What is Renal Leiomyosarcoma?
Renal leiomyosarcoma (LMS) is a rare and aggressive type of tumor that typically starts in the smooth muscle cells found in the small blood vessels or in the renal pelvis, an area where urine collects in the kidney. This type of cancer is not very common, making up only 1%-2% of all kidney cancers. It usually affects people between 50 to 60 years old, with women being more likely to get it.
Rhe renal LMS cancer has a high risk of coming back in the same area, so doctors usually recommend removing it completely through surgery. Unfortunately, this type of cancer often spreads to other parts of the body through the bloodstream and generally has a poor prognosis, meaning the outlook for successful treatment is low.
Diagnosing this form of kidney cancer should be done very carefully. Its prognosis is notably poor, so it’s important to distinguish it from a similar type of kidney cancer known as sarcomatoid renal cell carcinoma. By differentiating between these two, doctors can make sure they’re choosing the right treatment approach.
What Causes Renal Leiomyosarcoma?
Just a few factors that might increase the risk of developing leiomyosarcoma (a type of rare cancer that can occur in many parts of the body) have been identified. Even less is known about what specifically increases the risk for renal leiomyosarcoma, a form of this cancer that affects the kidneys. In general, both types of leiomyosarcoma are more common in women, with females making up 59-76% of all cases.
Some experts suggest that this might be due to the impact of hormones, but evidence to support this idea is currently not strong enough to draw conclusive results.
Risk Factors and Frequency for Renal Leiomyosarcoma
Leiomyosarcoma, also known as LMS, is a type of sarcoma that often occurs in the kidney. Even though it’s the most common kidney sarcoma, it’s still a very rare type of tumor, accounting for just 0.1% of all kidney malignancies. Renal LMS is found more often in females. Typically, people are diagnosed with this condition between the ages of 40 and 60, with the average age of diagnosis being 58.5. However, it can occur in people as young as 22 and as old as 85.
Signs and Symptoms of Renal Leiomyosarcoma
Leiomyosarcoma (LMS) is a type of kidney cancer that often shows similar symptoms to other kidney cancer types, making it difficult to distinguish initially. The signs of LMS usually appear later in the disease’s progression. Common symptoms include blood in the urine, abdominal pain, and a noticeable lump in the abdomen. There have even been cases where LMS has led to unexpected bleeding in the retroperitoneal space (the area in the back of the abdomen) and drops in blood pressure.
Testing for Renal Leiomyosarcoma
Advances in imaging technology like CT (a type of X-ray) and MRI (a type of scan that uses magnetic fields and radio waves) have greatly improved the way we diagnose kidney cancers, and tell apart harmless kidney tumors from the harmful ones. This information is vital for picking the best treatment options. However, a rare kind of kidney cancer called renal leiomyosarcoma doesn’t have any unique characteristics that can be picked up by these scans. In addition, while CT scans are usually good for detecting tumors in the abdomen, they are not as good for spotting renal leiomyosarcoma.
Further complicating things, it’s often hard to distinguish this rare kidney cancer from a more common kidney cancer called renal cell carcinoma just by looking at the images. Therefore, doctors often need to take a small piece of the tumor for testing in the lab. They do this with a procedure called a CT-guided core needle biopsy, which involves using a CT scan to guide a needle into the tumor to take a sample. Without this biopsy, making the right treatment plan would be very difficult.
Getting this biopsy, which is done by inserting a needle into the kidney area, is generally safe and very good at giving accurate results.
Treatment Options for Renal Leiomyosarcoma
Leiomyosarcoma, often abbreviated as LMS, is a rare type of kidney cancer, contributing to 50-60% of total renal sarcomas. To confirm a diagnosis of this rare tumor, it’s typically recommended to conduct a needle core biopsy – a procedure where a small sample of the tumor is taken out with a needle for examination. This method is safe and the risks of spreading cancer cells during this procedure is very low.
There isn’t a universally agreed-upon treatment method for renal LMS as it’s a relatively rare disease. Consequently, there aren’t a lot of trial studies available that focus directly on this specific form of cancer. Hence, treatment methods are typically adapted from other abdominal tumors of similar type and case reports. It is recommended that patients considering treatment should consult with a team of experts specialized in sarcomas (a type of cancer). When possible, it might be beneficial for patients to participate in ongoing clinical trials.
Surgical removal of the tumor, performed with the aim of leaving no cancer cells behind, is traditionally a potentially curative route of treatment. For a large number of aggressive leiomyosarcomas that have a tendency to come back, a radical nephrectomy, which involves removing the entire kidney, could be a better management option providing long-term cancer control. In cases where a surgeon is expecting to leave some tumor cells behind after surgery, intraoperative radiation therapy can help improve control of the disease and survival rate.
