What are the other Names for this Condition? (Also known as/Symptoms)
- MCRNLMP (Multilocular Cystic Renal Neoplasm of Low Malignant Potential)
- Multilocular Cystic Neoplasm of Low Malignant Potential of Kidney
- Renal Cell Carcinoma Extensive Cystic Change
What is Multilocular Cystic Renal Neoplasm of Low Malignant Potential? (Definition/Background Information)
- Multilocular Cystic Renal Neoplasm of Low Malignant Potential (MCRNLMP) is a rare type of renal cell carcinoma (kidney cancer) that develops from the renal cortex. It is a malignant tumor that develops due to an abnormality within the tissue lining in the tubules of the kidney
- MCRNLMP is considered to be a variant of clear cell renal cell carcinoma (CCRCC). The tumor contains multiple cysts, and hence, the name Multilocular Cystic Renal Neoplasm of Low Malignant Potential
- The exact cause of development of Multilocular Cystic Renal Neoplasm of Low Malignant Potential is presently unknown. These well-defined tumors generally affect middle-aged men and women and are usually present in only one kidney (it is also not multifocal)
- The risk factors for the development of Multilocular Cystic Renal Neoplasm of Low Malignant Potential include a family history of the condition (that may result in hereditary MCRNLMP), smoking, exposure to toxins, high blood pressure, and even obesity
- The typical signs and symptoms of Multilocular Cystic Renal Neoplasm of Low Malignant Potential include blood in urine, a lump on the side (abdomen), unexplained fever, flank pain, and inexplicable weight loss. Genetic testing may be required to diagnose the tumor, apart from blood tests and imaging studies
- A surgical removal of the tumor is the main treatment option considered for Multilocular Cystic Renal Neoplasm of Low Malignant Potential. The other treatment measures include radiation therapy, chemotherapy, and targeted drug therapy. The prognosis of MCRNLMP is generally excellent with treatment; a spread of the tumor outside the kidney is not reported
- Although inherited Multilocular Cystic Renal Neoplasm of Low Malignant Potential may not be preventable, being aware of one’s family history and having regular check-ups might help in an early diagnosis. Moreover, leading a healthy lifestyle, managing an ideal body weight, controlling blood pressure, avoiding unnecessary medication, smoking cessation, and other such measures can help one reduce the risk for cancer formation
The main types of kidney cancers that arise from the renal cortex include:
- Clear cell renal cell carcinoma (CCRCC): This is also called conventional renal cell carcinoma. 65% of kidney cancers belong to this carcinoma type. And, approximately 4% of CCRCCs are Multilocular Cystic Renal Neoplasm of Low Malignant Potential
- Papillary renal cell carcinoma: It is also known as chromophil renal cell carcinoma. They constitute 15% of kidney cancers. Some of these cancers can be seen in syndromes such as hereditary papillary renal cell carcinoma syndrome. A majority of individuals with this carcinoma type do not have a family history of kidney cancer
- Chromophobe renal cell carcinoma: They make up for 5% of the kidney cancers. Usually, it is a slower-growing cancer when compared to other kidney cancers
- Collecting duct carcinoma of kidney: They are rare and make up for approximately 1% of the kidney cancers. This cancer arises from the collecting ducts of the kidney
Who gets Multilocular Cystic Renal Neoplasm of Low Malignant Potential? (Age and Sex Distribution)
- Multilocular Cystic Renal Neoplasm of Low Malignant Potential typically affects middle-aged adults; the average age of diagnosis is 47 years. The condition is observed in a wide age range - from 18 to 80 years
- MCRNLMP affects both men and women. However, it is reportedly more common in men (male-female ratio of 2:1)
- The cancer is not specific to any particular ethnic or racial group and all races/ethnic groups are affected
What are the Risk Factors for Multilocular Cystic Renal Neoplasm of Low Malignant Potential? (Predisposing Factors)
In a majority of children, no well-defined risk factors for Multilocular Cystic Renal Neoplasm of Low Malignant Potential have been identified. Some studies have shown that the following may predispose an individual to MCRNLMP or kidney cancer development
- Family history of MCRNLMP: If the cancer is present among close family members, then the risk for developing the same is increased
- Obesity
- High blood pressure (hypertension)
- Smoking history
- Exposure to toxins, such as naphthylamine dye, asbestos, lead, polycyclic aromatic hydrocarbons, cadmium, and other chemical compounds
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 Multilocular Cystic Renal Neoplasm of Low Malignant Potential? (Etiology)
Currently, scientists do not know the definitive factor(s) causing Multilocular Cystic Renal Neoplasm of Low Malignant Potential. The cause of tumor development is being researched into; and, certain genetic mutations have been detected. It is believed that these may be responsible for tumor development.
The genetic mutations include:
- VHL gene mutations in 25% of the cases
- Deletion in chromosome 3p in 75% of the cases
What are the Signs and Symptoms of Multilocular Cystic Renal Neoplasm of Low Malignant Potential?
The signs and symptoms associated with Multilocular Cystic Renal Neoplasm of Low Malignant Potential may vary from one individual to another. Typically, MCRNLMP tumors do not cause any significant symptoms in many; but occasionally may cause pain, if they compress the surrounding structures.
