Sarcoma is a rare group of malignant tumors. It develops in connective and supportive tissues, including the muscle, fat, nerves, cartilage, blood vessels, and bone from the top of the head all the way down to the toes. Despite its aggressive nature, it receives far less public attention and research funding than more common malignancies, thus earning the nickname “forgotten cancer.” This lack of recognition, unfortunately, delays diagnoses and limits access to less invasive treatments. This article compiles evidence-based facts about sarcoma to raise public awareness.
1. There are 100 types of sarcomas (and counting)
Sarcoma is not a single disease but a broad category of cancers. Oncologists classify them into two main groups: soft tissue sarcomas and bone sarcomas. Each group has multiple subtypes, with differences in the tissue affected, cellular structure, rate of growth, and how the disease spreads.
The muscle, fat, fibrous tissue, blood vessels, and nerves are where soft tissue sarcomas form, while bone sarcomas originate in the cells that form bone. Within these categories, some of the more common subtypes include:
- Osteosarcoma occurs in bone-forming cells, most often in the long bones of the arms and legs.
- Ewing sarcoma affects the bone or the surrounding soft tissue. It’s more prevalent in adolescents and young adults, although a small percentage appears in adults over 20.
- Liposarcoma arises from fat cells and can occur in any area of the body, though the thighs and abdominal cavity are more common sites.
- Leiomyosarcoma originates in smooth muscle tissue, such as that in the uterus, gastrointestinal tract, or blood vessels.
- Synovial sarcoma develops near joints, particularly in the arms or legs.
- Angiosarcoma affects the cells lining the blood vessels or lymphatic vessels.
Rarer forms include chondrosarcoma, peripheral nerve sheath tumor, and rhabdomyosarcoma in adults. Each subtype has its own diagnostic criteria, treatment approach, and prognosis. For this reason, accurate histological identification by a pathologist with sarcoma expertise is critical before starting treatment.
2. Sarcoma affects more children than adults
Sarcoma is rare in the general adult population, accounting for 1% of all adult cancer diagnoses. In contrast, it’s responsible for a higher percentage of cancers in children and adolescents. Data from the National Cancer Institute shows that approximately 15% of malignancies diagnosed in individuals under 20 are sarcomas of the bone or soft tissue.
Every year, an estimated 1,500 to 1,700 children and adolescents in the United States receive a diagnosis of sarcoma. Treatment for this age group requires pediatric oncologists, orthopedic surgeons, radiation oncologists, and rehabilitation specialists to collaborate. In addition to treating the cancer, healthcare teams must address the potential consequences of therapy on growth, development, and long-term health.
3. Many doctors have never met or treated a person with sarcoma
Sarcoma’s extreme rarity means that many physicians, including general oncologists, encounter very few cases during their careers. Some may only see one or two patients with this diagnosis in a year. Limited exposure can affect familiarity with the disease’s clinical signs, diagnostic pathways, and treatment protocols.
Patients achieve the best outcomes when they receive care at a cancer center with a dedicated sarcoma program. These facilities bring together specialists in medical oncology, surgical oncology, orthopedic oncology, radiation oncology, and pathology who have devoted their medical careers to understanding and treating this disease. Access to this level of expertise improves accuracy in diagnosis and effectiveness in treatment planning.
4. The causes of sarcoma are still a mystery
Researchers have not identified a single cause of sarcoma. Since the disease includes over a hundred subtypes with distinct cellular characteristics and genetic patterns, it complicates the search for definitive origins.
In hindsight, several factors appear to increase the likelihood of developing sarcoma. Inherited genetic syndromes, such as Li-Fraumeni syndrome, familial retinoblastoma, and neurofibromatosis type 1, can predispose an individual to specific subtypes. Acquired genetic mutations within tumor cells also play a role in disease development, although the triggers for these changes are not fully understood.
Some cases in adults develop years after cumulative environmental or occupational exposures. A systematic review and meta-analysis of 85 publications linked prolonged contact with certain chemicals, such as dioxins and vinyl chloride monomers, to higher sarcoma incidence and mortality rates. Prior high-dose radiation therapy to treat other conditions may also cause sarcoma in the irradiated area years later.
5. Previous radiation treatment may lead to sarcoma
As discussed above, exposure to high-dose radiation may cause sarcoma years after receiving treatment for another condition. Radiation damages the DNA of healthy cells, which can trigger mutations that can cause cancer.
Radiation-induced sarcoma is rare, but it has profound implications for long-term survivorship. The risk increases with higher radiation doses or when treatment targets a larger area of the body. Younger patients are even more at risk because developing tissues are vulnerable to DNA damage. These sarcomas often appear a decade or more after the original treatment, making lifelong monitoring crucial for early detection.
Modern radiation therapy techniques aim to limit exposure to surrounding healthy tissues. Even with these advancements, oncologists must weigh the benefits of radiation against the risk of developing a secondary cancer, such as sarcoma.
