Sarcoma Awareness Month 2024: Recognizing Symptoms, Scanning Patients Key to Diagnosis

Adam C. Berger, MD, FACS

Sarcoma symptoms typically manifest as a mass or lump and may cause tingling or, according to Adam C. Berger, MD, FACS, who added early diagnosis of sarcoma remains a challenge since symptoms may not present until the tumor is large enough.

“The biggest thing is awareness. If people have a small lump or have a nagging symptom that doesn't go away, they should go to their doctor and try to get a scan done,” Berger explained in an interview with OncLive^® during Sarcoma Awareness Month, which occurs in July each year.

In the interview, Berger discussed common symptoms associated with a sarcoma diagnosis; expanded on the various subtypes of sarcomas and how symptoms may differ with each type; and highlighted ongoing research within the sarcoma space.

Berger is the chief of melanoma for the Melanoma and Soft Tissue Surgical Oncology Program at the Rutgers Cancer Institute of New Jersey and a professor of surgery at Rutgers Robert Wood Johnson Medical School in New Brunswick.

OncLive: What symptoms do you look for when considering sarcoma as a possible diagnosis?

Berger: The symptoms often are related to a feeling mass lesion or a lump. [Patients may also experience] tingling if [the tumor] is pushing on a nerve. Sometimes patients will get swelling. If [the sarcoma] is in the arm or a leg, it could cause swelling not only at that site but distal to that site if it's blocking blood return or lymph return.

Occasionally, there will be pain associated with [sarcomas]. However, a lot of times, they grow slowly, and they're deep, so they don't get noticed early. They get noticed when they grow big enough that somebody can feel them. I'd say the most common symptoms are feeling a mass or sometimes feeling pain.

Do symptoms vary based on the type of sarcoma a patient may have?

There are bony sarcomas, which I don't typically treat; usually, orthopedic oncologists take care of [bony sarcomas]. Those often cause pain in bones, and sometimes they can cause fractures in the bones.

We also see soft-tissue sarcomas, and there is a large variety of soft-tissue sarcomas. They can arise from fatty elements, meaning liposarcomas; they can arise from muscle, so leiomyosarcoma or rhabdomyosarcoma; [Sarcomas can] arise from other connective tissues or fibrous tissue. They can arise from cell-binding blood vessels, nerve sheets, or nerve roots.

We see sarcomas in the extremities, so the arms and legs; sometimes we see them in the chest wall in the bones or the muscle of the chest. Then we see a lot of retroperitoneal sarcomas, which are sarcomas that [present] in the abdomen behind where all the organs are. Those can get quite large because they can just keep taking up space without causing a lot of symptoms until they get very large.

What are some of the primary challenges in diagnosing sarcoma at an early stage? What strategies do you employ to overcome these challenges in your own care?

The biggest challenge is that they often grow for long periods of time before any symptoms happen. There is no routine surveillance or screening that's done for sarcoma, especially since they're so rare. It's not like you can do a mammogram or a CAT scan of the chest for people at high risk because we don't have a lot of high-risk groups due to [sarcoma being] a rare cancer. Oftentimes, sarcomas are diagnosed later because they have to grow to a certain point to cause any symptoms.

Sometimes we find them incidentally, such as when patients have scans for other reasons. For example, somebody might have a kidney stone, and they get a scan of their abdomen to look for kidney stones, and then we find a retroperitoneal sarcoma that [wasn’t causing] any symptoms. That's just luck, though, unfortunately.

What advancements have been made in the treatment of sarcomas in recent years?

The good thing and the bad thing about sarcomas is that there is diversity to them. There are probably over 60 different types of sarcomas now, and a lot of that is based on molecular alterations that they have found that are specific for [different] kinds of sarcomas. That makes it complicated, but [these alterations] make things interesting because these are [aberrations] that could be targeted with certain treatments that are being developed.

We also have sarcomas where we're able to give immunotherapy [to patients] that we were never able to in the past. Immunotherapies have become important over the last 5 to 10 years and have become the standard of care in a lot of cancers; however, [these agents] have only recently started being used for [patients with] sarcomas.

There was a recent study [presented at] the 2024 ASCO Annual Meeting [the phase 2 SU2C-SARC032 trial (NCT02301039)] looking at undifferentiated pleomorphic sarcoma, which is a very aggressive variant, that showed that the responses of [patients with] those sarcomas to [neoadjuvant] immunotherapy [with pembrolizumab (Keytruda)] plus radiation was dramatic. That is a treatment that we can start to use for something that otherwise we would give standard chemotherapy, [which] has not worked very well.

There's also another medication— nirogacestat [Ogsiveo]—approved [by the FDA] for [patients with [progressing] desmoid tumors [who require systemic treatment]. It's a targeted treatment for desmoid tumors, which are hard [to treat] because when they are resected, they often recur, and they're often seen in younger patients. They can get quite large, and surgery for them can be quite disfiguring. A lot of times for desmoid tumors, we don’t operate on them unless they start to grow. They don't respond to traditional chemotherapy; therefore, [nirogacestat] is a new FDA-approved medication for [patients with] desmoid tumors.

Are there any ongoing studies at Rutgers Cancer Institute for patients with sarcomas?

We have a few clinical trials open. We just opened [the phase 3 STRASS 2 trial (NCT04031677)], which is an international clinical trial looking at giving chemotherapy preoperatively for patients with retroperitoneal sarcomas to see whether we can decrease the rate of recurrence and the survival rates for [those with] leiomyosarcomas and differentiated liposarcomas, which are 2 of the more common retroperitoneal sarcomas that have a very high rate of recurrence. Although we may not have many other trials currently open, we're always looking for new, innovative ways to treat [patients with sarcomas].

Reference

Mowery YM, Ballman KV, Hong AM, et al. SU2C-SARC032: a randomized trial of neoadjuvant RT and surgery with or without pembrolizumab for soft tissue sarcoma. J Clin Oncol. 2024;42(suppl 16):11504. doi:10.1200/JCO.2024.42.16_suppl.11504