Angiomyolipomas

As most of you know I have a disease called Tuberous Sclerosis (TS) it has never effected me in the past but I have always been diligent about getting screened. This time I wasn't so lucky. Today I received the results of my ultra sound and they found 4 little (less then 4cm) tumors called angiomyolipomas. My doctor is now going to monito me more carefully. Here is some info from the TS alliance website.

RENAL ANGIOMYOLIPOMAS
Angiomyolipomas are named because they consist of blood vessels ("angio"), smooth muscle ("myo") and fat
("lipoma"). Usually angiomyolipomas are multiple and occur in both kidneys. The presence of fat in angiomyolipomas
often allows them to be distinguished from other renal tumors by CT scan or ultrasound.
Recent studies have given urologists (specialists on the urinary system) and nephrologists (kidney specialists)
insight into the course of renal angiomyolipoma. In this study, two particular factors appear to be somewhat predictive
of the course of the disease. The two factors include the presence or absence of symptoms related to the
angiomyolipoma and the size (less than 4 centimeters or greater than 4 centimeters) of the growth itself.
In their studies, some individuals with tumors less than 4 cm in diameter had no symptoms, while approximately 90
percent of individuals with a tumor greater than or equal to 4 cm appeared to have symptoms. These symptoms
most commonly included abdominal or back pain, nausea and vomiting and fever. For the individual who is nonverbal,
this may be present as irritability and vomiting. However, bleeding or rupture rarely occurred in children;
larger tumors occurred at an older age (greater than 10 years of age). It appears in the limited number of individuals
that they have followed with TSC and angiomyolipoma, in some cases the growths will continue to grow. The
tumors that are larger in size appear to have a greater propensity for growth than the smaller tumors.
Our understanding of the growth of renal angiomyolipoma and TSC is in its infancy and we will have further information
in a few more years. The real danger of a large angiomyolipoma is that it can rupture and bleed. This bleeding
can be significant and occasionally life threatening. Therefore, diagnosis and treatment guidelines have been
proposed to initially identify which individuals have kidney involvement in TSC and then, depending on the extent
(or size) of this involvement, propose either close surveillance or some form of intervention.
It is recommended that individuals with TSC should undergo an initial diagnostic radiologic evaluation with a CT
scan or ultrasound of the kidneys to identify those with kidney involvement. Then, depending on the size of the
involvement (less than 4 cm or greater than or equal to 4 cm), further management can be recommended.
Individuals with TSC and angiomyolipomas less than 4 cm would benefit from examination with either ultrasonography
or CT scanning every six months to one year. If the angiomyolipoma appears to grow or become a source of
symptoms, then some intervention should be entertained. This may include surgically removing the angiomyolipoma
or utilizing the radiologist to perform an embolization of the artery that goes to this growth. In individuals with
TSC and an angiomyolipoma greater than 4 cm, because of the high risk of further growth and the development of
symptoms, consideration should be given to treatment as previously described.
For those who do not want to have any form of intervention, they should be aware of the type of symptoms that are
associated with bleeding from the angiomyolipoma. This includes back or abdominal pain, nausea, vomiting and
fever. If these growths involve both kidneys, renal failure is a possibility; however, this is relatively uncommon. If
kidney function became so poor as to not sustain life, then dialysis or transplantation would be indicated.


This disease is genetic and I have been very lucky that I was never effected by it until now. I have always known I have had it but I guess was in denial because I was so healthy. Please keep me in your prayers.