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

• Adult Renal Cystic Nephroma
• Multilocular Cystic Tumor of Kidney in Adults
• Perlman Tumor in Adults

What is Adult Cystic Nephroma? (Definition/Background Information)

• Cystic nephroma of kidney is an uncommon, benign, cystic tumor that may be present in both children and adults. The tumor is usually single and affects one kidney. It can occasionally affect both kidneys
• The kidney consists of 2 zones; the cortex and the medulla. The cortex is the peripheral or outer portion of the kidney, and the medulla is the central or inner portion of the kidney. The tumor may be present in the cortex or medulla of the kidney
• The cause and risk factors for Adult Cystic Nephroma tumors are not well-established, but it may be linked to hormone therapy. Many tumors are found incidentally while examining the individual for other medical conditions
• The signs and symptoms depend upon the size of the tumors. 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 Adult Cystic Nephroma with its entire removal is the treatment of choice. The prognosis is excellent with its complete removal, since it is a benign tumor

Who gets Adult Cystic Nephroma? (Age and Sex Distribution)

• Adult Cystic Nephroma are usually seen in the age group of 40-69 years
• Even though both genders are affected, the female-male ratio is 8:1 (high female predominance observed)
• No ethnic or racial preference is seen

What are the Risk Factors for Adult Cystic Nephroma? (Predisposing Factors)

Currently, no definitive risk factors have been noted for Adult Cystic Nephroma. Nevertheless, the following factors have been suggested for cystic nephromas:

• Hormonal therapy in women
• Hormonal therapy in men for prostate cancer

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 Adult Cystic Nephroma? (Etiology)

The exact cause and mechanism of Adult Cystic Nephroma formation, in a majority of cases, is unknown. 

• The tumor may be caused by excessive hormones, such as estrogen and progesterone, since the tumors are positive for estrogen receptor (ER) and progesterone receptor (PR) on special studies
• Some researchers believe that cystic nephroma may be caused by developmental defects during fetal growth

Note:

• Unlike cystic tumor of childhood, the adult variant does not seem to arise from nephrogenic rests (certain abnormal cell clusters)
• Also, no mutations on the DICER1 gene is noted (that have been recorded in some pediatric tumors)

What are the Signs and Symptoms of Adult Cystic Nephroma?

The signs and symptoms of Adult Cystic Nephroma 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 kidney symptoms may be caused due to mass effect (presence of bulky tumors).

The signs and symptoms of Adult Cystic Nephroma may include the following:

• Most of these tumors are solitary, sporadic, and unilateral (affecting one kidney)
• The tumor can affect the cortex (peripheral portion) or medulla (central portion) of the kidney
• The tumor may be present as an abdominal mass and the tumor size may vary from 5 to 10 cm
• It is often well-defined and demarcated
• 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
• Tumors may grow larger in size due to hormonal influence

How is Adult Cystic Nephroma Diagnosed?

In many cases, Adult Cystic Nephroma is diagnosed incidentally during imaging studies for other medical conditions. The diagnosis of the tumor may involve the following tests and procedures:

• Complete physical exam with evaluation of medical history.
• Plain X-ray of the abdomen
• Ultrasound scan of the abdomen
• CT or CAT scan with contrast of the abdomen may show a well-defined mass. This radiological procedure creates detailed 3-dimensional images of structures inside the body
• MRI scan 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
• 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
• 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 definitive diagnosis. These include:

• Cystic renal cell carcinoma
• Lymphangioma

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 Adult Cystic Nephroma?

The complications of Adult Cystic Nephroma may include:

• Stress and anxiety due to a concern of kidney cancer
• If bleeding occurs in the tumor suddenly, it can cause retroperitoneal hemorrhage, which can result in large blood loss leading to severe shock. This is a potentially life-threatening complication requiring emergency care. In this situation, the signs and symptoms could be nausea, vomiting, back pain, sudden drop in blood pressure, and palpitations. This clinical condition is termed Wunderlich syndrome
• The presence of kidney cysts can cause kidney failure, due to compression of the kidney tissue
• Chronic renal failure affecting kidney function, if tumors are large; especially, when the condition is bilateral (affecting both the kidneys)
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

Research has not conclusively proven that Adult Cystic Nephroma can turn malignant.

How is Adult Cystic Nephroma Treated?

The treatment options vary from one individual to another. The treatment measures for Adult Cystic Nephroma 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, after a diagnosis of the tumor is made through a fine needle aspiration or a core biopsy
• Surgical intervention with complete excision can result in a cure. It can also help reduce the chances of tumor recurrence

The surgical treatment methods for Cystic Nephroma in Adults 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
• Sclerotherapy to collapse the cyst may be effective in some cases. Multiple sessions of sclerotherapy may be needed

A partial or complete nephrectomy may be considered, when large-sized tumors are noted in the kidneys. 

• 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
• Postoperative 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 Adult Cystic Nephroma be Prevented?

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

What is the Prognosis of Adult Cystic Nephroma? (Outcomes/Resolutions)

The prognosis of Adult Cystic Nephroma depends upon the severity of the signs and symptoms. It also depends upon the overall health of the individual.

• Typically, individuals with small-sized tumors have better prognoses than those with larger-sized tumors
• In most cases, the prognosis of small-sized solitary tumors is excellent with surgical intervention or appropriate treatment, since these are benign

Additional and Relevant Useful Information for Adult Cystic Nephroma:

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

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