Raising awareness for effective patient care

Adam C. Berger, MD, FACS

Chief of Melanoma and Soft Tissue Surgical Oncology

Rutgers Cancer Institute

RWJBarnabas Health

Sarcomas, often referred to as the “forgotten cancers,” are a rare and heterogeneous group of malignant tumors that arise from mesenchymal tissues such as bone, muscle, fat, and connective tissue. The American Cancer Society1 estimates that approximately 13,590 new cases of soft tissue sarcoma will be diagnosed in 2024.

Despite its rare nature, sarcoma is extremely challenging to diagnose in a timely manner and treat effectively due to its diverse manifestations. To increase awareness and understanding of sarcoma among healthcare professionals, it is necessary to focus on the epidemiology, clinical manifestations, risk factors, and latest advances in treatment of the disease.

Epidemiology and Pathophysiology

Sarcomas can occur anywhere in the body, including bones, muscles, tendons, cartilage, and other connective tissues, from head to toe. Detecting sarcomas can be difficult because they can occur in many different locations (often deep) and symptoms are often subtle. Sarcomas can occur in people of any age, but certain types, such as Ewing's sarcoma, occur primarily in children and adolescents during their growth spurts.

Clinical findings

The clinical symptoms of sarcomas vary depending on the size, location, and grade of the tumor. Often, sarcomas present as a painless lump or swelling, but may be painful if the tumor presses on adjacent nerves or muscles. Other symptoms include limited range of motion and generalized symptoms such as weight loss and fatigue if the disease is significantly advanced. The most common sites for tumor growth are the extremities, neck, chest, shoulders, abdomen, and lower back. Educating patients about the signs and symptoms of sarcoma and the importance of early medical evaluation will facilitate timely diagnosis and treatment.

Tumor microenvironment background with cancer cells, T cells, nanoparticles, molecules and blood vessels. Oncology research concept: © rataosk – stock.adobe.com

Risk factor

Certain risk factors are associated with an increased chance of developing these malignancies. The main risk factors for sarcoma include:

1. Year: Certain types of sarcomas, such as Ewing's sarcoma and rhabdomyosarcoma, occur primarily in children and adolescents, often during periods of rapid growth and development. Other types of sarcomas, such as liposarcoma and leiomyosarcoma, are more commonly diagnosed in adults, especially those over the age of 50.
2. Radiation ExposurePrevious exposure to ionizing radiation, especially for the treatment of other cancers, is a well-documented risk factor for the development of secondary sarcomas. Risk is dose-dependent; the higher the dose of radiation, the higher the risk.
3. Genetic disordersDue to a genetic mutation, people with Li-Fraumeni syndrome are at significantly increased risk of developing various cancers, including sarcoma. TP53 Survivors of hereditary retinoblastoma caused by a genetic mutation RB1 People with the gene mutation are at increased risk of developing osteosarcoma and other soft tissue sarcomas.
4. Chemical exposureLong-term exposure to certain chemicals, including vinyl chloride, arsenic, and dioxin, is known to increase the risk of developing angiosarcoma and other soft tissue sarcomas.
5. Chronic lymphedema: Lymphedema predisposes the patient to developing angiosarcoma in the affected limb.

Understanding these risk factors is crucial for healthcare professionals to identify high-risk individuals and implement appropriate surveillance and prevention strategies.

Treatment

Treatment strategies for sarcomas are highly individualized based on the tumor's histologic subtype, location, size, stage, and the patient's overall health. Treatment options include:

• Surgery: The cornerstone of sarcoma treatment, enabling complete resection with negative margins.
• Radiation therapy: It is used to shrink tumors or to eradicate residual disease after surgery to prevent recurrence.
• chemical treatment: Used for certain high-grade sarcomas and metastatic disease.
• Targeted Therapies and ImmunotherapyNew treatments, especially for cases where traditional treatments are not effective.

Treatment and Research Advances

Sarcoma is a complex and rare disease, so it is important to receive specialized care. At Rutgers Cancer Institute and RWJBarnabas Health, we continue to pursue precision medicine initiatives tailored to individual patient profiles, with a focus on improving individualized treatment strategies and early detection methods for metastatic sarcoma. Clinical Trials at Rutgers Cancer Institute² We are exploring innovative approaches, including targeted and immunotherapies, with the goal of increasing the effectiveness of treatments and expanding the therapeutic armamentarium available to combat this diverse group of cancers.

Although sarcomas are rare, increased awareness and a multidisciplinary approach are needed to improve treatment outcomes. Understanding the risk factors, clinical findings and evolving treatment options allows healthcare professionals to play a key role in early detection and management. Raising awareness and promoting research is essential to ensure sarcoma does not remain the “forgotten cancer”.

reference:

1. Soft Tissue Sarcoma. American Cancer Society. Accessed July 17, 2024. https://tinyurl.com/2s4mckrj

2. Clinical Trials Overview. Rutgers Cancer Institute. Accessed July 17, 2024. https://www.cinj.org/clinical-trials/clinical-trials-overview