Aditi Dhir, MD, a clinical assistant professor in the Department of Pediatrics, Division of Hematology/Oncology at Sylvester Comprehensive Cancer Center, University of Miami Health System, discusses ongoing efforts in epithelioid and synovial sarcoma.

Survival rates in patients with recurrent or metastatic disease remain dismal and underscore the need for more efficacious treatment options, according to Dhir. However, it is difficult to draw conclusions from individual studies due to the rarity of epithelioid and synovial sarcomas, Dhir notes. Multi-institutional and global efforts are needed to advance knowledge within the field and push the treatment paradigm forward.

Ongoing efforts of cooperative groups across the globe are showing promise and may be successful in identifying a cure for this patient population, Dhir concludes.

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Aditi Dhir, MD, discusses ongoing efforts in epithelioid and synovial sarcoma.


Dr. Dhir on Ongoing Research Efforts in Epithelioid and Synovial Sarcoma

Aditi Dhir, MD, a clinical assistant professor of the Department of Pediatrics, Division of Hematology/Oncology at Sylvester Comprehensive Center, University of Miami Health System, discusses risk-based stratification in patients with epithelioid and synovial sarcoma.

Similar to most sarcomas, a multimodal approach is often utilized in the treatment of patients with epithelioid and synovial sarcoma, Dhir explains. However, the standard-of-care treatment is a wide surgical resection of the tumor with negative margins, Dhir says.

Treatment is often based on risk stratification. For example, the Children's Oncology Group organized the non-randomized ARST0332 trial, which utilized a risk stratification approach for treating pediatric patients. Patients were classified as being low or high risk, based on whether their tumor was resectable, whether they had any metastases, as well as the grade and size of their tumor, Dhir adds.

For low-risk patients, surgery alone is often a viable treatment option, according to Dhir. However, due to the high rate of local recurrence and metastases for patients with epithelioid and synovial sarcoma, systemic therapy in the form of radiation therapy or chemotherapy is often needed, Dhir concludes.

Aditi Dhir, MD, discusses risk-based stratification in patients with epithelioid and synovial sarcoma.


Dr. Dhir on Risk-Based Stratification in Epithelioid and Synovial Sarcoma

Aditi Dhir, MD, clinical assistant professor, Department of Pediatrics, Division of Hematology/Oncology, Sylvester Comprehensive Cancer Center, University of Miami Health System, discusses the challenges of diagnosing patients with sarcoma.

Although many sarcomas are aggressive, they can be slow growing because they are rooted in deeper tissues, says Dhir. As such, the average time to diagnose sarcoma ranges from a few months to 1 year, depending on the tumor location and associated symptoms, Dhir explains. 

Notably, when a sarcoma diagnosis is confirmed, it is important to obtain a core biopsy rather than an excisional biopsy. Core biopsies can shed light on the true extent of the disease and minimize the risk of incomplete resection or positive surgical margins, which can influence treatment, concludes Dhir.

Aditi Dhir, MD, discusses the challenges of diagnosing patients with sarcoma.  



Dr. Dhir on Diagnostic Challenges in Sarcoma 

Aditi Dhir, MD, clinical assistant professor, Department of Pediatrics, Division of Hematology/Oncology, Sylvester Comprehensive Center, University of Miami Health System, discusses modifying treatment for adults versus pediatric patients with epithelioid sarcoma.

Although no head-to-head comparisons of treatment options between adults and pediatric patients exist, there are subtle differences in dosing, as well as preferred regimens between these populations, Dhir explains. Often, treatment selection is driven by tolerance and potential toxicities.

For example, the frontline combination of gemcitabine and docetaxel is preferred for adult patients, whereas pediatric patients are more likely to receive ifosfamide plus doxorubicin, says Dhir. Notably, some regimens, such as radiation therapy, may lead to long-term toxicities, including risk of secondary malignancy or neurocognitive decline. As such, these potential toxicities limit or prohibit the use of high-dose radiation therapy among pediatric patients, explains Dhir. Conversely, adult patients with baseline comorbidities often cannot tolerate very high-dose chemotherapy that is given to pediatric patients, concludes Dhir.

Aditi Dhir, MD, discusses modifying treatment for adults versus pediatric patients with epithelioid sarcoma.  


Aditi Dhir, MD

Articles

Dr. Dhir on Ongoing Research Efforts in Epithelioid and Synovial Sarcoma

Dr. Dhir on Risk-Based Stratification in Epithelioid and Synovial Sarcoma

Dr. Dhir on Diagnostic Challenges in Sarcoma

Dr. Dhir on Modifying Treatment for Adults Versus Pediatric Patients With Epithelioid Sarcoma