SARCOMAS: AN OVERVIEW WITH DR BREELYN WILKY

Presenter:
Breelyn Wilky, MD, University of Colorado School of Medicine

Conference:
2024 Meeting of the Oncology Nursing Society (ONS) Metro Denver Chapter

At the 2024 Meeting of the Oncology Nursing Society (ONS) Metro Denver Chapter, Dr Breelyn Wilky from University of Colorado School of Medicine presented an in-depth overview of sarcomas, a rare and heterogeneous group of cancers that accounts for only about 1% of adult malignancies, but approximately 15% of pediatric cases. Sarcomas can develop in almost any part of the body, from the limbs to internal organs, and can affect individuals across all age groups. Unlike most other cancers, sarcomas typically metastasize to the lungs rather than lymph nodes, and certain abdominal sarcomas may not manifest as palpable masses, instead presenting with symptoms like unexplained weight gain or bloating, which can complicate early detection.

Dr Wilky emphasized the critical role of a multidisciplinary approach in sarcoma care, involving a team of specialists, including surgeons, medical and radiation oncologists, pathologists, and radiologists. Tumor boards are essential for case discussions in sarcoma, particularly for complex or advanced cases. Oncology nurses play an indispensable role in the patient journey in sarcoma, assisting with the management of side effects from intensive chemotherapy, providing emotional support, and coordinating comprehensive care.

To illustrate the complexities of sarcoma diagnosis and treatment, Dr. Wilky discussed two clinical cases. The first case involved a 57-year-old firefighter who initially presented with upper arm pain, suspected to be a biceps tear, which was later diagnosed as a large soft tissue sarcoma (myxofibrosarcoma). Through a combination of chemotherapy, radiation, and limb-sparing surgery, the patient achieved remission and has remained cancer-free with full arm function at five years post-diagnosis. The case example highlighted the importance of using neoadjuvant chemotherapy to shrink tumors, which, in turn can facilitate more effective surgical resection.

Dr Wilky’s second case example underscored the challenges in treating metastatic sarcoma. A 28-year-old woman with a prior history of melanoma developed a suspicious breast mass after childbirth. Despite multiple surgeries, chemotherapy, and radiation, her disease recurred and eventually spread. Genetic testing identified a rare mutation, enabling her to receive targeted therapy with imatinib, which stabilized her disease temporarily. However, Dr. Wilky noted that metastatic sarcomas remain quite challenging to treat, with many therapies focused on enhancing quality of life rather than achieving cure.

Dr. Wilky reviewed the standard chemotherapy protocols for sarcoma, which primarily rely on doxorubicin and ifosfamide, both of which have been in use for several decades. While these agents can offer survival benefits, they do carry significant side effects. Doxorubicin, for example, has known cardiotoxic effects, which necessitate regular heart monitoring, while ifosfamide is associated with neurotoxicity, which can range from mild confusion to severe hallucinations. Dr Wilky noted that oncology nurses are pivotal in managing these toxicities, by administering supportive care such as hydration and growth factors, and educating patients on the potential risks of treatment and toxicities that may occur. For patients on doxorubicin, for example, dexrazoxane (Zenicard) can be used to mitigate cardiotoxicity, which can extend the feasibility of continued treatment.

Dr. Wilky highlighted some more recent advances with targeted therapies, which have shown promise in specific sarcoma subtypes. For example, gastrointestinal stromal tumors (GISTs) respond well to tyrosine kinase inhibitors (TKIs) such as imatinib, which significantly improve outcomes. Many sarcoma types, however, lack actionable mutations, which limits the application of targeted therapy. In this regard, Dr. Wilky emphasized the importance of genetic testing in metastatic cases to identify rare mutations, which may allow sarcoma patients access to targeted therapies and/or enrollment in clinical trials.

Another promising area of research is immunotherapy, although its effectiveness varies across different sarcoma subtypes. Checkpoint inhibitors, such as anti-PD1 and anti-CTLA-4 therapies, for example have shown rather limited efficacy for most sarcomas, with the notable exception of cutaneous angiosarcoma, which appears to respond favorably. Dr. Wilky presented a case involving a patient with cutaneous angiosarcoma who achieved a complete response after receiving a low-dose checkpoint inhibitor in a clinical trial, demonstrating the potential of immunotherapy in specific sarcoma types. She noted that further research is necessary to identify biomarkers that could predict patient response to these therapies.

Sarcomas remain some of the most challenging cancers to treat, with progress often hindered by limited research funding, limited numbers of patients, and a lack of effective therapies for certain subtypes. Dr. Wilky emphasized that each sarcoma case is unique, necessitating individualized care. She noted that referral to high-volume sarcoma centers is crucial, as these centers provide patients with access to expert care, clinical trials, and the latest treatment options. Clinical trials are especially important for advancing sarcoma care, offering opportunities to explore novel therapies and learn from diverse patient experiences.

Summarizing her presentation, Dr. Wilky underscored that sarcoma treatment requires a collaborative, multidisciplinary approach. Oncology nurses play a vital role in this process, supporting patients in managing treatment side effects, navigating complex care pathways, and maintaining quality of life. Despite the substantial challenges associated with sarcoma care, advancements in genetic testing, targeted therapies, and immunotherapy provide new hope for patients with these rare cancers.

Speaker Disclosure Information: Dr. Wilky reported no disclosures for this presentation.


You can see Dr Wilky’s full presentation from the 2024 Meeting of the Oncology Nursing Society (ONS) Metro Denver Chapter here.

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