Solitary fibrous tumor

Overview

Solitary fibrous tumors are growths of cells that can form in almost any part of the body. The growths, called tumors, start from cells in the tissues that support other tissues in the body, known as connective tissues. Solitary fibrous tumors are rare. They mainly affect older adults.

Solitary fibrous tumors most often occur in the lining around the outside of the lungs, called the pleura. Solitary fibrous tumors that happen in the pleura are called pleural solitary fibrous tumors. Solitary fibrous tumors also have been found in the head and neck, abdomen, breast, kidney, prostate, spinal cord, and other parts of the body.

Solitary fibrous tumors tend to grow slowly. They might not cause symptoms until they grow large. Symptoms depend on where the tumor is in the body. If it's in the lungs, symptoms might include cough and shortness of breath.

Treatment for solitary fibrous tumor often involves surgery to remove the tumor. Sometimes the tumor comes back after surgery or spreads to other parts of the body. Healthcare professionals assign solitary fibrous tumors a risk category that says how likely they are to come back or spread. Solitary fibrous tumors can be low, intermediate, or high risk. The risk is based on different factors such as your age, the size of the tumor and what the tumor looks like under a microscope.

Symptoms

Solitary fibrous tumor signs and symptoms can depend upon where the tumor grows. Solitary fibrous tumors often grow so slowly that they don't cause symptoms for some time. They most often happen around the lungs, abdomen, and head and neck.

Symptoms of a solitary fibrous tumor in or around the lungs include:

Chest pain.
Cough.
Coughing up blood.
Swelling of fingers or toes, called clubbing.
Shortness of breath.
Swelling in the lower legs.

Symptoms of a solitary fibrous tumor in the abdomen include:

Abdominal pain.
Constipation.
Involuntary urine leaking.
Painful urination.
Urinating frequently at night.
Vomiting.

Symptoms of a solitary fibrous tumor in the head and neck include:

Bulging eyes.
Frequent tearing.

Solitary fibrous tumors that happen in other places in the body may cause painless lumps or masses.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have ongoing symptoms that worry you.

Causes

The cause of solitary fibrous tumors isn't known. A solitary fibrous tumor starts when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In tumor cells, the DNA changes give different instructions. The changes tell the tumor cells to grow and multiply quickly. This causes too many cells.

The cells might form a mass called a tumor. The solitary fibrous tumor can grow to press on blood vessels and healthy body tissue. Rarely, the cells can break away and spread to other parts of the body, which is called metastasis.

Risk factors

The main risk factor for solitary fibrous tumors is older age. Solitary fibrous tumors are most common between ages 50 and 70.

There is no way to prevent solitary fibrous tumors.

Diagnosis

Tests and procedures used to diagnose solitary fibrous tumors include imaging tests and a procedure to remove some tissue for testing, called a biopsy.

Imaging tests

Imaging tests make pictures of the body. They can show where a solitary fibrous tumor is, how large it is and if it has spread to other areas of the body. Imaging tests for solitary fibrous tumors might include MRI, X-ray, CT, ultrasound and positron emission tomography (PET) scan.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the tumor. Sometimes surgery is needed to get the tissue sample.

The sample is tested in a lab to see if it is cancer. Testing is done by doctors who specialize in analyzing blood and body tissue, called pathologists. Other special tests give more details about the tumor. Your healthcare team uses this information to make a treatment plan.

Treatment

Treatment for solitary fibrous tumors often involves surgery. Other treatments may include radiation therapy, chemotherapy and targeted therapy.

Surgery

Most often, surgery is the only treatment needed for a solitary fibrous tumor. Surgeons remove the tumor and a small amount of healthy tissue around it. The type of operation used to remove a solitary fibrous tumor depends on where the tumor is in the body.

Other treatments might be used after surgery to lower the risk that the tumor will come back. These other treatments might include radiation therapy or chemotherapy.

Radiation therapy

Radiation therapy uses powerful energy beams to kill tumor cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

Radiation therapy might be used before or after surgery to lower the risk that the solitary fibrous tumor will come back. Sometimes radiation therapy is used instead of surgery. This might happen if the tumor is in a location that is not safe to do surgery.

Chemotherapy

Chemotherapy uses strong medicines to kill tumor cells. For a solitary fibrous tumor, chemotherapy might be used if the tumor has spread or can't be removed with surgery.

Targeted therapy

Targeted therapy uses medicines that attack specific chemicals in the tumor cells. By blocking these chemicals, targeted treatments can kill tumor cells. Targeted therapy might be used if a solitary fibrous tumor has spread to other parts of the body.

Preparing for an appointment

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your health professional thinks you might have a solitary fibrous tumor, you will likely be referred to a specialist. Specialists who care for people with solitary fibrous tumors include:

Cancer doctors, called medical oncologists.
Surgeons who operate on the lungs and chest, called thoracic surgeons.
Surgeons who operate on the brain and nerves, called neurosurgeons.
Surgeons who operate on the abdomen, called general surgeons.

Appointments can be short and being prepared can help. Here's some information that may help you get ready.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test.

Make a list of:

Your symptoms, including any that seem unrelated to the reason for your appointment.
Key personal information, including major stresses, recent life changes and family medical history.
All medications, vitamins or other supplements you take, including the doses.
Questions to ask your doctor.

Take a family member or friend along, if possible, to help you remember the information you're given.

For solitary fibrous tumors, some basic questions to ask your doctor include:

Do I need more tests?
What are my treatment options?
What are the potential risks for these treatment options?
Do any of the treatments keep the tumor from growing back?
Can I have a copy of my pathology report?
How much time can I take to consider my treatment options?
Are there brochures or other printed materials that I can take with me? What websites do you recommend?
What would happen if I choose not to have treatment?

Don't hesitate to ask other questions.