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What Are Mediastinal Tumors?
Mediastinal tumors are tumors that occur in the mediastinum. This is an area in the center of the chest cavity that contains important structures like:
- The heart.
- The esophagus.
- Large blood vessels.
- The trachea (windpipe).
- The thymus gland.
- Connective tissues between the lungs.
Mediastinal tumors in infants and children are typically secondary tumors. That means they're the result of a variety of other cancers that have spread. The chest cavity is a common migration point for many secondary tumors. Liver, kidney, and other cancers may spread to this area.
Primary mediastinal tumors are tumors that begin in the mediastinum. They include neuroblastomas, lymphomas, germ cell tumors, and thymomas.
What are the types of mediastinal tumors?
Cancers in the mediastinum are identified by the region affected:
Anterior region tumors
Anterior region tumors affect the area from the sternum to the front of the heart.
Tumors of the anterior mediastinum can include:
- Mediastinal germ cell tumors.
- Mediastinal lymphangioma (benign growths on lymphatic vessels).
- Thymic cysts (benign tumors on the thymic gland).
- Tumors (thymoma).
Lymphangiomas and thymic cysts are removed in surgery and have excellent recovery rates.
Middle mediastinal region tumors
These tumors are in the heart, aorta, trachea, bronchi, and lymph nodes.
The most common types of pediatric cancers in this region are Hodgkins lymphoma and non-Hodgkins lymphoma. Both are typically treated with radiation therapy and chemotherapy.
There are dozens of types of specific non-Hodgkins and Hodgkins lymphomas. The precise type and extent determine the treatment plan.
Posterior region tumors
These tumors involve the space behind the heart extending to the spinal column.
The most common posterior mediastinal tumor is neuroblastoma or ganglioneuroblastoma. Other posterior tumors are not typically found in pediatric patients.
What are the types of chest wall tumors?
- Bony tumors — These most often include Ewing sarcoma or osteosarcoma. Chondrosarcoma can also occur in the cartilage of the chest wall.
- Soft tissue tumors — These most often include neuroblastoma, soft tissue sarcoma, or Ewing sarcoma coming from the lining of the chest wall.
How common are mediastinal tumors?
Mediastinal tumors in children are very rare. Large studies show that they make up only a tiny fraction of childhood cancers — about 0.02%.
What causes mediastinal tumors?
Mediastinal tumors in children can develop for different reasons, depending on where they form in the chest.
- Tumors in the front part of the mediastinum are most often caused by cancers of the immune system, such as lymphoma or leukemia. Sometimes, germ cell tumors can cause them.
- Tumors in the middle area are usually linked to enlarged lymph nodes from leukemia or lymphoma. They are rarely found on their own.
- Tumors in the back part of the mediastinum are most often caused by nerve‑related cancers. Neuroblastoma is the most common type.
Mediastinal tumor risk factors
Certain factors make mediastinal tumors more likely in children, including:
- Being a boy — Mediastinal tumors are more common in males.
- Being a teenager — The average age of diagnosis is about 13.
Some tumor types, such as neuroblastoma:
- Are more common in girls.
- Mostly occur in children under the age of 5.
Complications of mediastinal tumors
- Children who develop symptoms such as neck masses, trouble breathing, or a condition called superior vena cava (SVC) syndrome are more likely to have lymphoma.
- Tumors that cause airway blockage or fluid buildup around the lungs or heart can be especially dangerous. They increase the risk of serious complications.
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What Are the Signs and Symptoms of Mediastinal Tumors?
Mediastinal tumor symptoms in infants and children are more prominent than those of adults with the same tumors.
The most common symptoms include:
- Bone pain.
- Breathing problems (fast, noisy, or difficult breathing).
- Coughing up blood.
- Fluid in the lungs.
- Headaches.
- High blood pressure.
- Hoarse voice.
- Low calcium levels.
- Muscle weakness.
- Swelling in face or neck.
- Trouble swallowing (if the tumor is pressed against esophagus).
When should I see a doctor about my child’s mediastinal tumor symptoms?
If your child has any of the following symptoms, contact their provider right away:
- Chest pain.
- Coughing or breathing difficulty.
- Fever and/or night sweats.
- Neck mass.
- Stridor (wheezing).
Early detection is critical for the best treatment outcomes.
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How Do You Diagnose Mediastinal or Chest Wall Tumors?
Your child's provider will ask you questions about their physical symptoms and health history. You can also expect them to perform a physical exam to look for signs of tumors. From there, you can expect them to order a biopsy and imaging tests.
Tests to diagnose mediastinal and chest wall tumors
Common testing includes:
- Biopsy — Removes a small part of the tumor’s tissue to be tested for cancer in a laboratory. Tumors in the mediastinum are near many vital organs. Because of that, biopsies typically require a procedure under general anesthesia performed by a surgeon or interventional radiologist.
