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

• Renal Spiradenocylindroma
• Sweat Gland Tumor-like Renal Tumor

What is Spiradenocylindroma of Kidney? (Definition/Background Information)

• Spiradenocylindroma of Kidney is an extremely rare benign tumor of the kidney. Due to its rarity, the age and gender parameters are not well-known
• The tumor is observed to be present in the cyst of the kidney and has features of both a spiradenoma and a cylindroma (both benign tumors). This renal tumor histologically resembles a sweat gland type tumor
• The cause of formation of Spiradenocylindroma of Kidney tumors is due to genetic alterations. In many cases, the tumors may be found incidentally while examining the individual for other medical conditions
• The signs and symptoms depend upon the size of the tumors and may include abdominal pain and blood in urine. Large tumors may cause symptoms due to mass effect of the tumor
• Typically, a surgical excision of Spiradenocylindroma of Kidney with its entire removal is the treatment of choice. The prognosis is excellent with its complete removal, since it is usually a benign tumor

Who gets Spiradenocylindroma of Kidney? (Age and Sex Distribution)

• Spiradenocylindroma of Kidney is an extremely uncommon tumor
• No clear data on demographics (age and gender) are presently available, due to the rarity of the tumor
• No specific ethnic or racial preference has been recorded

What are the Risk Factors for Spiradenocylindroma of Kidney? (Predisposing Factors)

• Currently, no definitive risk factors have been noted for Spiradenocylindroma of Kidney
• However, the presence of cutaneous spiradenocylindroma may be a risk factor, in some cases

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 Spiradenocylindroma of Kidney? (Etiology)

The cause of Spiradenocylindroma of Kidney formation is due to genetic factors. The following abnormalities have been recorded:

• Chromosomal anomaly, particularly loss of heterozygosity (LOH) at 16q12-13 involving the CYLD1 gene
• Isochromosome i(16p)

What are the Signs and Symptoms of Spiradenocylindroma of Kidney?

The signs and symptoms of Spiradenocylindroma of Kidney 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 Renal Spiradenocylindroma may include the following:

• It may be present as an abdominal mass
• The tumor is well-defined and demarcated
• It may be associated with urinary tract infections, blood in urine, increased blood pressure, and flank pain
• In general, 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

How is Spiradenocylindroma of Kidney Diagnosed?

The diagnosis of Spiradenocylindroma of Kidney 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 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
• 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 may be performed

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

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

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 Spiradenocylindroma of Kidney?

The complications of Spiradenocylindroma of Kidney may include:

• Stress and anxiety due to a concern of kidney cancer
• Chronic renal failure affecting kidney function, if tumors are large; especially, when the condition is bilateral (affecting both kidneys). In majority of cases, the tumor affects one kidney
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Spiradenocylindroma of Kidney Treated?

The treatment options vary from one individual to another. The treatment measures for Spiradenocylindroma of Kidney 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 is made
• Surgical intervention with complete excision can result in a cure. It can also help reduce the chances of tumor recurrence

The surgical treatment methods may include:

• Endoscopic surgery
• Nephron-sparing surgery
• Partial or complete nephrectomy

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
• 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 Spiradenocylindroma of Kidney be Prevented?

• Current medical research has not established a method of preventing Spiradenocylindroma of Kidney
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Spiradenocylindroma of Kidney? (Outcomes/Resolutions)

The prognosis of Spiradenocylindroma of Kidney depends upon the severity of the signs and symptoms. It also depends upon the overall health of the individual. However, since these tumors are benign, the prognosis is generally excellent.

Additional and Relevant Useful Information for Spiradenocylindroma of Kidney:

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

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