If standard treatment isn’t possible or if the patient isn’t eligible for it, the approach to treatment can vary depending on the grade and growth of the tumor. If there is a high risk of the cancer spreading, treatments may involve preoperative radiotherapy or chemotherapy. Patients who respond to these treatments tend to do better in the long run. Neoadjuvant therapy, where medicine is given before surgery, is currently being studied in a European trial.
Radiotherapy is typically given before the surgery and isn’t used after surgery if all visible tumor has been removed. Postoperative or adjuvant chemotherapy, is recommended if the whole tumor can’t be removed, and in cases where the cancer has spread. A common chemotherapy regimen may include drugs Anthracycline and Ifosfamide.
A study evaluated the efficacy of a drug known as sunitinib malate in treating patients with a certain type of abdominal tumor that cannot be surgically removed or has spread to other parts of the body. Results varied among different subtypes, but showed promising results for patients with LMS. More extensive and multicentric, phase 3 studies are required to validate these initial findings.
What else can Renal Leiomyosarcoma be?
Renal leiomyosarcoma (LMS) is a rare type of kidney cancer, and it’s the most prevalent kind of another rare cancer called primary renal sarcoma. However, before diagnosing renal LMS, it’s important to rule out the presence of other conditions that may mimic its symptoms or characteristics.
- Retroperitoneal leiomyosarcoma, which affects the area behind the abdomen and can involve the kidneys. Radiological techniques are often used to help identify this.
- Sarcomatoid carcinoma of the kidney, which typically arises from the renal cell or sometimes from the urothelial carcinoma. If a biopsy shows malignant epithelial component, it suggests sarcomatoid carcinoma rather than LMS. Immunohistochemistry, which identifies cells in tissues, can also be helpful.
- Angiomyolipoma, another type of kidney tumor, can look very similar to renal LMS under a microscope. However, specific characteristics, such as mature adipose tissue and the reactivity for a melanocytic marker known as HMB-45, serve as distinguishing factors.
- Renal leiomyomas are benign and very rare types of tumor. They look similar to LMS from the outside but can be distinguished through a histological evaluation, which involves studying the minute structure, composition, and function of tissues.
In summary, each of these conditions have certain unique factors that help differentiate them from Renal LMS. Making the right diagnosis often involves a detailed microscopic study of the tumors and several additional tests.
What to expect with Renal Leiomyosarcoma
Renal leiomyosarcoma, a type of kidney cancer, typically has a tough outlook with people surviving around 25 months on average. Among different types of soft-tissue sarcomas (a group of cancers growing in the tissues that connect, support, and surround other body structures), the stage or progress of the disease significantly affects the patient’s survival chances. Other factors also influence the prognosis, including the subtype of the tumor, the severity or grade of the tumor, the person’s age, and gender.
The tendency for the tumor to return locally (in the same place) and the risk of it spreading to other parts of the body is decided by the severity of the tumor and whether it extends into the inferior vena cava (a large vein that carries deoxygenated blood from the lower and middle body into the heart).
Possible Complications When Diagnosed with Renal Leiomyosarcoma
There have been rare instances where patients unexpectedly experienced retroperitoneal hemorrhage (an internal bleed in the rear area of the abdomen) and lowered blood pressure. A study of 67 patients who had renal vein leiomyosarcoma (a type of cancer in the kidney), showed disease progression in about 50% of them despite undergoing treatment. Additionally, the cancer spread to distant parts of the body such as the liver, lungs, bones, and soft tissue in 30% of the patients. Notably, local recurrence, or the cancer coming back in the same area, was seen in 4.5% of the patients. Combined local recurrence and distant spread of cancer was witnessed in 15% of the subjects. Intra-renal leiomyosarcoma displayed similar trends.
- Unexpected internal bleed in rear of abdomen and low blood pressure were rare findings.
- In a group of 67 kidney cancer patients, the disease progressed in 50% even after treatment.
- Cancer spread to other parts of the body in 30% of these patients.
- 4.5% experienced a return of the cancer in the same area.
- 15% had the cancer return in the same area and spread to other areas of the body.
- Intra-renal leiomyosarcoma, another type of kidney cancer, showed similar behavior.
Preventing Renal Leiomyosarcoma
Renal leiomyosarcoma (LMS) is a rare type of kidney cancer that is very aggressive and generally has a low chance of successful treatment. There are no known risk factors, which means there’s no current way to prevent it. Research indicates that many people with this cancer, particularly those with more severe forms of the tumor, sadly don’t survive the disease.
Education is very important for patients with this condition. Because this type of cancer often comes back within the first two years, it’s crucial for patients to consistently attend their follow-up appointments. By doing this, doctors may be able to spot the disease’s return early, which could help improve treatment results.
