What are the other Names for this Condition? (Also known as/Symptoms)
- Primary RMS of Kidney
- Primary Renal Rhabdomyosarcoma
- Primary Renal RMS
What is Primary Rhabdomyosarcoma of Kidney? (Definition/Background Information)
- A rhabdomyosarcoma (RMS) is a very uncommon, high-grade malignant ‘skeletal muscle’ tumor of the soft tissues. RMS tumors are poorly-defined, fleshy, expanding masses that form deep within the body tissues, or below the skin surface
- Primary Rhabdomyosarcoma of Kidney is a very rare, aggressive form of kidney cancer. The tumor is observed in both children and adults
- However, in order for the tumor to be called ‘primary’, the tumor must arise from kidney tissue, there must be no rhabdomyosarcoma present anywhere else in the body, and the sarcomatoid variant of renal cell carcinoma needs to be excluded
- 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 Rhabdomyosarcoma 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 Rhabdomyosarcoma 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 Rhabdomyosarcoma of Kidney? (Age and Sex Distribution)
- Primary Rhabdomyosarcoma of Kidney is seen in individuals of all age groups (children and adults)
- Both males and females are affected and no gender preference is noted
- The cancer is not specific to any ethnic or racial group
- Renal Rhabdomyosarcomas are extremely rare sarcomas (types of malignancies)
What are the Risk Factors for Primary Rhabdomyosarcoma of Kidney? (Predisposing Factors)
No clearly established risk factors are noted for Primary Rhabdomyosarcoma of Kidney. But, in general, rhabdomyosarcomas may be associated with the following factors:
- Family history of the condition has been observed, though very rarely
- Use of drugs, such as cocaine or marijuana, by the parent mother
- Carcinogens (some have been found on mice and certain types of fish)
- Beckwith-Wiedemann syndrome
- Costello syndrome
- Li-Fraumeni syndrome
- Neurofibromatosis (type) 1
- Noonan syndrome
Also, some 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 Rhabdomyosarcoma of Kidney? (Etiology)
- Currently, scientists do not know the definitive factor(s) causing Primary Rhabdomyosarcoma of Kidney
- The tumor may arise due to inherited genetic defects or de novo (sporadic and spontaneous) genetic mutations
In general, it is known that cancers form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor.
- The transformation of normally healthy cells into cancerous cells may be the result of genetic mutations. Mutations allow the cancer cells to grow and multiply uncontrollably to form new cancer cells
- These tumors can invade nearby tissues and adjoining body organs, and even metastasize and spread to other regions of the body
What are the Signs and Symptoms of Primary Rhabdomyosarcoma of Kidney?
The signs and symptoms associated with Primary Rhabdomyosarcoma of Kidney may include:
- Blood in the urine (hematuria)
- Fatigue due to anemia
- A visible lump may be seen on the side of the abdomen
- The tumor can vary in size from a few mm to over 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)
- Small-sized tumors may be asymptomatic
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 Rhabdomyosarcoma of Kidney Diagnosed?
A healthcare provider might employ one or several of the following tools to diagnose Primary Rhabdomyosarcoma 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 and 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
- The differential diagnosis of Primary Rhabdomyosarcoma of Kidney may include:
- Metastasis of RMS to kidney
- Nephroblastoma in children
Note:
- Extensive tumor sampling during pathological examination is important to arrive at a definitive diagnosis
- In children, Renal Rhabdomyosarcoma can be confused with nephroblastoma
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 Rhabdomyosarcoma of Kidney?
The possible complications associated with Primary Rhabdomyosarcoma 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 Rhabdomyosarcoma of Kidney Treated?
There are no established treatment guidelines, since a Renal Rhabdomyosarcoma is a very rare entity. Nevertheless, the treatment measures for Primary Rhabdomyosarcoma 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 rhabdomyosarcoma) 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 Rhabdomyosarcoma 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 rhabdomyosarcoma)
- 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 Rhabdomyosarcoma of Kidney be Prevented?
Current medical research has not established a method of preventing Primary Rhabdomyosarcoma of Kidney.
- Genetic counseling and testing: If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
- 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 for Primary Rhabdomyosarcoma of Kidney? (Outcomes/Resolutions)
The prognosis of Primary Rhabdomyosarcoma 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 Rhabdomyosarcoma 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 Rhabdomyosarcoma of Kidney:
There are a few subtypes of RMS tumors that include:
- Alveolar variant of rhabdomyosarcoma
- Spindle cell variant of rhabdomyosarcoma
- Embryonal variant of rhabdomyosarcoma
Please visit our Cancer & Benign Tumors Health Center for more physician-approved health information:
http://www.dovemed.com/diseases-conditions/cancer/
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