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

• ORTI (Ossifying Renal Tumor of Infancy)
• Ossifying Renal Tumour of Infancy

What is Ossifying Renal Tumor of Infancy? (Definition/Background Information)

• Ossifying Renal Tumor of Infancy (ORTI) is a very uncommon benign tumor of childhood, usually observed within the first 24 months
• 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 is typically present in the medulla of the kidney
• The cause and risk factors for Ossifying Renal Tumor of Infancy are not well-established or identified
• The signs and symptoms depend upon the size of the tumors. It may include blood in urine, although abdominal pain is generally not observed. Large tumors are even known to cause chronic kidney failure
• Typically, a surgical excision of Ossifying Renal Tumor of Infancy with its entire removal is the treatment of choice. This is also performed to confirm a diagnosis of ORTI. The prognosis is excellent with its complete removal, since it is a benign tumor

Who gets Ossifying Renal Tumor of Infancy? (Age and Sex Distribution)

• Ossifying Renal Tumor of Infancy affects children below age 2 years. The age at diagnosis may range from 4 days to 30 months
• Many of these tumors affect boys more than girls (male-female ratio is 3:1)
• No ethnic or racial preference is seen
• ORTI is extremely rare; less than 20 cases have been observed worldwide

What are the Risk Factors for Ossifying Renal Tumor of Infancy? (Predisposing Factors)

• Currently, no definitive risk factors have been noted for Ossifying Renal Tumor of Infancy.

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 Ossifying Renal Tumor of Infancy? (Etiology)

The exact cause and mechanism of Ossifying Renal Tumor of Infancy is generally unknown.

What are the Signs and Symptoms of Ossifying Renal Tumor of Infancy?

The signs and symptoms of Ossifying Renal Tumor of Infancy depend on the size and location of the tumor. It can also vary from one child 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 ORTI may include the following:

• It may be associated with painless hematuria (blood in urine)
• The tumor affects the medulla (central portion) of the kidney
• The tumor size is usually about 2-3 cm, but some are larger
• It is often well-defined and demarcated

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 Ossifying Renal Tumor of Infancy Diagnosed?

The diagnosis of Ossifying Renal Tumor of Infancy 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 and conditions is considered, before arriving at a conclusion. These include:

• Renal staghorn calculi
• Calcified hematoma
• Infections

Note:

• A diagnosis is made after careful analysis of the tumor specimen and extensive sampling of tumor to rule out malignancy
• Calcification may be observed on radiological studies

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 Ossifying Renal Tumor of Infancy?

The complications of Ossifying Renal Tumor of Infancy may include:

• Stress and anxiety due to a concern of kidney cancer
• Chronic renal failure affecting kidney function, if tumors are large
• 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
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Ossifying Renal Tumor of Infancy Treated?

The treatment measures for Ossifying Renal Tumor of Infancy may include the following:

• 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
• 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 Ossifying Renal Tumor of Infancy be Prevented?

• Current medical research has not established a method of preventing Ossifying Renal Tumor of Infancy
• Regular medical screening at periodic intervals with tests and physical examinations are recommended following tumor diagnosis

What is the Prognosis of Ossifying Renal Tumor of Infancy? (Outcomes/Resolutions)

• The prognosis of Ossifying Renal Tumor of Infancy is typically excellent with appropriate treatment (surgical excision and removal of tumor), since these are benign
• However, the prognosis may also depend upon the severity of the signs and symptoms. It also depends upon the overall health of the affected child

Additional and Relevant Useful Information for Ossifying Renal Tumor of Infancy:

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

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