Soft tissue sarcoma (STS) is a rare type of cancer that originates in the connective tissues of the body, such as muscles, fat, blood vessels, nerves, tendons, and fibrous tissues. Understanding the disease’s trajectory is important for patients and their families.
Factors Influencing Survival
Several factors influence the prognosis and survival rates for individuals diagnosed with soft tissue sarcoma. The aggressiveness of the tumor, known as its grade, is a primary factor. Tumors are graded as low, intermediate, or high, based on cellular features like mitotic rate (how quickly cells are dividing), cellular differentiation, and the amount of tumor necrosis (dead tissue within the tumor). Higher-grade sarcomas indicate a poorer prognosis because they are more likely to grow quickly and spread.
The size of the tumor also plays a role, with tumors 5 cm or smaller having a better prognosis than larger ones. Larger tumors are associated with a higher risk of recurrence and metastasis. The location of the tumor can also impact outcomes; deep-seated tumors or those in the retroperitoneum (the area behind the abdominal organs) can be more challenging to remove completely and may carry a worse prognosis compared to superficial tumors or those in the limbs.
Soft tissue sarcoma encompasses over 50 different histological subtypes, each with unique behaviors and prognoses. Myxoid liposarcoma may have a better prognosis compared to malignant peripheral nerve sheath tumors (MPNSTs). Leiomyosarcoma, desmoplastic small round cell tumors, and epithelioid sarcoma are also associated with a less favorable outlook. The stage of the cancer at diagnosis is another key factor, as localized disease has a better prognosis than cancer that has spread to regional lymph nodes or distant organs.
A patient’s overall health and age can also influence their ability to tolerate aggressive treatments and their general outcome. Younger patients have a more favorable prognosis than older individuals. Whether the tumor was completely removed during surgery, indicated by clear or “negative” surgical margins, also impacts the likelihood of recurrence and survival.
How Soft Tissue Sarcoma Progresses
Soft tissue sarcoma can progress, leading to severe health complications. Even after initial treatment, the tumor can regrow at its original site, known as local recurrence. This poses challenges for further treatment and can increase the risk of the cancer spreading.
Cancer cells can spread from the primary tumor to distant parts of the body, primarily through the bloodstream. While soft tissue sarcoma can metastasize to almost anywhere in the body, the lungs are the most common site, accounting for approximately 77% to 83% of metastatic cases. Other common sites include bone (30%), distant lymph nodes (18%), and the liver (15%), especially for sarcomas originating in the abdomen. The spread to these organs compromises their function, leading to symptoms like difficulty breathing with lung metastases or liver dysfunction.
Large or strategically located tumors can also compress or invade adjacent organs, nerves, or blood vessels. A tumor in the retroperitoneum might press on the kidneys or digestive tract, leading to organ dysfunction, pain, or even internal bleeding. This direct invasion can impact quality of life and organ function.
Aggressive treatments for STS, such as extensive surgery, radiation therapy, and chemotherapy, can have severe side effects. These complications can include infections, organ damage, or severe fatigue. As the cancer progresses, patients may experience systemic effects such as cachexia, a severe wasting syndrome characterized by muscle loss and weight loss, fatigue, and immune suppression. These systemic effects make the body more vulnerable to infections and other complications.
Supportive Care for Advanced Disease
Supportive care, also known as palliative care, focuses on alleviating symptoms and improving the quality of life for individuals with advanced or incurable soft tissue sarcoma, as well as their families. This type of care can be introduced at any stage of the illness, not just at the end of life, and can be provided alongside active cancer treatments like chemotherapy or radiation.
A primary focus of palliative care is pain management, which involves using various medications and interventions to control cancer-related pain effectively. Beyond pain, supportive care addresses other common symptoms that can significantly impact a patient’s well-being, such as fatigue, nausea, loss of appetite, and shortness of breath. Managing these symptoms proactively helps maintain comfort and daily function.
Psychological and emotional support is also a significant component of palliative care, addressing the anxiety, depression, and distress that often accompany a serious diagnosis. This support can involve counseling for both patients and their families, helping them cope with the emotional burden of the disease. Spiritual care and connecting patients with social services for practical needs like financial assistance or community resources are also part of this holistic approach.
As the disease progresses and active treatment options become limited, the focus of care may shift more entirely towards comfort and dignity, transitioning into end-of-life care and hospice services. Hospice care provides specialized support for individuals in the final stages of their illness, emphasizing comfort, symptom control, and emotional and spiritual well-being in a supportive environment. This transition ensures that patients receive compassionate care tailored to their evolving needs, prioritizing their comfort and quality of life.