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

• Cystic Lymphangioma of Kidney
• Renal Lymphangiectasia
• Renal Lymphangioma

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

• Lymphangioma of Kidney is a rare benign tumor of lymphatic blood vessels. A lymphangioma is seen among a wide age range of adults and can occur at various locations in the body. But, the kidney is an uncommon location for the tumor
• Lymphangioma of Kidney occurs more commonly in middle-aged women. Generally, the tumor is 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 Lymphangioma of Kidney with its entire removal is the treatment of choice. The prognosis is excellent with its complete removal, since it is 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 one’s response to treatment

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

• Lymphangioma of Kidney is usually seen in adults (average age is 34 years), but may be seen from birth to 79 years. About a third of the tumors are observed in children, while the rest are noted in adults
• There are cases that are repeated at birth indicating the tumor may be caused by congenital malformation
• Lymphangioma of Kidney have a predilection for females, for about 60% of the reported cases occur in them
• No specific ethnic or racial preference is seen

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

Currently, no definitive risk factors have been noted for the Lymphangioma of Kidney. It may occur when there is a positive family history of the condition.

Lymphangioma of Kidney can be associated with conditions such as the following:

• Renal vein thrombosis
• Hypertension
• Pregnancy-related lymphatic obstruction

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 Lymphangioma of Kidney? (Etiology)

The exact cause and mechanism of Lymphangioma of Kidney formation is unknown. The tumors arise from lymphatic blood vessels due to congenital malformation.

• The tumor is associated with a family history of the condition
• Studies have shown that Lymphangioma of Kidney may show certain genetic mutations. These mutations may help in the diagnosis. Some of the mutation detected include:
  • Monosomy X chromosome
  • Trisomy 7q
  • Mutations in the VHL gene (von Hippel-Lindau gene)

What are the Signs and Symptoms of Lymphangioma of Kidney?

The signs and symptoms of Lymphangioma 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 signs and symptoms of Lymphangioma of Kidney may include the following:

• The kidney symptoms may be caused by large tumors due to mass effect (presence of bulky tumors)
• The tumor may be present as an abdominal mass
• 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
• Size of the tumor, up to 19 cm, has been observed
• Collection of fluid in abdomen (ascites)
• The tumors may grow larger during pregnancy

The tumor can occur in the capsule, cortical area, or peri-pelvic area of the kidney.

How is Lymphangioma of Kidney Diagnosed?

In many, Lymphangioma of Kidney is diagnosed incidentally during imaging studies undertaken for some other medical conditions. The diagnosis 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

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:

• Cystic renal dysplasia
• Hereditary polycystic kidney disease
• Familial renal lymphangiomatosis

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 Lymphangioma of Kidney?

The complications of Lymphangioma of Kidney 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
• 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 is small and present in one kidney. Rarely both kidneys may be affected
• 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 Lymphangioma of Kidney can turn malignant.

How is Lymphangioma of Kidney Treated?

The treatment options vary from one individual to another. The treatment measures for Lymphangioma 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 of Lymphangioma of Kidney 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 surgical treatment methods 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
• Treatment of the underlying or associated conditions, if any
• 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 Lymphangioma of Kidney be Prevented?

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

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

The prognosis of Lymphangioma of Kidney depends upon the severity of the signs and symptoms. It also depends upon the overall health of the individual and response to therapy.

• Typically, individuals with small-sized tumors have a better prognosis 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
• Complications are seen in some cases leading to severely affected kidney function, even causing fatalities. This can occur from hemorrhage of tumor into the retroperitoneal space - mostly with large tumors. Individuals with retroperitoneal hemorrhage may have a worse prognosis, if the condition is not treated immediately as a medical emergency
• In pregnant women, such hemorrhage from Lymphangioma of Kidney tumors can be significantly serious affecting both the mother and child

Additional and Relevant Useful Information for Lymphangioma of Kidney:

A lymphangioma can occur at various locations in the body, such as the soft tissues of the head/neck region, liver, and lung.