Angiomyolipoma

A Angiomyolipoma refers to a benign tumor in the kidney that is characterized by a particularly high proportion of adipose tissue. Angiomyolipomas are extremely rare and affect women between the ages of 40 and 60 in the majority of cases. In about 80 percent of the diseases, the angiomyolipoma in the kidneys is asymptomatic, so that it does not cause any symptoms.

What is Angiomyolipoma?

Angiomyolipoma, a benign tumor with a high fat content, originates from special cells in the kidney, the so-called epithelial cells. It is believed that the tumor grows in a hormone-dependent manner. Angiomyolipomas are round to oval in shape and bulge over the kidney capsule. Sometimes they grow in multiple places, which can also involve the lymph nodes.

However, there is no risk that the angiomyolipomas will begin to metastasize. Histological examinations of angiomyolipomas show that these kidney tumors are characterized in particular by mature fat cells as well as smooth muscle cells and blood vessels.

Asymptomatic angiomyolipomas, which can occur in both kidneys and cause no symptoms, are found with about the same frequency in women and men. This group of patients has an age peak at around 30 years. In some cases of asymptomatic angiomyolipoma, the kidney tumors are associated with tuberous sclerosis (medical name Bourneville-Pringle's disease).

causes

There are several known causes of angiomyolipoma, which can affect the kidneys. In most cases, the angiomyolipoma starts from perivascular epithelial cells. These cells are special cells of the connective tissue that are located around a vessel. According to current knowledge, the growth and control of these perivascular epithelial cells are dependent on certain hormones.

Although angiomyolipomas do not metastasize, in some cases the tumors may grow into the adipose tissue of the kidney, the renal pelvis, or sometimes into the renal veins. This can lead to various complications. The presence of angiomyolipomas accounts for about one percent of all surgical findings. In addition, about 20 percent of the angiomyolipoma is associated with the disease tuberous sclerosis.

Symptoms, ailments & signs

If the patient has an angiomyolipoma, various symptoms can occur. In most cases it is an asymptomatic angiomyolipoma that does not cause any symptoms or complications. As a result, the disease is not noticed by those affected because there are no symptoms.

As a result, angiomyolipoma cannot be diagnosed and treated by a doctor. This is relatively common, as angiomyolipoma is asymptomatic in about 80 percent of cases. In the remaining cases, the affected patient may experience various symptoms caused by angiomyolipoma.

In most cases, the symptoms manifest themselves as flank pain, which gives an indication of the tumor in the kidney. In addition, angiomyolipoma can also lead to potentially dangerous complications.For example, life-threatening bleeding can occur, which is caused by a spontaneous rupture in the retroperitoneum (medical name Wunderlich syndrome). If you are pregnant, the risk of such a rupture is increased.

Diagnosis & course

Various examination methods are available for establishing the diagnosis of angiomyolipoma, which are selected by the attending physician depending on the symptoms shown. For example, imaging methods can be used to diagnose the disease. Ultrasound examinations can reveal pronounced echogenic masses in the kidneys, which can be caused by the high fat content of the angiomyolipoma.

On the other hand, the analysis of the blood vessels in the kidney is not a useful method for differential diagnosis, because angiomyolipoma can lead to neovascularization, similar to the presence of renal cell carcinoma. Computed tomography is also possible. This makes it possible to distinguish it from malignant renal cell carcinoma.

In addition, no calcifications occur in angiomyolipoma, which also contribute to a reliable demarcation. A particularly high fat content can also be visualized as part of an MRI, which is evidence of the presence of an angiomyolipoma and does not refer to renal cell carcinoma.

Complications

Angiomyolipoma is a benign, fatty tissue tumor that lodges in the kidney. Mid-life women are most affected by this symptom. Angiomyolipoma is sometimes associated with tuberous sclerosis. Due to the variety of symptoms such as: flank pain and cramps in the pelvic area, a medical evaluation is recommended.

Once the diagnosis has been made, the resulting complications can be avoided. The rupture spontaneously occurring in the retroperitoneum, also known as Wunderlich's syndrome, is particularly life-threatening. Pregnant women in particular are at increased risk, as there is heavy bleeding behind the peritoneum. The assumption is that the tumor grows hormone-dependent and can spread to the kidney area.

Angiomyolipoma cannot metastasize. Therapeutic measures are initiated depending on the nature and size of the tumor. If the angiomyolipoma is larger than four centimeters, a partial removal of the kidney must be targeted. Often, however, it is only a minimally invasive procedure. As a complication of selective embolization, recurrences can occur.

