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

• JCT (Juxtaglomerular Cell Tumor)
• Reninoma

What is Juxtaglomerular Cell Tumor? (Definition/Background Information)

• Juxtaglomerular Cell Tumor (JCT) is a mostly benign kidney tumor that secretes renin. The secretion of renin leading to its increased level in blood, results in high blood pressure (hypertension)
• The cause and risk factors for Juxtaglomerular Cell Tumor are not clearly established. The tumor may form due to certain genetic mutations. JCT is typically seen among young adults
• Many Juxtaglomerular Cell Tumors are found incidentally while examining the individual for other medical conditions. The signs and symptoms depend upon the size of the tumors and it may include blood in urine, abdominal pain, and frequent infections affecting the urinary tract system. Large tumors are even known to cause chronic kidney failure
• Typically, a surgical excision of Juxtaglomerular Cell Tumor with its entire removal is the treatment of choice. The prognosis is excellent with its complete removal, since it is usually a benign tumor
• However, the prognosis also depends upon a set of factors that includes the severity of the signs and symptoms, age and overall health of the individual, and response to treatment

Who gets Juxtaglomerular Cell Tumor? (Age and Sex Distribution)

• Juxtaglomerular Cell Tumor is usually seen in the age group 23 to 38 years (average age 27 years)
• However, even children can be rarely affected
• JCT has a predilection for females; the female-male ratio is 2:1
• No specific ethnic or racial preference is seen

What are the Risk Factors for Juxtaglomerular Cell Tumor? (Predisposing Factors)

• Currently, no definitive risk factors have been noted for Juxtaglomerular Cell Tumor
• It may occur when there is a positive family history of the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s 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 healthcare provider.

What are the Causes of Juxtaglomerular Cell Tumor? (Etiology)

The exact cause and mechanism of Juxtaglomerular Cell Tumor formation, in a majority of cases, is unknown.

• Studies have shown that JCT may show certain genetic mutations. These mutations may help in tumor diagnosis
• Some of the mutations detected include:
  • Loss of chromosome 9
  • Loss of chromosome 11

What are the Signs and Symptoms of Juxtaglomerular Cell Tumor?

The signs and symptoms of Juxtaglomerular Cell Tumor depend on the size and location of the tumor. It can also vary from one individual to another. In general, small tumors are asymptomatic and large tumors can cause signs and symptoms.

The signs and symptoms of Juxtaglomerular Cell Tumor may include the following:

• The presence of a well-defined and demarcated tumor
• The tumors can affect either the cortex (peripheral portion) or medulla (central portion) of the kidney
• Increased blood pressure (hypertension) due to production of renin by the tumor cells. Generally, the hypertension does not respond to blood pressure medications
• Headache, urination in copious quantities (polyuria), vomiting, dizziness, and excessive urination at night (nocturia) can occur
• The tumor may be present as an abdominal mass
• The kidney symptoms may be caused by mass effect (presence of bulky tumors)
• Usually the tumors are 2 to 3 cm in size. However, rarely they can grow up to 8-9 cm
• It may be associated with frequent urinary tract infections, blood in urine, increased blood pressure, and flank pain
• Large tumors can severely affect the functioning of the kidney that is involved. Tumors growing to larger sizes can cause compression of adjoining organs and structures, but are not known infiltrate into them

High blood pressure can result in the following signs and symptoms:

• Headaches
• Fatigue
• Altered mental status
• Light-headedness
• Vision problems
• Chest pain when blood supply to the heart decreases
• Urinary symptoms can occur from damage to the kidney

How is Juxtaglomerular Cell Tumor Diagnosed?

