Sarcoma in Children

What is Pediatric Sarcoma?

A sarcoma is a cancer that develops in bone or soft tissue. Soft tissue is the connective tissue between body parts and organs and can include muscles, tendons, fat, bone and cartilage, blood vessels, and lymph vessels. Sarcomas can be found anywhere in the body, but they are most often found in the arms, legs, chest, or abdomen. 

Pediatric sarcoma is sarcoma occurring in children, and accounts for approximately 15% of pediatric cancer cases, with 1,500 to 1,700 new cases diagnosed each year. 

 

Types of Sarcoma Cancer in Children

There are two main types of sarcoma: soft tissue sarcoma and bone sarcoma. Types of soft tissue sarcoma, often referred to as STS, develops in the soft connective tissue such as the muscles, cartilage, tendons, or fat. Bone sarcoma forms in new tissue in growing bones. Bone and soft-tissue sarcomas are relatively rare childhood cancer tumors. Within the two main types of sarcoma, there are more than 70 subtypes based on the origin and location of cancer. 

The most commonly diagnosed types include: 

Ewing Sarcoma

A rare type of childhood sarcoma that grows in bone or the soft tissue surrounding it. It is typically found in the center of the body, such as the chest, pelvis, or vertebrae, but can appear in any bone, including the hands, feet, arms, legs, and skull. It is most often diagnosed during puberty, but can also occur in younger children or adults.

Rhabdomyosarcoma

This is the most common type of soft tissue sarcoma in children and accounts for nearly 5 percent of childhood cancers. It develops in the skeletal muscles, which are formed from cells called rhabdomyoblasts. Though it can affect anyone, it primarily occurs in children under four years of age. 

Osteosarcoma

This type of sarcoma cancer typically originates in the “long bones” of the body, such as the bones of the arms and legs. It is the most common type of bone cancer in children and is usually diagnosed during puberty or times of growth spurts. 

 

Other common types of sarcoma in children

    • Fibrosarcoma – develops in fibrous tissue
    • Liposarcoma – develops in fatty tissue
    • Synovial sarcoma – develops in cells near the tendons and joints
    • Angiosarcoma – develops in the inner lining of the blood vessels
    • Chondrosarcoma – develops in the cartilage

 

Causes, Symptoms, and Risk Factors of Sarcoma in Children

Unfortunately, medical professionals do not know exactly what causes childhood sarcoma. However, some factors that could increase the risk of developing sarcoma include:
   • Inherited genetic disorders such as neurofibromatosis and Li-Fraumeni syndrome
  • AIDS and Epstein-Barr viral (EBV) infections
  • Prenatal genetic changes caused by certain chromosome abnormalities
  • Exposure to certain chemicals, such as vinyl chloride
  • Previous radiation therapy treatment

Sarcomas often exhibit few symptoms, but symptoms can increase or worsen as the tumor grows and presses on nearby organs or blood vessels. Common sarcoma symptoms in children include:
  • A painless lump or mass
  • Swelling under the skin
  • Pain, tingling, or numbness
  • Weakness
  • Trouble breathing 

 

Classification of Sarcoma

Sarcoma cancer can be classified into nonmetastatic sarcoma, metastatic sarcoma, and recurrent sarcoma:

Nonmetastatic pediatric sarcoma

The cancer has been partly or completely removed in surgery and has not spread to other parts of the body.

Metastatic pediatric sarcoma

The cancer has spread from where it started to other parts of the body.

Recurrent sarcoma

The cancer has come back (recurred) after it has been treated. It may recur in the original location or in another part of the body.

 

Diagnosis and Stages of Childhood Sarcoma

Because symptoms of sarcoma often appear in other childhood conditions, diagnosis of sarcoma in children requires a comprehensive medical examination, which includes a physical exam as well as various types of imaging tests, such as an X-ray, CT scan, MRI, or PET scan. If a mass is found, the diagnosis will be confirmed with the results of a biopsy. 

Once diagnosed, doctors will assign a stage to cancer. Staging is a way to classify the severity of cancer by identifying where it is located, if or where it has spread, and whether it is affecting other parts of the body. There are several staging systems for pediatric sarcoma that help to determine the most effective treatment options.

One tool that doctors use to universally describe stages of sarcoma is the TNM system. Using results from diagnostic tests, doctors assess the below criteria and assign a stage of severity from 1 to 4.
  • Tumor (T): Size and location of the mass
  • Node (N): Spread to lymph nodes – quantity and location of affected lymph nodes
  • Metastasis (M): Spread beyond lymph nodes to other parts of the body – quantity and location of spread

In addition to the TNM system, doctors use “Grade” (G) to compare cancerous tissue with healthy tissue and examine how much they differ. 

