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

• Biphasic Synovial Sarcoma of Kidney
• Monophasic Synovial Sarcoma of Kidney
• Primary Renal Synovial Sarcoma

What is Primary Synovial Sarcoma of Kidney? (Definition/Background Information)

• Primary Synovial Sarcoma of Kidney is a rare, high-grade type of kidney cancer. The tumor is more commonly observed during adolescence and young adulthood. However, in order for the tumor to be called ‘primary’, there must be no synovial sarcoma present anywhere else in the body
• In general, a synovial sarcoma (SS) is a malignant tumor of the connective tissue, involving the tendon sheath and synovial cells of the joints. However, a synovial sarcoma is not confined to the joint locations alone; but, it may occur in soft tissues all over the body. The main sites are the lower limb joints, followed by the head and neck region
• The risk factors for carcinoma development are unidentified. However, the general risk factors for kidney cancers include a family history of kidney cancer, smoking, exposure to toxins, high blood pressure, obesity, and other factors
• The signs and symptoms of Primary Synovial Sarcoma of Kidney may include blood in urine, a lump on the side of the abdomen, flank pain, unexplained fever, and unexplained weight loss. It can give rise to complication such as the spread of cancer to other parts of the body (metastasis)
• The treatment options include surgery, radiation therapy, chemotherapy, and targeted drug therapy. The prognosis of Primary Synovial Sarcoma of Kidney depends on size of tumor and whether it is localized in the kidney or has metastasized (if it is in its early or advanced stage, at the time of diagnosis). However, despite adequate treatment, the prognosis is usually poor

Who gets Primary Synovial Sarcoma of Kidney? (Age and Sex Distribution)

• Primary Synovial Sarcoma of Kidney is usually seen in adolescents and young adults. However, a wider age range of 17-61 years is observed
• Both males and females are affected and no gender preference is noted
• The cancer is not specific to any ethnic or racial group
• Renal Synovial Sarcomas are extremely rare sarcomas (type of malignancies) and less than 100 cases have been reported worldwide

What are the Risk Factors for Primary Synovial Sarcoma of Kidney? (Predisposing Factors)

No clearly established risk factors are noted for Primary Synovial Sarcoma of Kidney. However, a few genetic defects occurring due to chromosomal translocation have been identified as a recurring feature in many synovial sarcoma case study reports.

Generally, studies have shown that the following factors may predispose an individual to kidney cancer development:

• Family history of kidney cancer: If the cancer is present among close family members, then the risk of developing the cancer is increased
• Obesity
• High blood pressure
• Smoking
• Exposure to toxins, such as naphthylamine dye, asbestos, lead, polycyclic aromatic hydrocarbons, cadmium, and other chemical compounds
• Continuous use of certain medications such as non-steroidal anti-inflammatory drugs
• Long-term dialysis

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 health care provider.

What are the Causes of Primary Synovial Sarcoma of Kidney? (Etiology)

• Currently, scientists do not know the definitive factor(s) causing Primary Synovial Sarcoma of Kidney
• It has been proposed that certain chromosomal translocation (specifically, the translocation of genetic material between chromosome X and chromosome 18) leading to gene abnormalities, may be the initiator of synovial sarcoma development

What are the Signs and Symptoms of Primary Synovial Sarcoma of Kidney?

The signs and symptoms associated with Primary Synovial Sarcoma of Kidney may include:

• In the initial growing phase of these generally deep tumors, they are asymptomatic. An individual may not experience any symptoms, until the tumor has grown into a substantial size
• The soft tissue tumors grow initially at a moderate rate and then, suddenly start progressing rapidly in size
• Due to large size of the tumor, the adjoining organs, nerves, and muscles, may be compressed or restricted. Often these signs, along with pain and tenderness, may be the first indications of the tumor

Some of the symptoms that may be observed include:

• Blood in the urine (hematuria)
• Fatigue due to anemia
• A visible lump may be seen on the side of the abdomen
• The tumors can be large and range in size from 5-20 cm
• Fluid accumulation in the lower legs (pedal edema)
• Flank pain
• Unexplained weight loss
• Night sweats, unexplained fever
• Generalized weakness and pain in the body
• Abnormal liver function test
• If the tumor is associated with paraneoplastic syndrome; then, the following may be noted:
  • Increased blood pressure (hypertension)
  • Increased calcium in blood (hypercalcemia)
  • Increased red blood cells (erythrocytosis)
  • Increased platelets (thrombocytosis)

The signs and symptoms may depend on the size of the kidney tumor. The combination of signs and symptoms may also vary from individual to individual.

How is Primary Synovial Sarcoma of Kidney Diagnosed?

