Papillary renal cell carcinoma

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Papillary renal cell carcinoma
Synonyms	Papillary RCC
Pronounce	N/A
Specialty	N/A
Symptoms	Hematuria, flank pain, abdominal mass
Complications	Metastasis, renal failure
Onset	Typically in adulthood
Duration	Chronic
Types	Type 1, Type 2
Causes	Genetic mutations, hereditary syndromes
Risks	Smoking, obesity, hypertension, family history
Diagnosis	Imaging studies, biopsy
Differential diagnosis	Renal cell carcinoma, oncocytoma, angiomyolipoma
Prevention	Lifestyle modification, genetic counseling
Treatment	Surgery, targeted therapy, immunotherapy
Medication	Sunitinib, pazopanib, cabozantinib
Prognosis	Variable, depends on stage and type
Frequency	10-15% of renal cell carcinoma cases
Deaths	N/A

Other Names: RCCP; Renal adenocarcinoma; Papillary renal cell carcinoma, sporadic - (subtype); Papillary renal cell carcinoma, bilateral - (subtype); Papillary renal cell carcinoma, multiple - (subtype); Papillary renal cell carcinoma, familial - (subtype); Papillary renal carcinoma, malignant - (subtype); Papillary renal cell adenocarcinoma Papillary renal cell carcinoma, or PRCC, is a type of kidney cancer. The kidneys work by removing waste products from the blood. Papillary renal cell carcinoma is a cancer of the tubes that filter those waste products from the blood.

Types

There are two types of papillary renal cell carcinoma. Type 1 is more common and grows slowly. Type 2 is more aggressive and grows more quickly.

Epidemiology

PRCC makes up about 15% of all renal cell carcinoma, which is the most common type of kidney cancer.

Cause

Though the exact cause of papillary renal cell carcinoma is unknown, smoking, obesity, and genetic predisposition conditions (such as hereditary leiomyomatosis and renal cell cancer) may contribute to the development of this type of cancer. Scientists are always working to understand how cancer forms, but it can be hard to prove. We do know that patients with PRCC have many mutations in their chromosomes. This can cause the cells not to function as they should.

Inheritance

PRCC usually occurs randomly, which is called sporadic papillary renal cell carcinoma. PRCC can also run in families, but this is rare. One form of PRCC that runs in families is called hereditary papillary renal cell carcinoma. Hereditary papillary renal cell carcinoma usually has a mutation in a part of the chromosomes (the parts of your cells that contain all of your genes) called the MET gene. Another form of PRCC that runs in families is called Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC). This is characterized by alterations in a gene called fumarate hydratase. Patients or affected relatives with this condition may also have skin tumors called leiomyomas and uterine fibroids, which usually happen at a young age.

Symptoms

Some people have symptoms with PRCC, but others don’t. Symptoms can include:

• Blood in the urine
• Pain
• Weight loss
• Feeling tired
• Fever
• A lump in the side

Diagnosis

Sometimes, papillary renal cell carcinoma is found in a patient accidentally while performing a physical exam or a scan for something else. Imaging: If you have symptoms of PRCC, your doctor will use imaging scans such as CT, MRI, and ultrasound to look at where the PRCC tumor is in the kidney and how big it is. They will also check for signs that the tumor has spread to other parts of the body. Biopsy: To check if the tumor is PRCC, your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study the cells from the sample under the microscope to see what kind of tumor it is.

Treatment

Treatment for papillary renal cell carcinoma for each patient is unique. You should go to an expert in PRCC treatment to decide the best approach for your tumor. You can contact MyPART for help fining experts near you. Surgery: Once papillary renal cell carcinoma is diagnosed, you may have surgery to remove the tumors from the kidney. In later stages, surgery may be difficult, in which case your doctor will discuss othe options with you. Radiation Therapy: The radiation is aimed at the tumor area to kill the tumor cells and to prevent it from growing back. Chemotherapy: When surgery is not possible or when the cancer has spread, chemotherapy may be used to treat PRCC, but it is not used often. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Sorafenib (Brand name: Nexavar) Treatment of patients with advanced renal cell carcinoma.

• Aldesleukin (Brand name: Proleukin¬Æ)Treatment of adults (>18 years old) with metastatic renal cell carcinoma.
• Temsirolimus (Brand name: Torisel¬Æ)Treatment of advanced renal cell carcinoma.

Prognosis

In general, type 2 papillary renal cell carcinoma has a poorer prognosis than type 1.

NIH genetic and rare disease info

• NIH info on Papillary renal cell carcinoma

Papillary renal cell carcinoma is a rare disease.

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