Using the T, N, M and G classifications, doctors assign a stage to the cancer. General stages for sarcoma cancer are:
  • Stage I: The tumor is small and low grade.
  • Stage II: The tumor is small and higher grade.
  • Stage III: The tumor is larger and higher grade.
  • Stage IV: The cancer has spread to other parts of the body. The original tumor can be any size, any grade, and may or may not have spread to the regional lymph nodes.

Once the sarcoma has been assigned a stage, doctors will coordinate a treatment plan.

 

Treatment and Prognosis of Sarcoma in Children

Treatment for pediatric sarcoma depends on the size, grade, location, and spread of the tumor. Treatment often includes a combination of:
  • Surgery to remove the tumor and nearby tissue
  • Chemotherapy
  • Radiation 

Some sarcomas can be completely removed by surgery. More aggressive chemotherapy or radiation is typically reserved for high-grade sarcomas that have larger tumors or have spread to other parts of the body. 

Fortunately, children with sarcoma cancer often respond better to treatment than adults and thus have a better prognosis. The prognosis of children with sarcoma is described in terms of the five-year survival rate based on how much a tumor has spread. 

Localized Spread: Limited to the part of the body where it originated; 81% five-year survival rate

Regional Spread: Spread to nearby structures or lymph nodes; 57% five-year survival rate

Distant Spread: Spread to distant parts of the body or other organs such as the lungs; 16% five-year survival rate

The overall five-year survival rate for pediatric sarcoma is 65%, but this could be lower or higher and is influenced by several factors including tumor size, grade, and location; the age and overall health of the child at the time of diagnosis; and the response to treatment. 

Despite significant improvements in outcomes for some pediatric cancers, like acute lymphoblastic leukemia, survival rates in metastatic sarcomas have not improved in 40 years — representing some of the greatest unmet needs in pediatric cancers. 

 

Pediatric Sarcoma Research

Despite modern medical advancements, children with sarcoma have very few promising treatment options — especially in the case of recurrent sarcoma. By investing in innovative, next-generation approaches to therapy and forging collaborative, international partnerships, Solving Kids’ Cancer aims to change the prognosis for children with pediatric sarcoma and rare childhood cancers. 

Currently, clinical trials are exploring a combination of approaches, including immunotherapy,1 to counteract the various ways sarcoma cells evade the host immune system. Our project, Can We Train a Child’s Immune Cells to Recognize and Kill Cancer Cells?, used vaccines created from a patient’s immune cells to target and kill cancer cells. We also helped fund a phase I/II study using armed T-cells with GD22 that had at least one signal (immune response) in osteosarcoma pediatric patients. 

Pushing the limits of innovation can change the world for children with sarcoma. When you give to Solving Kids’ Cancer, you help change lives by making better treatment options possible — because every kid deserves to grow up®.

A pink button that says `` help solve kids ' cancer ''.
Sources
1 Current State of Immunotherapy and Mechanisms of Immune Evasion in Ewing Sarcoma and Osteosarcoma – https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9818129/
2 Activated T Cells Armed With GD2 Bispecific Antibody in Children and Young Adults With Neuroblastoma and Osteosarcoma – https://clinicaltrials.gov/ct2/show/NCT02173093
A man and a little girl are sitting in front of a wooden wall.

Ahana’s Story of Strength

By duda • November 20, 2024
Ahana’s Story of Strength with High-Risk Neuroblastoma Every day, over 1,000 children worldwide are diagnosed with cancer,1 and for many families, that diagnosis means facing an uncertain future. At just 20 months old, Ahana was diagnosed with a rare and aggressive cancer that had already spread through her small body. Her family’s search for life-saving childhood cancer treatments led them from South Africa to Spain and possibly toward a clinical trial in New York. Ahana’s journey shows the importance of supporting organizations like Solving Kids’ Cancer, which works to find, fund, and advocate for advanced treatments that give hope to families fighting against tough-to-treat rare pediatric cancers.
A poster for 2024 year in review for solving kids ' cancer

2024 Year-in-Review

November 11, 2024
Advancing Childhood Cancer Research: SKC’s 2024 Milestones
A group of children are holding a sign that says `` lace up for kids 2024 ''.

