Type 1 papillary renal cell carcinoma
Summary[edit | edit source]
- 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?[edit | edit source]
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[edit | edit source]
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?[edit | edit source]
The exact cause of papillary renal cell carcinoma is unknown.
What are the risk factors?[edit | edit source]
- Smoking,
- obesity,
- genetic predisposition conditions (such as hereditary leiomyomatosis and
- renal cell cancer)
What are the signs and symptoms?[edit | edit source]
- 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?[edit | edit source]
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?[edit | edit source]
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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Contributors: Prab R. Tumpati, MD