A healthcare provider might employ one or several of the following tools to diagnose Primary Synovial Sarcoma of Kidney tumors:

• Evaluation of the patient’s personal and family medical history
• A complete physical examination
• Blood tests such as complete blood count (CBC), serum calcium, serum albumin, liver function test (LFT), etc.
• Urine analysis such as albumin levels
• Plain X-ray of the abdomen
• Ultrasound scan of the abdomen
• Computed tomography (CT) scans of the kidneys
• Magnetic resonance imaging (MRI) and positron emission tomography (PET) scans may help differentiate benign versus malignant tumors by detecting areas of metastasis (if any)

Although the above modalities can be used to make the initial diagnosis, a tissue biopsy of the tumor may be required to make a definitive diagnosis to begin treatment.

• The tissue for diagnosis can be procured in multiple different ways, and they include:
  • Fine needle aspiration (FNA) biopsy of the 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 tumor
  • Open biopsy of the tumor
• 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 of Primary Synovial Sarcoma of Kidney is considered to eliminate the following tumors prior to arriving at a definitive diagnosis:
  • Ewing’s sarcoma
  • Solitary fibrous tumor of kidney
  • Sarcomatoid renal cell carcinoma
  • Primary retroperitoneal sarcoma
  • Hemangiopericytoma
  • Malignant peripheral nerve sheath tumor (MPNST)
  • Congenital mesoblastic nephroma 

Note:

• Extensive tumor sampling during pathological examination is important to arrive at a definitive diagnosis
• The tumor can occur in 2 histological subtypes when examined by a pathologist under the microscope and these include: Monophasic synovial sarcoma and biphasic synovial sarcoma

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 Primary Synovial Sarcoma of Kidney?

The possible complications associated with Primary Synovial Sarcoma of Kidney include:

• The rarity of the condition may cause a delayed diagnosis leading to metastasis
• Metastasis: The cancer can spread to other areas of the body, since it is an aggressive tumor
• Recurrence of the tumor due to its partial or incomplete surgical removal
• Complications due to surgery
• Side effects of chemotherapy (such as toxicity) and radiation

How is Primary Synovial Sarcoma of Kidney Treated?

There are no established treatment guidelines, since a Renal Synovial Sarcoma is a very rare entity. Nevertheless, the treatment measures for Primary Synovial Sarcoma of Kidney may include the following:

• Surgery:
  • In majority of individuals, surgical resection of the tumor with clear margins may result in a cure, especially if the tumor is confined to the kidney. In some cases, due to location of the tumor, a complete surgical removal may be difficult
  • Several different surgical procedures may be available, such as partial/radical nephrectomy or laparoscopic surgery, depending on the type, size, and location of the tumor
  • Postoperative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Radiation therapy:
  • Radiotherapy can be used as primary therapy in situations where the tumor cannot be removed completely, or when the tumor reappears (recurrent synovial sarcoma) after surgery
  • Radiotherapy can also be used as an additional therapy after surgery, if there is a possibility of tumor recurrence after surgery, or if there are inadequate margins following surgery (possibility of tumor left behind)
• Chemotherapy can be used for treating Primary Synovial Sarcoma of Kidney under the following circumstances (depending on case by case basis):
  • The tumor cannot be removed completely (incomplete surgical resection)
  • Tumors that recur after surgery 
  • Tumors that have spread to distant parts of the body (metastatic synovial sarcoma)
• Immunotherapy: A patient’s immune system is activated to combat the cancer in this kind of therapy
• Targeted drug therapy: This kind of drug treatment targets and kills cancer cells specifically, not harming surrounding normal/healthy cells

A long-term follow-up is required, because recurrence of the tumor at the site of surgery or metastasis in distant sites have been reported many years after surgery.

How can Primary Synovial Sarcoma of Kidney be Prevented?

Current medical research has not established a method of preventing Primary Synovial Sarcoma of Kidney.

• Regular health check-ups might help those individuals with a history of the condition in the immediate family and help diagnose the sarcoma early
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations for those who have already endured the tumor are helpful

In general, preventive methods for kidney cancers include reducing contributory risk factors, such as:

• Smoking
• Unhealthy diet and lifestyle
• Obesity
• Exposure to toxins
• Unnecessary medication

What is the Prognosis of Primary Synovial Sarcoma of Kidney? (Outcomes/Resolutions)

The prognosis of Primary Synovial Sarcoma of Kidney depends on the size of the tumor, their localization, and spread. In general, the prognosis is poor, since the tumors are aggressive malignancies.

• Typically, the prognosis also depends upon a set of several factors, which include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually better with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor. In majority of cases, the tumor is low stage.
  • The surgical resectability of the tumor (meaning, if the tumor can be removed completely)
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
• When Primary Synovial Sarcoma of Kidney is diagnosed in the early stages, the possibility of survival increases and the prognosis is considered good with treatment
• In cases where both kidneys are affected, the prognosis is not projected to be favorable. Additionally, if the cancer has spread to other parts of the body, such as to the liver, lungs, or bones, the possibility of prolonged survival following diagnosis is limited

As with any tumor, it is important to have follow-up appointments with a physician to monitor for any returning tumors.

Additional and Relevant Useful Information for Primary Synovial Sarcoma of Kidney:

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

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