What is Renal Cell Carcinoma?

Renal cell carcinoma (RCC) is a rare type of kidney cancer. Treatment often leads to a cure when the cancer is found in the early stages. If RCC spreads or happens again, treatment is less successful.

Renal cell carcinoma is the second most common type of kidney cancer in children. It accounts for about four to five percent of kidney cancer in children. Every year in the United States, fewer than 100 children and young people are found to have RCC.

Children as young as 18 months can have RCC. There is greater risk of RCC as children get older.

What Are the Different Types of Renal Cell Carcinoma in Children?

There are many different types of pediatric RCC. About half of children with renal cell carcinoma have translocation renal cell carcinoma. This can be called TFE-RCC. Other types of RCC are:

• Papillary, chromophobe, sarcomatoid or clear cell carcinoma
• Renal medullary carcinoma, a very aggressive cancer that affects young people who have sickle cell trait
• Even less common types of renal cell carcinoma

Knowing what type of renal cell carcinoma a person has helps doctors decide the how to treat the disease. That’s why it is so important for people with RCC to receive care from doctors who have experience in diagnosing and treating it.

Renal Cell Carcinoma Causes

For most children who get RCC, there is no known cause.

In a small number of children with translocation renal cell carcinoma, there may be a link to some risk factors.

• Some of these patients were treated before with chemotherapy for another type of cancer, such as neuroblastoma, leukemia, sarcoma or Wilms tumor.
• Others were born with poorly developed kidneys and received some form of immunosuppression therapy.

Although this doesn't happen often, a cancer predisposition syndrome can be identified (examples: von-Hippel-Lindau syndrome, sickle cell trait).

Renal Cell Carcinoma Symptoms

The symptoms of RCC are the same as those for other kidney cancers.

They are:

• Blood in the urine
• Belly fullness and pain
• A mass or lump in the belly or back

These symptoms are sometimes present, especially when the renal cell carcinoma is more advanced.

Renal Cell Carcinoma Diagnosis

The first step in diagnosing RCC is a physical exam and blood work. Imaging tests are next. These tests can help doctors figure out the size and position of the tumor. They can see if the cancer has spread. These tests help with surgical planning.

Imaging tests might include:

• Magnetic resonance imaging (MRI)
• Computed tomography (CT)
• Bone scan using positron emission tomography (PET)

Testing can include a genetic analysis to help the cancer team figure out what type of renal cell carcinoma a person has. The team may do a biopsy (look at a small piece) of the tumor and analyze it under the microscope. This can help them find therapies specific to your child. This is most helpful for people with advanced forms of renal cell carcinoma.

Renal Cell Carcinoma Staging

When a person is diagnosed with RCC, the care team will “stage” the disease. This is based on the tumor size and if the cancer has spread.

Most of the time, renal cell carcinoma is discovered in the early stages (stages 1-3). This means that the cancer is only in one kidney or has spread to nearby lymph nodes.

Stage 4 renal cell carcinoma is rare. It occurs when the cancer is discovered after it has already spread to other parts of the body. This can include areas like distant lymph nodes, the blood, the chest, spine, soft tissues or brain.

Renal Cell Carcinoma Treatment Options: Stages 1-3

When the tumor is found in stage 1-3, the standard treatment is surgery to remove part or all of the kidney. The surgeon will likely recommend removing some lymph nodes as well. Luckily, most people can live healthy lives with one kidney or even most of one kidney.

Surgery provides a cure for about 80% of children with renal cell carcinoma. These patients do not need additional therapy, such as chemotherapy or radiation therapy.

Renal Cell Carcinoma Treatment Options: Stage 4 and Relapse

About 20% of children with RCC need a different treatment than surgery alone. This may be because their cancer is cannot be operated on, has spread to other parts of the body or has returned after initial treatment.

There are no “standard” treatments for these patients. Treatment is based on what works for adults with renal cell carcinoma. Researchers at Cincinnati Children’s and other pediatric hospitals are creating and testing new therapies for pediatric forms of renal cell carcinoma.

Treatment options include:

• Immunotherapy. These drugs boost the body’s immune system. They help ward off the cancer cells.
• Biologics (anti-angiogenic drugs). These drugs block blood flow to the tumor. They target proteins that promote tumor growth.
• Radiation therapy. This can help with pain control. It treats tumors that are hard to remove with surgery.
• Chemotherapy. This is rarely used to treat patients with renal cell carcinoma. It can be part of the treatment plan.

What Is the Long-Term Outlook for People with Renal Cell Carcinoma?

When diagnosed and treated in the early stages, children and young people with RCC typically recover and are often considered cured (five-year survival is greater than 95%).

The disease is more difficult to treat when:

• The cancer has returned after the first therapy.
• The cancer has spread to other parts of the body.
• The patient has renal medullary carcinoma, a form of renal cell carcinoma associated with sickle cell trait. Renal medullary carcinoma usually appears in an advanced form at diagnosis.

Researchers are learning more and more about renal cell carcinoma. They are also exploring new ways to treat this disease. An international registry for TFE renal cell carcinoma exists.

Learn about kidney tumor clinical trials at Cincinnati Children’s