. The diagnosis of Juxtaglomerular Cell Tumor may involve the following tests and procedures:

• Complete physical exam with evaluation of medical history
• The combination of increased blood pressure, increased level of renin in blood, and the presence of a renal mass (kidney mass) is very helpful in diagnosing the condition
• The hypertension does not respond to blood pressure medications. This provides a clue that a Juxtaglomerular Cell Tumor may be present
• It must be noted that not all Individuals with the tumor may have increased blood pressure. This makes the diagnosis challenging; it can also result in diagnostic delays
• Plain X-ray of the abdomen
• Ultrasound scan of the abdomen
• CT or CAT scan with contrast of the abdomen usually shows a well-defined mass. This radiological procedure creates detailed 3-dimensional images of structures inside the body
• MRI scans of the abdomen: Magnetic resonance imaging (MRI) uses a magnetic field to create high-quality pictures of certain parts of the body, such as tissues, muscles, nerves, and bones. These high-quality pictures may reveal the presence of the tumor
• Urine analysis to check for the presence of blood cells
• Kidney function test
• Blood tests to determine the following:
  • Serum aldosterone levels
  • Serum renin levels
  • Serum potassium levels
• Intravenous pyelogram (IVP): A dye is injected into the blood vessels and the image of kidney structure is obtained
• Vascular angiographic studies of the tumor

Invasive diagnostic procedures such as:

• Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
• Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

• Fine needle aspiration (FNA) biopsy of the kidney 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 kidney tumor
• Open biopsy of the kidney tumor

Tissue biopsy:

• 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

A differential diagnosis, to eliminate other tumor types is considered, before arriving at a conclusion. The differential diagnosis includes:

• Hemangiopericytoma
• Glomus tumor
• Metanephric adenoma
• Epithelioid angiomyolipoma
• Wilms tumor
• Solitary fibrous tumor

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 Juxtaglomerular Cell Tumor?

The complications of Juxtaglomerular Cell Tumor may include:

• Stress and anxiety due to a concern of kidney cancer
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication
• Research has shown that Juxtaglomerular Cell Tumor can turn malignant, on rare occasions, resulting in tumor metastasis

How is Juxtaglomerular Cell Tumor Treated?

The treatment options vary from one individual to another. The treatment measures for Juxtaglomerular Cell Tumor may include the following:

• Majority of asymptomatic tumors are not surgically removed after the tumor is determined to be benign on a biopsy: The healthcare provider may recommend a ‘wait and watch’ approach for small-sized tumors presenting mild signs and symptoms (such as no increase in blood pressure is noted), after a diagnosis of JCT is made, through a fine needle aspiration or a core biopsy
• Surgical intervention with complete excision can result in a complete cure. It can also help reduce the chances of tumor recurrence
• The increased blood pressure also gets better after surgical removal of the tumor

The surgical treatment methods for JCT may include:

• Endoscopic surgery
• Nephron-sparing surgery
• Partial or complete nephrectomy
• Tumor embolization is a possible treatment option. Here the blood supply to the tumor is blocked resulting in its shrinkage or death

A partial or complete nephrectomy may be considered, when large-sized tumors are noted in the kidneys. In severe cases of bilateral tumors, kidney transplantation may be necessary.

• A kidney dialysis may be required, if the kidney function is severely compromised due to renal failure
• Prompt diagnosis and emergency treatment of any abdominal (retroperitoneal) bleeding due to the tumor should be immediately considered
• Post-operative care is important: A minimum activity level is ensured, until the surgical wound heals
• Follow-up care with regular screening may be recommended by the healthcare provider

How can Juxtaglomerular Cell Tumor be Prevented?

• Current medical research has not established a method of preventing Juxtaglomerular Cell Tumor
• Regular medical screening at periodic intervals with tests and physical examinations are strongly recommended

What is the Prognosis of Juxtaglomerular Cell Tumor? (Outcomes/Resolutions)

• The prognosis of Juxtaglomerular Cell Tumor depends upon the severity of the signs and symptoms. It also depends upon the overall health of the individual, and response to therapy
• In most cases, the prognosis of small-sized solitary tumors is excellent with surgical intervention or appropriate treatment, since these are mostly benign

Additional and Relevant Useful Information for Juxtaglomerular Cell Tumor:

Please visit our Cancer & Benign Tumors Health Center for more physician-approved health information:

http://www.dovemed.com/diseases-conditions/cancer/