2024 Lace Up for Kids Recap

October 1, 2024
Lace Up for Kids: Your Impact on Childhood Cancer Research At Solving Kids’ Cancer, our mission has always been driven by hope — hope for better treatments, hope for breakthroughs, and most importantly, hope for every child fighting cancer. This year’s Lace Up for Kids campaign was nothing short of inspiring. Together, we’ve raised over $264,000 across the entire campaign, funding innovative childhood cancer research and providing more treatment options for children in need.  From coast to coast, nearly 4,000 participants have stepped up to lace up their gold shoelaces and make a difference. Whether it was through schools, sports teams, or community groups, the show of support has been overwhelming throughout the years. Over 7,823 donations have been made to accelerate new treatments, and the hashtag #CareWearShare has been used 13,864 times, spreading the message far and wide!
A girl is standing in front of a car in a parking lot.

Stories of Impact: Erin Martin

September 2, 2024
Gold Laces for Childhood Cancer Awareness Month: Erin’s Story 
A group of children are standing in a circle with their shoes on the floor.

Smithtown Goes Gold for Childhood Cancer

August 8, 2024
Smithtown Goes Gold for Childhood Cancer Awareness Month!
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Lace Up for Kids 2024

August 6, 2024
Childhood Cancer Awareness Month: Lace Up for Kids 2024
A man standing on top of a mountain with the words osteosarcoma survivor to surgeon dr. kurt weiss ' story

Osteosarcoma Survivor Dr. Kurt Weiss

August 1, 2024
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Jacob 's journey : a childhood cancer survivor 's story

National Cancer Survivors Month

May 10, 2024
Jacob’s Journey: A Childhood Cancer Survivor’s Story Every battle has its heroes, and among the bravest are young children like Jacob Mozer, who faced stage 4 high-risk neuroblastoma when he was barely old enough to walk. Diagnosed at only 16 months old, Jacob’s early years were filled with constant medical appointments and intense treatments. Despite these challenges, today, Jacob is a shining example of the remarkable progress in pediatric oncology. He’s not merely surviving; he’s thriving as a pharmacy resident, driven to give back to the medical community that gave him a second chance at life. During National Cancer Survivors Month this June, we honor and celebrate fighters like Jacob who have battled cancer and emerged stronger. This month also serves to amplify the conversation about pediatric cancer survivorship and push for vital research that continues to save lives. Solving Kids’ Cancer (SKC) plays an essential role in this effort, supporting children like Jacob by funding crucial clinical trials that enhance survival rates and improve the quality of life for those fighting fatal childhood cancers.
A close up of a person holding a test tube in a laboratory.

Trailblazing Treatments for Pediatric Brain Tumors

May 2, 2024
Trailblazing Treatments for Pediatric Brain Tumors Picture a fortress built to guard precious treasure but accidentally also keeps out the very allies it needs in a time of crisis. This is the challenge faced by medical researchers when dealing with the brain’s natural defense — the blood-brain barrier — especially in children with brain tumors. At Columbia University Medical Center, Dr. Luca Szalontay is leading the charge to breach this fortress with groundbreaking approaches. Recently, Dr. Timothy Cripe from Solving Kids’ Cancer’s podcast, This Week in Pediatric Oncology (TWIPO) , interviewed Dr. Szalontay to delve deeper into these innovative treatments. This Brain Tumor Awareness Month, we’re shining a light on these important advances in treating children’s cancer. Understanding the Challenge Treating pediatric brain tumors like diffuse midline glioma (DMG) is especially tough because of the brain’s own protective shield, known as the blood-brain barrier. This barrier keeps out most chemotherapy drugs as well as large molecules, like antibodies, making it hard to treat the tumors effectively. Dr. Szalontay describes the dilemma: “Our field hasn’t been able to advance as quickly as it has with leukemias or other solid tumors because the drugs don’t penetrate the brain efficiently.” She adds on the nature of this blood-brain barrier, “It’s evolutionary, very important to protect us from different toxins and harmful agents, but this is a shield which protects the tumor as well.” Even when drugs work well in lab tests, they struggle to get to the tumor in strong enough doses without harming the body. Dr. Szalontay also points out that DMG tumors are found in very sensitive areas of the brain, making traditional surgery or radiation risky because they could damage vital brain functions. This makes finding safe and effective treatments for these tumors even more challenging. So, what’s being done to tackle these obstacles and help kids fight these tumors?
A group of stick figures are holding hands around a globe.