Patients with a history of radiation exposure should report any new lumps, swelling, or unexplained pain in previously treated areas. Imaging tests and biopsies can confirm or rule out sarcoma and determine the best intervention.
6. Sarcomas can be asymptomatic
Early-stage sarcoma may not cause any symptoms. In some patients, the first indication is a lump or swelling in the affected area. This lump is typically painless, which may delay seeking medical evaluation.
Symptoms vary by the tumor’s location and size. Abdominal tumors may cause pain, a sense of fullness, or digestive changes. Tumors that press on nerves can cause numbness or weakness in a limb. Rarely, sarcoma leads to recurrent pain, nausea, vomiting, diarrhea, or unexplained bleeding.
Because many benign conditions can cause similar signs, physical examination alone cannot confirm a sarcoma diagnosis. Imaging studies such as MRI or CT scans help identify suspicious masses, but only a biopsy can confirm the presence of cancerous cells. For this reason, patients should report new or unexplained lumps to their physician so that appropriate diagnostic tests can be ordered.
7. Detecting sarcoma early may prevent amputation
Surgeons perform an amputation in cases where the tumor is too large or has invaded major nerves and blood vessels. However, when detected early and confined to one area, surgery can remove the tumor and preserve as much healthy tissue as possible.
Depending on the subtype and location, oncologists may recommend chemotherapy, radiation therapy before surgery to shrink the malignant growth, or afterward to reduce the risk of recurrence. Immunotherapy for sarcoma is also an option in many cases, and New Hope Unlimited offers this approach as part of its individualized cancer treatment programs.
If sarcoma has already metastasized to other areas, treatment may transition toward relieving symptoms and controlling disease progression. Systemic therapies, which include chemotherapy, targeted therapy, or immunotherapy, may slow growth and reduce complications. Palliative care specialists can alleviate pain and improve quality of life during treatment.
8. Genetics-focused research advances sarcoma treatments
Some sarcomas occur due to changes in specific genes, others to problems in how cells send and receive growth signals. These differences explain why certain treatments work for some patients but not for others.
Dr. Vivek Subbiah, who led a successful clinical trial using Radium-223 for relapsed osteosarcoma, explains that identifying the exact drivers behind each subtype can help medical researchers design treatments that target those changes. Studies have already found mutations in genes such as TP53 and RB1, changes in signaling pathways like PI3K/AKT and MAPK, and, in some cases, chromosome changes that fuse two genes together. These findings form the basis for developing new treatments.
Because sarcoma is rare, doctors and researchers work together across cancer centers to share tumor samples and patient data. This teamwork speeds up research and brings new treatment options to patients sooner.
9. Misdiagnosis is common
Sarcoma’s symptoms can resemble those of more common conditions. In its early stages, a tumor may cause a painless lump or swelling, which many patients dismiss as a minor injury or cyst. This delay in seeking medical evaluation allows the cancer to grow and spread.
Symptoms vary based on the malignant tumor’s location and size. Some examples include:
- Abdomen or pelvis: Pain, an unusual feeling of fullness before a meal or after having a few bites, digestive changes, or urinary symptoms.
- Arms or legs: Numbness, weakness, or reduced mobility from nerve or muscle compression.
- Chest: Shortness of breath, chest pains, or cough.
- Head and neck: Difficulty swallowing, changes in vision, or a persistent lump.
- Digestive tract: Nausea, vomiting, diarrhea, or bleeding.
- Rare cases: Recurrent pain, nausea, vomiting, diarrhea, or unexplained bleeding.
Because sarcoma is rare, it’s sometimes mistaken for benign growths, muscle injuries, or other soft tissue disorders. The opposite is also extremely common. About 42.5% of patients with a rare condition known as arteriovenous malformation receive an incorrect sarcoma diagnosis, which leads to unnecessary anxiety and, in rare cases, unwarranted treatments.
In the absence of MRI or CT scans and a biopsy, patients risk receiving ineffective treatment for a condition they do not have. A coordinated review by radiologists, pathologists, and oncologists helps ensure the diagnosis is correct before starting treatment.
10. Some sarcoma subtypes grow and spread faster
Sarcomas grow and spread in multiple ways. High-grade tumors progress faster than low-grade forms, with a higher likelihood of spreading to distant organs before detection.
Aggressive subtypes include:
- Angiosarcoma
- Synovial sarcoma
- Undifferentiated pleomorphic sarcoma
- Rhabdomyosarcoma
The pathway of metastasis varies depending on tumor biology and location. For instance, most sarcomas spread through the bloodstream, usually toward the lungs. Others travel through the lymphatic system, particularly tumors that develop in areas with dense lymph node networks, such as the groin, armpits, and neck. In some cases, cancer cells reach multiple organs before doctors detect the primary tumor.
The tumor’s size, depth, and grade affect how quickly the disease advances. Larger tumors with higher mitotic activity tend to progress faster. Subtypes with specific genetic changes, such as EWSR1 translocations in Ewing sarcoma, may behave more aggressively than others.
