Type 1 papillary renal cell carcinoma

Summary

• A papillary renal cell carcinoma characterized by the presence of papillae covered by small cells with scant amount of cytoplasm.
• The cells are arranged in a single layer on the basement membrane of the papillae.

How common is the condition?

It accounts for about 10-15% of all renal cell carcinomas. Renal cell carcinomas are a type of kidney cancer that develop in the lining of very small tubes (tubules) in the kidney. The term "papillary" describes the finger-like projections that can be found in most of the tumors.

What are the different types

PRCC can be divided into two types: type 1, which is more common and usually grows more slowly and type 2, which are usually more aggressive.

What causes it?

The exact cause of papillary renal cell carcinoma is unknown.

What are the risk factors?

• Smoking,
• obesity,
• genetic predisposition conditions (such as hereditary leiomyomatosis and
• renal cell cancer)

What are the signs and symptoms?

• Blood in the urine called hematuria
• Pain in the loin or abdomen
• Weight loss
• Feeling tired
• Fever
• A lump in the side

Sometimes, papillary renal cell carcinoma is found in a patient accidentally while performing a physical exam or a scan for something else.

Imaging: Imaging scans such as CT scan, MRI, and ultrasound to look at where the PRCC tumor is in the kidney and how big it is.

Biopsy:Taking a small sample from the tumor with a needle.

How is it diagnosed?

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

What is the treatment?

Treatment often begins with surgery to remove as much of the cancer as possible, and may be followed by radiation therapy, chemotherapy, biological therapy, or targeted therapy.

FDA-Approved Treatments 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) - Manufactured by Bayer Pharmaceutical Corporation
• Aldesleukin (Brand name: Proleukin®) - Manufactured by Chiron Corporation
• Temsirolimus (Brand name: Torisel®) - Manufactured by Wyeth Pharmaceuticals, Inc.

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