What are the other Names for this Condition? (Also known as/Symptoms)

• Chromophil Renal Cell Carcinoma
• PRCC (Papillary Renal Cell Carcinoma)

What is Papillary Renal Cell Carcinoma? (Definition/Background Information)

• Papillary Renal Cell Carcinoma (PRCC) is a type of renal cell carcinoma (kidney cancer) that develops from the renal cortex. It is a malignant tumor that develops due to an abnormality within the tissue lining in the tubules of the kidney, bladder, ureter, or urethra
• There are 2 types of PRCC reported:
  • Type 1 Papillary Renal Cell Carcinoma: It is typically a slow-growing tumor. Hereditary PRCC belongs to this type
  • Type 2 Papillary Renal Cell Carcinoma: It is usually a more rapid-growing tumor
• Papillary Renal Cell Carcinoma generally affects adult males and females; though it is more common in older men. The exact cause for the development of non-hereditary types of Papillary Renal Cell Carcinoma is presently unknown
• The risk factors for developing PRCC include a family history of the disease (that may result in hereditary PRCC), smoking, exposure to toxins, high blood pressure, obesity, and other factors
• A small number of individuals have hereditary Papillary Renal Cell Carcinoma, which is linked to mutation in a gene known as c-met. This type of carcinoma is inherited in an autosomal dominant manner. In other words, inheriting a single defective copy of the gene is enough to cause the disease
• The typical symptoms of Papillary Renal Cell Carcinoma are blood in the urine, a lump on the side, unexplained fever, flank pain, and unexplained weight loss. PRCC may give rise to complication such as spreading of cancer to other parts of the body.
• The healthcare provider might diagnose the condition using blood tests and imaging studies as necessary. Genetic testing may be required, if hereditary Papillary Renal Cell Carcinoma is suspected
• The treatment options include surgery, radiation therapy, chemotherapy, and targeted drug therapy. The prognosis of PRCC depends on the type and size of tumor, whether it is localized in the kidney or has metastasized, and if the disease is in its early or advance stages, at the time of diagnosis
• Although inherited PRCC might not be preventable, being aware of one’s family history and having regular check-ups might help diagnose the condition early. Leading a healthy lifestyle, managing an ideal body weight, controlling blood pressure, avoiding unnecessary medication, smoking cessation, and other such factors can potentially help one avoid cancer formation

The main types of kidney cancers that arise from the renal cortex include:

• Clear cell renal cell carcinoma: This is also called conventional renal cell carcinoma. 65% of kidney cancers belong to this type
• Papillary Renal Cell Carcinoma: It is also called Chromophil Renal Cell Carcinoma. They constitute 15% of renal cancers. Some of these cancers can be seen in syndromes such as Hereditary Papillary Renal Cell Carcinoma Syndrome. A majority of individuals with PRCC, do not have a family history of kidney cancer
• Chromophobe renal cell carcinoma: They make up for 5% of the renal cancers. Usually, it is a slower-growing cancer when compared to other kidney cancers
• Collecting duct carcinoma of kidney: They make up for approximately 1% of the kidney cancers. The cancer arises from the collecting ducts of the kidney

Who gets Papillary Renal Cell Carcinoma? (Age and Sex Distribution)

• Papillary Renal Cell Carcinoma affects older individuals; the average age of diagnosis is 64 years
• PRCC affects both men and women. However, the disease is reported to be more common in men. 
• The cancer is not specific to any particular ethnic or racial group

What are the Risk Factors for Papillary Renal Cell Carcinoma? (Predisposing Factors)

In a majority of individuals, no clear-cut risk factors for Papillary Renal Cell Carcinoma have been established to date. Some studies have shown that the following factors may predispose an individual to PRCC development:

• Family history of Papillary Renal Cell Carcinoma: If the cancer is present among close family members, then the risk of developing it is increased. A positive family history of hereditary PRCC also increases the risk in some individuals
• Obesity
• High blood pressure
• Smoking
• Exposure to toxins, such as naphthylamine dye, asbestos, lead, polycyclic aromatic hydrocarbons, cadmium, and other chemical compounds
• Continuous use of certain medications such as non-steroidal anti-inflammatory drugs
• Long-term dialysis

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your health care provider.

What are the Causes of Papillary Renal Cell Carcinoma? (Etiology)

Currently, scientists do not know the factor(s) causing Papillary Renal Cell Carcinoma.

• In a majority of individuals, hereditary Papillary Renal Cell Carcinoma is linked to mutation in a gene known as c-met. This type of PRCC is inherited in an autosomal dominant manner. In other words, inheriting a single defective copy of the gene is enough to cause development of the cancer 
• The exact cause for development of non-hereditary types of Papillary Renal Cell Carcinoma is not known at this time
• Certain genetic mutations have been detected. Currently, research is underway to well characterize these mutations

What are the Signs and Symptoms of Papillary Renal Cell Carcinoma?