World Health Day: Pediatric Cancer’s Global Divide

April 4, 2024
World Health Day: Pediatric Cancer’s Global Divide Imagine this: Two children, born at the same moment, oceans apart. In a country brimming with medical resources, one child quickly gets top-notch treatment for cancer. Meanwhile, across the globe, the other child faces a drastically different reality, with limited access to basic care. This isn’t just a thought experiment — it’s the harsh truth of pediatric cancer treatment worldwide. Observed every year on April 7, World Health Day shines a light on the mission to make healthcare accessible for all. This day is particularly significant in the context of pediatric cancer, where vast disparities in treatment availability highlight the global challenge of ensuring every child has the opportunity for health and healing. The Stark Contrast in Survival Rates: A Look at Pediatric Cancer Statistics Every day, more than 1,000 children are diagnosed with cancer, and the chances of survival vary greatly depending on where they live.1 Pediatric cancer survival rates serve as a clear indicator of the disparities between high-income countries (HICs) and low- and middle-income countries (LMICs). In wealthier nations such as the United States and Western Europe, the 5-year survival rate for children with cancer is over 80%, thanks to advanced healthcare systems and access to the latest treatments.2 But, here’s a troubling childhood cancer statistic: in LMICs where 80% of children with cancer live, fewer than 20% survive.1 This gap is due to several factors, including late diagnoses or, tragically, no diagnosis at all. Many children succumb to the disease without access to pain medication, and the burdens of travel and the high costs force most families to abandon care. These challenges are compounded by limited access to necessary treatments,3 as well as a lack of specialized equipment and trained healthcare professionals needed for effective pediatric cancer treatment. Addressing this divide requires a concerted global effort to improve early detection, make treatments more affordable, and enhance healthcare infrastructures, giving every child a fair chance to beat cancer. Navigating the Access Maze Access to cancer care shouldn’t be a treasure hunt. Yet, for many in LMICs, it feels like that — a relentless search for something seemingly out of reach. Here’s the reality revealed in childhood cancer statistics worldwide: many areas lack enough doctors who specialize in children’s cancer4 and the prices of cancer drugs can be sky-high.2 Even getting to a hospital can be an ordeal, with some families traveling hundreds of miles. And the financial strain? Devastating. The cost of care can quickly add up to more than a year’s income, forcing households into deep financial distress. In many LMICs, the lack of comprehensive insurance coverage means families are often left to pay for pediatric cancer treatment entirely out-of-pocket.5 Families often face the impossible choice between their child’s health and their financial survival. Solving Kids’ Cancer: Bridging the Gap Solving Kids’ Cancer (SKC) isn’t standing by; we’re on the front lines, actively working to make a difference in the global pediatric cancer landscape. Beyond funding research, advocating for policy changes, and fostering global partnerships, SKC is committed to educating and empowering the community to take action. We understand that while we might not have a global foothold to address every aspect of the pediatric cancer divide directly, there’s power in collective action. Through our efforts, we aim to catalyze advancements in pediatric cancer treatments and care that could dramatically change outcomes for children worldwide. • Research: We direct funds into cutting-edge studies, searching for breakthrough treatments that promise a brighter future for all kids, regardless of where they live. • Advocacy: We advocate for children’s needs by steering research funding towards unmet challenges and collaborating with experts to prioritize all children, including expanding trials to international sites to maximize access.2 • Global Partnerships: We don’t do it alone. By teaming up with researchers, hospitals, and other organizations across the globe, we’ve helped fund clinical trials that have extended to over 250 institutions across 15 countries worldwide . Uniting for a Cause Solving Kids’ Cancer extends its mission beyond research by fostering community and collaboration. Our signature event, Lace Up for Kids , held annually during September Childhood Cancer Awareness Month, is a powerful testament to this mission, drawing communities together to stand in solidarity against childhood cancer. Additionally, supporters can create their own fundraiser and have a platform to engage their local communities, sparking crucial conversations and garnering support for the cause. These initiatives highlight an important fact: While the challenge of pediatric cancer is significant, each of us has a part to play. By working together and supporting each other, we can fund the research needed to possibly change the direction of pediatric cancer treatment and care worldwide. World Health Day: A Chance for Every Child The fight against pediatric cancer is a stark reminder of the work ahead of us.6 It’s a battle that Solving Kids’ Cancer is dedicated to, but we need more than just our efforts — we need a global movement. This World Health Day, let’s rally for every child’s right to fight cancer on an even playing field. Donate today to help fund more innovative research and one day find a cure for childhood cancer — because every child, no matter where they are born, deserves a chance — a chance to fight, a chance to survive, and a chance to thrive.
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