- CT scan with contrast — An imaging test using contrast dye that is swallowed or injected to show the texture, size, and location of the tumor(s).
Some other tests that may help doctors diagnose mediastinal tumors include:
- Chest x-ray — This image helps doctors locate the tumor and is often the first x-ray performed.
- Echocardiogram — An ultrasound of the heart can help see if the tumor is impacting the heart space and if there is fluid around the heart that needs to be removed.
- Esophagogram — A special type of x-ray that uses contrast dye to see the imaging of the esophagus.
- MRI — These scans give similar information as a CT scan. They can help see the tumor a little better in some situations.
- Nuclear medicine tests — These scans use small amounts of radioactive material to help determine where the tumor is and what it's made of.
- PET scans — These scans can help diagnose cancerous tumors and track how cancer treatment is working.
- Tumor markers — These substances in the blood can help with diagnosis.
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How Do You Treat Mediastinal and Chest Wall Tumors?
The goal of treatment is to remove all the cancer or as much as possible. Many factors affect recovery rates and treatment options:
- Size of tumor — Tumors that can be completely resected (removed) by a surgical treatment have a higher recovery rate. Chemotherapy may also be used to shrink the tumor for removal or to reach cancerous cells in other parts of the body.
- Spread of cancer — Especially with lymphoma, how far away from the lymph nodes the cancer has spread determines the treatment and recovery.
- Stage of tumor — As with many cancers, lower-stage tumors are more easily cured. The earlier the tumor is found, the better the chance of removal and treatment.
The most common treatment for mediastinal tumors usually begins with surgical removal and includes both chemotherapy and radiation therapy.
Surgery for mediastinal and chest wall tumors
Surgical removal of cancerous tumors is important to decrease the risk of the cancer spreading or recurring.
Your child's surgical team will create a plan for removing the tumor. They may either recommend traditional open surgery or minimally invasive surgery, depending on the location, size, and stage of cancer.
Once the tumor is removed, your child may need chemotherapy and/or radiotherapy, depending on their diagnosis.
Mediastinal tumors
If the tumor is small and easy to access, surgeons may remove the entire tumor as the first step in treatment.
If the tumor is large, the surgeon may first take a small piece of the tumor for a biopsy. This can help your child's team learn more about the tumor and help decide on the best treatment.
Procedures can be either minimally invasive (thoracoscopy) or open (thoracotomy or sternotomy). If your child needs a sternotomy, the procedure often will involve a joint effort between cardiac surgeons and pediatric surgical oncologists.
Chest wall tumors
These tumors are usually large.
Some small malignant and benign tumors can be removed as the first step of treatment. This can occur as a combination of minimally invasive and open surgery or just as open surgery alone.
For large tumors, a biopsy usually takes place first. After chemotherapy, surgeons will perform a chest wall resection. This may require removal of multiple ribs and chest wall reconstruction, which may involve plastic surgery as well.
Chemotherapy for mediastinal and chest wall tumors
Some doctors may request chemotherapy before surgery to shrink the tumor size. This helps make surgery easier.
Chemotherapy involves cancer-killing drugs that can either shrink tumors before surgery or kill any cancer cells left in the body after therapy. Your child’s care team will come up with a plan for how to best use chemotherapy effectively.
Radiotherapy for mediastinal and chest wall tumors
Radiation therapy uses highly focused x-ray beams to accurately destroy tumors in the body without harming surrounding tissues.
Like the other methods, radiation can be used in addition to other treatment methods. It will be used only if your child’s care team thinks it is appropriate.
How effective is treatment?
When mediastinal tumors are found early and treated quickly, survival rates are high.
Using biopsy and chemotherapy for lymphoid tumors, or surgery followed by strong additional therapy for non‑lymphoid tumors, leads to survival in about 74% of children.
Children with benign (noncancerous) tumors almost always survive, with more than 96% doing well after surgery.
How long does it take to recover after surgery for mediastinal tumors?
Recovery time for children treated for mediastinal tumors depends on the type of surgery.
After minimally invasive surgery, most children recover in about one to two weeks. More extensive surgery can take six to eight weeks for full recovery.
Most hospital stays last around three to five days. But if complications occur, your child may need to stay a week or longer.
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Why Choose UPMC Children’s for Care?
At UPMC Children’s Hospital, every child diagnosed with a mediastinal tumor receives an individualized treatment plan and family-centered care. Here, you can expect:
- Advanced care — Cutting-edge research and the latest technology ensure our patients' best possible outcomes.
- Surgical expertise — Our surgeons have vast experience in the surgical removal of mediastinal and chest wall tumors.
- A team approach — We work together across many different medical specialties to ensure your child gets the best treatment for them.
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