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Sarcoma Deserves More Attention in the Spotlight

CURECURE Fall 2022
Volume 21
Issue 03

Even in the public health shadows of the more common cancers like breast cancer, for the few but strong patients with a sarcoma diagnosis, we are obligated to spread awareness, restore hope and with specialist care, ensure the best possible outcomes.

Sarcoma is a rare cancer of the soft tissues and bone that often just misses the spotlight for the general public, but certainly has quite an impact for those affected by it.

July is Sarcoma Awareness month, but all throughout the year, sarcoma deserves our attention!

The epidemiology of sarcoma is very different from the more common cancers. Every year, 17,000 people in the United States are given a new diagnosis of sarcoma, a number that is at least 20-fold less than breast cancer. While rare, anyone can get sarcoma.

Certain types of sarcoma are more common in children (e.g. rhabdomyosarcoma), but this cancer can impact those in any age group. Sarcoma can occur in both the rich and the poor, irrespective of access to health care, and regardless of gender or race.

Some hereditary cancer syndromes (e.g. Li-Fraumeni, neurofibromatosis) or prior history of radiation can predispose a person to developing specific sarcomas; however, common risk factors such as smoking, obesity, diet or alcohol intake have no clear association with sarcoma. While colonoscopy and blood levels of PSA are useful in screening for colon and prostate cancer respectively, there are currently no validated tools for early detection of sarcoma.

Sarcoma often presents in subtle ways. It can be a painless lump that has been slowly growing over the last year: “I’ll bring it up with my doctor at the next annual visit.” And then at that visit, it is not uncommon for the physician to inadvertently dismiss it: “we’ll watch it for a few more months.” When a work-up is initiated, the diagnosis is not always straightforward.

The radiologist reading the MRI or CT reports that the mass is indeterminate: “likely a lipoma, however liposarcoma cannot be ruled out.” A needle biopsy may be performed, and the pathologist is also unclear about the diagnosis either due to inadequate sampling or simply, unfamiliarity with sarcoma.

In fact, it is important to recognize that sarcoma encompasses almost 100 distinct types and subtypes based on the body tissue of origin (e.g. fat, muscle, blood vessel, etc.) and unique genetic features of the tumor cells. Therefore it may be more appropriate to think of sarcoma as a group of cancers as opposed to a single entity.

Unfortunately, with all of these potential obstacles to diagnosis, the net result for the sarcoma patient is often delay before any treatment is given.

Treatment for sarcoma is incredibly complex; referral to a specialist is critical. The treatment for sarcoma needs to be individualized for each patient. Ideally, a patient’s case should be discussed at a multidisciplinary “tumor board” among specialists in this cancer from radiology and pathology to verify the diagnosis and sarcoma type, as well as from surgical, medical and radiation oncology to mutually decide on the best course of treatment.

When sarcoma has not spread to other parts of the body, surgery is an option and often the main form of treatment.

The surgical oncologist who specializes in sarcoma balances the technical aspects of performing a safe and complete tumor removal (or resection) with a deep understanding of the expected disease behavior which can in fact, often nuance the surgical approach.

In some cases, there may also be a benefit to giving radiation or chemotherapy either before or after surgery. For some sarcoma types, specific targeted drug therapies or immunotherapy may be more ideal than chemotherapy. A medical oncologist who specializes in sarcoma can discuss these options and provide guidance to a relevant clinical trial, when appropriate.

With the complexities in the diagnosis and treatment of sarcoma, the patient is encouraged to take a proactive role in their care. Getting to a sarcoma specialist or multidisciplinary sarcoma center is a very important first step. A multitude of studies have shown improved survival for patients with sarcoma when they are treated by a specialist, a concept that would seem intuitive for a rare, complex disease.

While high patient volume is often used as a benchmark to denote specialization, there are also other factors such as dedication to sarcoma (versus other cancers) and active contribution to the field (with research and clinical trials) that should also be considered. The patient can directly facilitate a more effective consultation by having relevant records available and organized.

For a new diagnosis, this can include radiology reports along with the actual images to view (e.g. on a CD); pathology reports and in some cases, the actual tissue slides and paraffin blocks which may be used for re-review by a sarcoma pathologist. For patients who have had prior treatment, operative reports from the surgeon and treatment summaries from the radiation oncologist and medical oncologist are useful. In patients with a particularly extensive history, a concise outline of treatments given can be helpful.

Overall, sarcoma is an aggressive cancer. Although there are exceptions, for the majority of patients, sarcoma can indeed be lethal. At the same time, for the patient, sarcoma can be extremely anxiety provoking.

The rarity of the disease, the relative lack of knowledge about sarcoma and in some cases, inappropriate initial treatment by a non-specialist (e.g. a “whoops procedure”) further exacerbates the situation.

So, why sarcoma?

Because even in the public health shadows of the more common cancers, for the few but strong patients fighting sarcoma, we are obligated to spread awareness, restore hope and with specialist care, ensure the best possible outcomes.

William Tseng, MD, is a sarcoma surgical oncologist at City of Hope National Medical Center in Duarte, California, and serves on the Board of Directors and Medical Advisory Board for the Sarcoma Alliance.

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