The signs and symptoms associated with Papillary Renal Cell Carcinoma include:

• Small tumors usually do not cause any symptoms. But, occasionally they may become painful, if they compress the surrounding structure
• Blood in the urine
• Fatigue
• A visible lump may be seen on the side of the abdomen
• Fluid accumulation in the lower legs (pedal edema)
• Unexplained fever
• Flank pain
• Unexplained weight loss
• Night sweats
• Generalized weakness and pain in the body

The signs and symptoms may depend on the size of the kidney tumor.

How is Papillary Renal Cell Carcinoma Diagnosed?

A physician might employ one or several of the following tools to diagnose Papillary Renal Cell Carcinoma:

• Evaluation of the patient’s personal and family medical history
• A complete physical examination
• Blood tests
• Urine analysis
• Plain x-ray of the abdomen
• Ultrasound scan of the abdomen
• Computed tomography (CT) scans of the kidneys: CT scans with contrast of the affected region usually shows a well-defined mass, which may have calcifications
• Magnetic resonance imaging (MRI) and positron emission tomography (PET) scans may help differentiate between benign versus malignant tumors by detecting areas of metastasis (if any)

Although the above modalities can be used to make the initial diagnosis, a tissue biopsy of the tumor may be required to make a definitive diagnosis to begin treatment.

• The tissue for diagnosis can be procured in multiple different ways, and they include:
  • Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
  • Core biopsy of the tumor
  • Open biopsy of the tumor
• A tissue biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely electron microscopic studies to assist in the diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Papillary Renal Cell Carcinoma?

The possible complications associated with Papillary Renal Cell Carcinoma include:

• PRCC can occur in multiple locations in the same kidney or it may involve both kidneys
• Metastasis: The cancer can spread to other areas of the body such as to the lung, stomach, or bone

How is Papillary Renal Cell Carcinoma Treated?

The treatment measures for Papillary Renal Cell Carcinoma may include the following:

• Surgery:
  • In majority of individuals, surgical resection of the tumor with clear margins may result in a cure, especially if the tumor is confined to the kidney. In some cases, due to location of the tumor, a complete surgical removal may be difficult
  • Several different surgical procedures may be available, such as partial/radical nephrectomy or laparoscopic surgery, depending on the type, size, and location of the tumor
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Radiation therapy:
  • Radiotherapy can be used as primary therapy in situations where the tumor cannot be removed completely, or when the tumor reappears (recurrent Papillary Renal Cell Carcinoma) after surgery
  • Radiotherapy can also be used as an additional therapy after surgery, if there is a possibility of tumor recurrence after surgery, or if there are inadequate margins following surgery (possibility of tumor left behind)
• Chemotherapy can be used for treating Papillary Renal Cell Carcinoma under the following circumstances:
  • The tumor cannot be removed completely (incomplete surgical resection)
  • Tumors that recur after surgery (recurrent PRCC)
  • Tumors that have spread to distant parts of the body (metastatic PRCC)
• Immunotherapy: A patient’s immune system is activated to combat the cancer in this kind of therapy.
• Targeted drug therapy: This kind of drug treatment targets and kills cancer cells specifically, not harming surrounding normal/healthy cells
• Arterial embolization of Papillary Renal Cell Carcinoma is a possible treatment option. Here the blood supply to the tumor is blocked resulting in tumor death

A long-term follow-up is required, because recurrence of the tumor at the site of surgery or metastasis in distant sites have been reported many years after surgery, even with ‘benign-appearing tumors’.

How can Papillary Renal Cell Carcinoma be Prevented?

• Current medical research has not established a way of preventing Papillary Renal Cell Carcinoma formation
• Regular health check-ups might help those individuals with a history of the condition in the immediate family and diagnose PRCC early
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations for those who have already endured the tumor are helpful

In general, preventive methods for Papillary Renal Cell Carcinoma include reducing the contributory risk factors, such as:

• Smoking
• Unhealthy diet and lifestyle
• Obesity
• Exposure to toxins
• Unnecessary medication

What is the Prognosis of Papillary Renal Cell Carcinoma? (Outcomes/Resolutions)

The prognosis of Papillary Renal Cell Carcinoma depends on the size and number of tumors, their localization, and spread.

• The most reliable prognostic factor of Papillary Renal Cell Carcinoma is dependent on whether the tumor can be completely removed through surgery with free margins or not (no traces of the tumor in adjoining tissue)
• The prognosis of PRCC also depends upon a set of several factors, which include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
  • Overall health of the individual. Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual. Older individuals generally have poorer prognosis than younger individuals
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment. Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
• When Papillary Renal Cell Carcinoma is diagnosed in the early stages, the possibility of survival increases and the prognosis is considered good with treatment
• In cases where both kidneys are affected, the prognosis is not projected to be favorable. Additionally, if the cancer has spread to other parts of the body, such as to the liver, lungs, or bone, the possibility of prolonged survival following diagnosis is limited

As with any tumor, it is important to have follow-up appointments with a physician to monitor for any returning tumors.

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