Nephroma

What is nephroma?

Nephroma is a tumor of the kidney cortex. The renal cortex is the outer portion of the kidney that filters the blood and removes waste inside the body. There are several kinds of nephroma, which fit into two main types: non-cancerous (benign) and cancerous (malignant).

Non-cancerous forms of nephroma include: 

• Benign multiocular cyst
• Mesoblastic tumor
• Cystic nephroma

Cancerous forms of nephroma include:

• Multilocular cystic renal cell cancer (MCRCC)
• Wilms tumor (nephroblastoma)

Cancerous forms of nephroma are more dangerous because they tend to spread (metastasize) to surrounding tissues in the body.

The kidneys are prone to several types of cysts and other tumor disorders. So, doctors who specialize in treating kidney and other renal diseases (nephrologists) are especially careful when diagnosing a nephroma. 

How common is nephroma?

Research is not clear on how common nephroma is overall since it’s a catch-all category for various kidney tumors. However, there is information for certain types of nephromas:

• While rare, Wilms tumors are the most common kidney cancer in children. Five hundred new cases are diagnosed annually in the United States, appearing most often before age 5.
• Renal cell carcinoma is the most common kidney cancer among adults. But, its subset MCRCC is very rare, accounting for only 1% to 3% of all kidney tumors.

What are the signs and symptoms of nephroma?

A person with a non-cancerous or cancerous nephroma may have these symptoms:

• Pain in the abdomen or mid-to-lower back
• A mass in the mid-to-lower abdomen or stomach
• Blood in the urine
• Infection
• Anemia
• Fever
• General feeling of being tired and unwell (malaise)

How is nephroma treated?

Both non-cancerous and cancerous nephroma threatens kidney health and function. So, surgery is often the treatment of choice because it leads to the best outcomes. This may include removing the tumor or cysts or removing part or all of the kidney (nephrectomy). Most people can live healthy, normal lives with just one full kidney.  

Other treatments for cancerous forms of nephroma include the following:

• Chemotherapy (“chemo”)— the patient receives one or more anti-cancer drugs (by mouth or by vein) over a period of time, usually as an outpatient. Chemo drugs target fast-growing cells of any kind, so they are useful for types of nephromas that have spread beyond the kidneys. Chemo may be used before surgery to shrink a tumor, after surgery to kill any remaining cancer cells, or as its own treatment with no surgery. Side effects can be severe, including fatigue, nausea, mouth sores, intestinal problems and hair loss. Doctors often space treatments apart to give normal cells a chance to recover.
• Radiation—this treatment uses high-energy radiation to kill cancer cells and shrink tumors by damaging the DNA of the cancer cells. Since doctors often see good results with surgery and chemo, radiation is usually only used to treat certain types of nephromas, such as aggressive or late-stage Wilms tumors. Radiation treatments are usually short (30 minutes or less) and painless, but side effects such as fatigue, skin damage and nausea can occur.

What are the survival rates for nephroma?

Most forms of nephroma are quite treatable. Most have good outcomes after the affected tissue is removed by surgery. Survival rates for nephroma and kidney cancer in general include the following:

• The overall 5-year survival rate for all kidney cancers is about 73%.
• For Wilms tumors, the 5-year survival rate is even better at 90%.
• For MCRCC, a 2004 World Health Organization study found a 5-year survival rate of 100% among 45 patients observed with this condition.
• As with most tumors, the lower the tumor stage (severity) at treatment, the higher the chance of survival.  

Why choose St. Jude for your child’s nephroma treatment?

• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
• Families never receive a bill from St. Jude for treatment, travel, housing and food – because all a family should worry about is helping their child live.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• The nurse-to-patient ratio at St. Jude is unmatched—averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• St. Jude has extensive experience treating children with bilateral Wilms tumor (cancer on both kidneys). Nearly 20% of children in the United States with bilateral Wilms tumor have their surgery performed at St. Jude.
• St. Jude surgeons pioneered an approach to remove the malignant tumor, sparing healthy kidney tissue. This is called “nephron-sparing surgery.” Not only does this approach have excellent survival rates, but it also helps children avoid kidney failure, dialysis and transplant.
• The current treatment approach for patients with Wilms tumor tries to limit therapy and its side effects for patients with a low risk of the tumor coming back. Therapy is increased for patients who are at high risk for the tumor returning and for children with anaplastic histology.
• In the lab, scientists are trying to find tumors that are more likely to resist therapy. The researchers are finding genes that are connected with poor outcomes and tumors returning.
• Cancer survivors, especially those cured at a young age, face medical and psychosocial challenges as they age. The St. Jude After Completion of Therapy (ACT) Clinic provides resources and monitors patients for 10 years after diagnosis or until 18 years of age, whichever comes later.