels, nerves or nearby organs, which may produce symptoms such as pain, trouble breathing or inability to tolerate food. Bone sarcoma symptoms, however, are generally more consistent with localized pain and swelling in the affected area.
Late last year the fashion industry mourned the loss of an icon Virgil Abloh, a trailblazing fashion designer who became the first Black artistic director at Louis Vuitton and launched his own highly successful fashion brand Off-White. Abloh died after a long battle with a rare form of cancer, cardiac angiosarcoma. Abloh’s diagnosis is a type of soft tissue sarcoma found in the heart.
According to Dr. Ricardo Gonzalez, chair of the Sarcoma Department at Moffitt Cancer Center, angiosarcomas can form anywhere throughout the body. "They are one of the rarer subtypes of soft tissue sarcomas. And soft tissue sarcomas make up roughly 1% of all malignancies," he said.
Symptoms of cardiac angiosarcoma can vary depending on where the tumor is within the heart. If it is in the chamber, it can block blood flow to the heart; or if the tumor is growing into the muscle tissue, it could cause an irregular heartbeat.
"Angiosarcomas are aggressive because they can easily spread to other parts of the body. Unfortunately for patients with cardiac angiosarcoma, by the time the signs are noticeable the cancer is often in advanced stages and treatment options may be limited,” said Gonzalez.
Treatments for this type of sarcoma include surgery, chemotherapy and radiation. But even with therapy, the survival rate for these patients is anywhere from six months to a few years. Gonzalez says the key is for patients to be treated at a specialty cancer center that deals with rare sarcomas.
“My advice would be if a diagnosis of sarcoma is made, patients should be referred to cancer center that offers disease-focused multidisciplinary care for rare tumors, like Moffitt,” he said.
Just this past January popular YouTuber Melanie Ham, known for crafting and DIY videos died less than two years after a rare cancer diagnosis of epithelioid angiomyolipoma.
According to Dr. Damon Reed, an oncologist in Moffitt's Department of Individualized Cancer Management, epithelioid angiomyolipoma is another rare type of sarcoma. It is so rare that even major cancer centers like Moffitt may not treat more than two cases per year.
"Epithelioid angiomyolipoma is too rare to even conduct clinical trials," said Reed. "Each patient needs an individualized plan based on other cases and their particular targets."
Reed said the most common symptoms are a mass in the abdomen or blood in the urine. Those symptoms would lead to a CT scan, and based on those images surgery to remove the kidney might take place. Once removed, the pathology could determine that it is an epithelioid angiomyolipoma diagnosis instead of the far more common and less aggressive version, a benign angiomyolipoma.
“Most of the time surgery cures this but targeted therapies would be the most common route,” said Reed. "These might be guided by a precision medicine consultation after next-generation DNA testing of the tumor to look for rational approaches like we would do at Moffitt."
Moffitt has also been a leader in offering immunotherapy as another treatment option for sarcomas. The Sarcoma Department was the first in the country to open a sarcoma adoptive cell therapy clinical trial solely focused on sarcoma patients receiving an infusion of tumor-infiltrating lymphocytes.
"From the top of your head to your toe, sarcoma can occur in any connective tissue," said Mullinax. "When patients have a lump or mass, they’re often seen first by their primary care doctor or with an orthopedic surgeon. Because these tumors are rare, sarcomas are not commonly encountered by community physicians, making an early referral especially important. As a rule of thumb, any mass the size of a golf ball should be considered for a potential sarcoma diagnosis.
"Moffitt is fortunate to have a comprehensive, multidisciplinary clinic that sees a high volume of sarcoma patients, making the rare diagnosis common for our physicians. It is always important to get an opinion from a specialized center like Moffitt, especially for rare diagnoses like sarcoma."
If you’d like to refer a patient to Moffitt Cancer Center, complete our online form or contact a physician liaison for assistance. As part of our efforts to shorten referral times as much as possible, online referrals are typically responded to within 24 - 48 hours.