The kidneys are two small organs that work to remove waste products from the blood. Papillary renal cell carcinoma forms in the cells that line the small tubes in the kidney, known as renal tubules. These tubes filter waste from the blood and make urine.
Renal cell carcinoma is the most common type of kidney cancer, and papillary renal cell carcinoma makes up about 15% of those cases.
A papillary tumor usually looks like a long, thin growth. There are two types of papillary renal cell carcinoma.
This article will provide an overview of the causes and symptoms of papillary renal cell carcinoma. It will also discuss diagnostic tests and treatment options.
Types of Papillary Renal Cell Carcinoma
There are two types of papillary renal cell carcinoma, and their prognoses are quite different.
Type 1: This type of cancer tends to grow slowly and is less likely to spread than type 2. People who have been diagnosed with hereditary papillary renal cancer are more at risk of developing type 1. Type 2: This type of cancer tends to spread more quickly than type 1. People with hereditary leiomyomatosis and renal cell cancer are at higher risk of developing type 2.
Papillary Renal Cell Carcinoma Symptoms
Not everyone with papillary renal cell carcinoma experiences any symptoms. However, it is possible that you may notice the following signs and symptoms:
Blood in the urine Low back pain (flank pain) Abdominal pain or swelling Unintended weight loss Fatigue Fever A lump in the side of the abdomen Swelling in the veins around the testicle (varicocele) Excessive hair growth in women Pale skin Vision changes Liver changes Elevated erythrocyte sedimentation rate (blood test)
Causes
Papillary renal cell carcinoma usually occurs randomly and does not have a known cause. This is called sporadic papillary renal cell carcinoma.
While rare, it is possible for papillary renal cell carcinoma to run in families. Hereditary papillary renal cell carcinoma is a genetic disorder caused by a mutation in the MET gene. This mutation raises your risk of developing type 1 papillary renal cell carcinoma.
Diagnosis
The diagnostic process for identifying papillary renal cell carcinoma will include a physical exam, thorough medical history, laboratory tests, and imaging studies.
Smoking Obesity Hereditary leiomyomatosis Renal cell cancer Receiving dialysis treatment High blood pressure (hypertension) Family history of kidney cancer Horseshoe kidney (a condition where the two kidneys bind together at the bottom to create a horseshoe shape) Polycystic kidney disease Long-term use of pain or diuretic medications Von Hippel-Lindau disease Birt-Hogg-Dubé syndrome
If your doctor suspects kidney cancer, they will likely start the diagnostic process by ordering an imaging test such as:
Computed tomography (CT) scan Magnetic resonance imaging (MRI) Ultrasound
Lab tests used may include:
Blood chemistry Complete blood count (CBC) Intravenous pyelogram (IVP) Liver function tests Urinalysis Renal arteriography
Once a tumor has been identified, your doctor will obtain a biopsy of it by inserting a needle into the tumor and taking cells to study under a microscope. This sample will show which type of kidney cancer you have.
Treatment
Treatment for papillary renal cell carcinoma usually starts with surgery to remove part or all of the kidney. This procedure is known as a nephrectomy. Surgery is the most common treatment option for early-stage kidney cancers.
Bone scan Chest X-ray Chest CT scan Positron emission tomography (PET) scan
Radiation therapy may then be recommended to kill cancer cells in the area of the tumor. Your doctor may recommend radiation after surgery to ensure that all cancer cells have been killed.
Adjuvant therapy is an additional treatment used after surgery to help kill remaining cancer cells in the tumor area. Your doctor may recommend adjuvant therapy to help prevent cancer from returning.
Chemotherapy is currently not indicated in the treatment of papillary renal cell carcinoma.
Prognosis
The prognosis for papillary renal cell carcinoma is unique to every individual and depends on how advanced the cancer is and whether or not it has spread to other areas of the body.
Nexavar (sorafenib): Treats advanced renal cell carcinomaProleukin (aldesleukin): Approved for adults over age 18 with metastatic renal cell carcinomaTorisel (temsirolimus): Treats advanced renal cell carcinomaKeytruda (pembrolizumab): Approved as an adjuvant treatment to prevent kidney cancer from coming back in some patients
The prognosis for type 1 papillary renal cell carcinoma is believed to be better than for type 2. This is because type 2 carcinoma is a more aggressive form of cancer that spreads quickly.
Possible complications of papillary renal cell carcinoma include:
High blood pressure (hypertension)High red blood cell countHigh calcium levels in the bloodLiver and spleen problemsMetastasis
Coping
Once you learn that you have cancer, finding a support network will be an important aspect of your treatment. Talk with your medical team about options such as a support group or online community.
The following organizations may be able to help:
Action to Cure Kidney Cancer (ACKC): Gives education and support to those being treated for kidney cancer Patient Access Network (PAN) Foundation: Provides additional assistance to low-income patients who have health insurance The Social Security Administration: Includes papillary renal cell carcinoma in their Compassionate Allowances Initiative to speed up the processing of disability claims The Assistance Fund: Offers education and financial aid to individuals with chronic diseases
Summary
Papillary renal cell carcinoma is a type of kidney cancer that starts in the small tubes in the kidneys known as renal tubules. The tumors appear as finger-like projections in the tubules. Common symptoms include blood in the urine, pain, and feeling a mass in your side. Papillary renal cell carcinoma is usually treated with surgery and possibly radiation therapy as well.
A Word From Verywell
Learning that you may have papillary renal cell carcinoma is an overwhelming and frightening experience. It may be helpful to remember that help is available.
See your doctor as soon as you develop any new symptoms such as blood in the urine or pain in your side. Early diagnosis is critical to treating papillary renal cell carcinoma.
Once you have been diagnosed, reach out to a support group or online community to receive the support you and your loved ones need.
Blood in the urineLow back pain (flank pain)Abdominal pain or swellingUnintended weight lossFatigueFeverA lump in the side of the abdomenSwelling in the veins around the testicle (varicocele) Excessive hair growth in women Pale skinVision changes Liver changes Elevated erythrocyte sedimentation rate (blood test)