The signs and symptoms associated with MCRNLMP include:
- Blood in urine (hematuria)
- Fatigue due to anemia
- Flank pain
- A visible lump may be seen on the side of the abdomen (if the tumor is large)
- Fluid accumulation in the lower legs (or pedal edema)
- Unexplained fever and night sweats
- Unexplained weight loss
- Generalized weakness and pain in the body
- Increased blood pressure (hypertension)
- The tumors are well defined and usually measure less than 5 cm
Lab tests may reveal the following:
- Increased platelet count (thrombocytosis)
- Increased calcium in blood (hypercalcemia)
- Abnormal liver function tests
- Increased number of red blood cells (erythrocytosis)
The signs and symptoms may also depend on the size of the kidney tumor.
How is Multilocular Cystic Renal Neoplasm of Low Malignant Potential Diagnosed?
A healthcare provider might employ one or several of the following tools to arrive at a diagnosis of Multilocular Cystic Renal Neoplasm of Low Malignant Potential:
- Evaluation of the patient’s personal and family medical history
- A complete physical examination and assessment of the presenting signs and symptoms, if any
- Blood tests such as complete blood count (CBC), serum calcium, serum albumin, etc.
- Liver function tests (LFTs)
- Urine analysis
- 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 between benign versus malignant tumors by detecting areas of metastasis (if any present)
Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor may be required to make a definitive diagnosis to begin treatment of the condition.
- The tissue for diagnosis can be procured in multiple different ways, and they include:
- Fine needle aspiration (FNA) biopsy of the tumor: An FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, FNA biopsies as diagnostic tools have certain limitations, and open surgical biopsies are 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 MCRNLMP may include:
- Clear cell renal cell carcinoma
- Papillary renal cell carcinoma
- Cystic nephroma
- Benign renal cortical cyst
- Tubules cystic carcinoma
Note: Over 90% of the MCRNLMP tumors are diagnosed incidentally during imaging studies that are undertaken for some other conditions.
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 Multilocular Cystic Renal Neoplasm of Low Malignant Potential?
The possible complications associated with Multilocular Cystic Renal Neoplasm of Low Malignant Potential include:
- Renal dysfunction due to large-sized tumors
- Recurrence of the tumor following treatment
- Emotional and psychological stress due to cancer diagnosis
- Side effects due to cancer therapy; complications may arise from surgery to treat the condition
How is Multilocular Cystic Renal Neoplasm of Low Malignant Potential Treated?
The treatment of Multilocular Cystic Renal Neoplasm of Low Malignant Potential may depend upon a consideration of the following set of factors:
- The stage of the cancer
- Size and location of the tumor
- Severity of the signs and symptoms
- Age and overall health status of the individual
- The treatment preferences
The treatment measures for Multilocular Cystic Renal Neoplasm of Low Malignant Potential 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 kidneys. 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
- Post-operative 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 MCRNLMP) after surgery
- Radiotherapy can also be used as an additional therapy tool after surgery, if there is a possibility of tumor recurrence, or if there are inadequate margins following surgery (possibility of residual tumor being left behind)
- Chemotherapy can be used for treating Multilocular Cystic Renal Neoplasm of Low Malignant Potential under the following circumstances:
- The tumor cannot be removed completely (incomplete surgical resection)
- Tumors that recur after surgery (recurrent MCRNLMP)
- Immunotherapy for MCRNLMP: A patient’s immune system is activated to combat the cancer in this kind of therapy
- Targeted drug therapy for MCRNLMP: This kind of drug treatment targets and kills cancer cells specifically, avoiding harm to the surrounding normal/healthy cells
- Arterial embolization of MCRNLMP is a possible treatment option. Here, the blood supply to the tumor is blocked resulting in tumor death
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 Multilocular Cystic Renal Neoplasm of Low Malignant Potential be Prevented?
- Current medical research has not established a method of preventing Multilocular Renal Neoplasm of Low Malignant Potential.
- Regular health check-ups may help those individuals with a family history of the condition
- 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 MCRNLMP include reducing the contributory risk factors such as:
- Smoking
- Unhealthy diet and lifestyle
- Obesity
- Exposure to toxins
- Use of unnecessary medication
What is the Prognosis for Multilocular Cystic Renal Neoplasm of Low Malignant Potential? (Outcomes/Resolutions)
The prognosis of Multilocular Cystic Renal Neoplasm of Low Malignant Potential depends on the size of the tumor and on the success of excisional surgery (surgical removal of the tumor).
- But, typically, the tumor has excellent prognosis with treatment. It does not invade into the blood vessels is not known to metastasize. Also, no sarcomatous change (transformation to a sarcoma) is seen in the tumor
- The most reliable prognostic factor of MCRNLMP is dependent on whether the tumor can be completely removed through surgery with free margins or not (i.e., whether any traces of the tumor remain in the adjoining tissues or not)
- The prognosis also depends upon whether the tumor is occurring for the first time or is a recurrent tumor. Recurring tumors have poorer prognosis compared to tumors that are non-recurrent
As with any malignant conditions, it is important to have regular follow-up appointments with a healthcare provider to monitor for any returning tumors.
Additional and Relevant Useful Information for Multilocular Cystic Renal Neoplasm of Low Malignant Potential:
- Multilocular Cystic Renal Neoplasm of Low Malignant Potential (MCRNLMP) was earlier known as Multilocular Cystic Renal Cell Carcinoma (MCRCC)
The following article link will help you understand other cancers and benign tumors:
http://www.dovemed.com/diseases-conditions/cancer/
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