Patients diagnosed with aggressive types of sarcoma benefit from a multidisciplinary approach introduced early and coordinated across oncology specialties. As for patients with a high risk of recurrence or metastasis, regular monitoring is necessary to detect any new growths and begin treatment as soon as possible.
11. Surgery is the primary treatment for localized sarcoma (but it’s not the only option)
Surgery removes the tumor and a margin of surrounding healthy tissue to lower the chance of local recurrence. Depending on the growth’s location and size, surgeons may opt for limb-sparing methods instead of amputation. They may also remove the biopsy tract to prevent cancer cells from spreading into adjacent tissue.
For high-risk tumors, oncologists may combine chemotherapy with radiation. Preoperative radiotherapy can shrink a tumor and make surgery less invasive, while postoperative radiation can help eradicate remaining cancer cells in the body.
Immunotherapy is another option, which we will discuss in detail below.
12. Immunotherapy and targeted therapy are modern treatment methods
Immunotherapy and targeted therapy are precision oncology protocols that aim to address the disease in ways traditional chemotherapy and radiation cannot.
How Does Immunotherapy for Sarcoma Work?
Immunotherapy activates the patient’s immune system to detect and attack cancer cells. Certain sarcomas produce proteins that help them hide from immune surveillance, which makes them harder to treat with standard methods. Immunotherapy drugs block these signals, allowing immune cells to recognize and destroy tumor cells.
Checkpoint inhibitors are one example, targeting molecules such as PD-1 or PD-L1 to enhance immune activity. Clinical studies show that while not all sarcomas respond, several subtypes do and effectively control tumor growth and prolong survival. Other immunotherapy approaches include cancer vaccines and engineered T-cell therapies, though these are in earlier stages of development for sarcoma.
What Is Targeted Therapy?
Targeted therapy uses drugs designed to interfere with specific genetic mutations or molecular pathways that drive tumor growth. For example, some sarcomas have changes in the KIT or PDGFRA genes, which make them responsive to certain tyrosine kinase inhibitors. Unlike chemotherapy, which affects both healthy and cancerous cells, targeted drugs focus on the tumor’s unique molecular profile.
These treatments may be used alone or combined with other therapies, depending on tumor subtype, stage, and patient tolerance. Periodic imaging and blood tests help assess treatment response and adjust the plan as needed.
The Role of These Therapies in Modern Sarcoma Care
Precision oncology introduces new treatment possibilities for patients who have limited options with surgery, chemotherapy, or radiation alone. Both immunotherapy and targeted therapies are options for advanced or recurrent disease, or when conventional approaches are unlikely to achieve tumor regression. These methods are part of New Hope Unlimited’s integrated strategy aimed at maximizing sarcoma treatment effectiveness and quality of life.
RELATED: Avoid Chemo, Radiation, and Surgery with Immunotherapy
13. Sarcoma research is underfunded compared to common cancers
Compared to more common cancers such as breast, lung, skin, or colorectal cancer, sarcoma receives less than 1% of research funding worldwide. The disparity is partly because of its rarity, which affects the number of clinical trials, laboratory studies, and large-scale data analyses conducted each year. Limited funding slows the pace of progress in understanding the disease’s biology, improving diagnostic methods, and developing new treatment strategies.
Public awareness campaigns during Sarcoma Awareness Month and throughout the year can help draw attention to the disease and boost funding from public and private sources. Increased financing would expand access to clinical trials, strengthen the evidence base for treatment decisions, and hasten the development of targeted therapies and immunotherapies for common and rare sarcoma subtypes.
Make a donation to Sarcoma Awareness.
14. July is Sarcoma Awareness Month
Sarcoma Awareness Month brings attention to one of the deadliest rare cancers. Sarcomas, as mentioned earlier, account for less than one percent of all adult cancers, yet they encompass over 100 subtypes. July is a dedicated period for sarcoma education and advocacy.
Public health organizations, cancer treatment centers, and patient advocacy groups orchestrate initiatives during this month to inform communities about sarcoma. They address its low public recognition, the difficulty of early detection, and the need for more clinical research. Awareness campaigns explain that symptoms vary widely depending on the tumor’s location, which is a leading cause of delayed sarcoma diagnoses. They also stress that sarcoma affects individuals across all ages, from children as young as four months, teenagers, young adults, to older adults.
New Hope Unlimited participates in Sarcoma Awareness Month by publishing resources, such as this post, about sarcoma, including therapies available at our treatment facility. We use this period to encourage individuals at risk, current patients, and their families to seek evaluations from specialists familiar with sarcoma’s complexity. As with any cancer, early intervention gives patients the best chance of overcoming their disease.
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15. You can survive and thrive
Advances in diagnostics and alternative therapies have improved outcomes for many people living with sarcoma. Survival rates vary by subtype, tumor stage, and treatment response, yet long-term remission is within reach. “The important thing is diagnosis. The earlier we detect sarcoma, the better,” assures Dr. Vivek Subbiah.