The necrotic tissue is drained away by means of a drain. Asymptomatic angiomyolipoma, which affects both kidneys, but does not show any complications, is differentiated from the diagnosis. It occurs in women and men around the age of 30. Asymptomatic angiomyolipoma is not noticed by the person concerned.

When should you go to the doctor?

Recurring flank pain, blood in the urine, fatigue, and other symptoms that suggest a serious internal organ disease should be clarified by a doctor. The medical professional can use imaging tests and a medical history to determine whether it is an angiomyolipoma and, if necessary, initiate appropriate treatment. The benign tumor should always be removed surgically. The sooner this happens, the less likely it is for serious complications.

A visit to the doctor is recommended at the latest when the pain increases or suddenly intense, throbbing pain occurs in the flank region. The latter indicates a rupture behind the peritoneum (Wunderlich syndrome), which must be treated immediately. Risk groups such as pregnant women and people with chronic kidney disease should see a doctor immediately if they suspect angiomyolipoma.

Anyone who already suffers from cancer should speak to the responsible doctor if they have unusual symptoms. In the event of severe complications such as circulatory problems, severe pain or a rupture, an ambulance service should also be called.

Doctors & therapists in your area

Treatment & Therapy

Various options are available for the therapeutic treatment of angiomyolipoma, which are selected depending on the clinical picture and severity of the tumor. In the event that the angiomyolipoma is more than four centimeters in size and is associated with pronounced symptoms, partial removal of the kidney must be considered.

Another therapeutic option is what is known as selective embolization, which is a minimally invasive therapy method. With this form of treatment, however, recurrences are possible and drainage of the relevant necrotic tissue may be necessary.

Outlook & forecast

The prognosis of the prospect of a cure for angiomyolipoma depends on the time at which the irregularity is discovered and the subsequent start of treatment.

In the event of an early diagnosis, the changed tissue can be completely removed in a surgical procedure immediately before further growth. There is a possibility that the patient will be symptom-free afterwards and that it will remain permanently. In the further course of development, angiomyolipoma can also grow again.

If the tumor grows into organic tissue, there is a risk of malfunction and permanent impairment of the organ's function. The kidney function is limited and numerous complaints arise. Removal of the affected tissue can cause the kidney to malfunction for life. Depending on the existing damage, there is a need to carry out an organ transplant in order to save the patient's life and maintain his quality of life.

prevention

With regard to angiomyolipoma, according to current scientific knowledge, there are no known methods for preventing the disease. It is all the more relevant to have medical examinations carried out at the first symptoms that may indicate angiomyolipoma. As a symptom, the focus is particularly on flank pain, which can generally indicate various diseases of the kidney and should always be clarified medically.

Aftercare

Cancer requires continuous follow-up care. Because it cannot be ruled out that a tumor will form again. It is statistically proven that after five years the probability of a relapse has decreased significantly. After a close-knit investigation network, only one or two appointments per year are then to be kept.

A follow-up plan is usually discussed as part of the initial therapy. Patients should discuss this with the treating doctor in good time. If the kidney is damaged during the initial treatment, blood washes may be necessary. So far there are no scientifically proven ways to stop angiomyolipoma from developing again.

The general rules for cancer patients prove to be useful. Those affected should maintain a balanced diet after the first illness. Addictive substances such as nicotine and alcohol should be avoided. Daily exercise is also part of a healthy life. Psychological pressure should be avoided and, if necessary, reduced at work.

In the follow-up examination, the attending physician asks extensively about the patient's state of health. Even minor complaints can indicate a new illness. Usually a blood test is done. Imaging methods such as CT and MRT are also used.

You can do that yourself

Depending on its cause, angiomyolipoma can be treated in different ways. Drug therapy is usually used to relieve pain and regulate the hormonal balance. The patient can support this by noting any side effects and interactions as well as the positive effects of the preparations used. An optimally adjusted medication improves the chances of recovery and the patient's well-being.

The actual symptoms can be treated with conservative measures. Cooling pads and protection, for example, help against flank pain. If bleeding is found, the doctor must first be informed.

In addition, avoiding strenuous physical activity can help reduce pain. The patient should follow the doctor's guidelines regarding diet and physical activity during treatment.

Stress and other psychological stress should be reduced if possible. If the course is severe, a therapist can be called in. The doctor in charge must answer what measures those affected can take to reduce the symptoms.