Periungual and subungual fibromas are commonly known as Koenen tumors. These tumors are benign as they are tuberous sclerosis manifestations. They are extremely painful and disfiguring. Most doctors find it challenging to treat this condition.
Currently, there is no standard treatment that has long-lasting effects. It is considered a genetic condition that is quite rare. Usually, it is present at the time of birth but is not symptomatic. These non-cancerous tumors can affect several parts of the body that include skin, eyes, brain, heart, kidneys, and lungs.
Koenen tumors can also lead to a variety of health-related issues that can range from mild to severe. These problems include:
- Learning disabilities
- Epilepsy
- Hyperactivity syndrome or autism
- Skin abnormalities like red acne patches light in color and rough skin
- Hydrocephalus
- Breathing difficulties
- Kidneys may not function properly
Koenen Tumor Symptoms
The symptoms of a koenen tumor can be broad range. Moreover, it also depends and varies among the severity of the patient case. If the case is mild, the patient presents with few symptoms but the severe cases have more than one symptom. This largely affects the quality of life.
The diagnosis of a koenen tumor is a bit daunting. There are several clinical investigational tests performed to confirm the prognosis. The series of tests include a CT scan of the head, MRI of the brain, ECG, EKG, Examination of the eye, kidney ultrasound, and your skin is examined under the wood’s lamp.
Now let’s get back to the symptoms and have a look at what kind of symptoms confirm that a patient is suffering from the koenen tumor.
- Developmental skills delay is the first sign seen with the patients suffering from koenen tumor
- Seizures are also a vital symptom to consider before diagnosing the patientIntellectual disabilities
- Heart rhythm abnormality
- Tumors of the brain that are not cancerous
- Calcium deposits on the brain
- Red patches
- Decreased pigment on the skin
- Skin texture is like an orange peel
- Fingernails and toenails have growth underneath
Koenen Tumor Causes
The TSC1 and TSC2 gene mutations cause a koenen tumor. Normal cell growth is regulated with these genes. The uncontrolled gene mutation leads to the abnormal growth of uncontrolled cells. This is one of the main reasons which can lead to the formation of multiple tumors in the body.
This genetic fault has no specific reason in a few cases. It is usually seen in 3 out of 4 cases around the world. The genetic abnormality is not present in any family member of the patient, still, the patient suffers from the benign tumor. In the majority of cases, parents pass this faulty gene to their children in heredity.
The faulty gene can be passed from one parent only. Tuberous sclerosis is also diagnosed in the parent who carries the abnormal gene. As discussed earlier, sometimes it is asymptomatic and the patient doesn’t feel anything. The diagnosis of the koenen tumor also involves genetic testing.
Although the mutation of these genes is still a mystery and there is no definite treatment for this. Several clinical studies have stated that early diagnosis can help to manage the symptoms.
Koenen Tumor Treatment
There is no universal treatment of koenen tumors. Each patient is treated according to the symptoms they experience. In short, the treatment plan for each patient is different. The tailored treatment plan is according to the symptoms of the patients. Regular exams and monitoring are equally important. Kidney ultrasounds are vital to ensure that there is no dysfunctionality.
The most commonly experienced symptom is seizures. The quality of life is greatly affected by seizures. Anti-seizure medications can help to reduce the episodes. Brain surgery can be another option if the patient experiences multiple seizures.
Behavioral therapy, special educational programs, medications, and occupational therapy are some of the treatment options which can be considered while treating developmental delay and mental disability.
Laser treatment can be used to remove the skin tags and small growths which are resulted due to the koenen tumor.
The function of vital organs can be restored by choosing the surgical procedure. It is not recommended for everyone. It depends on the condition of the patients. Moreover, parents must observe the behavior of children, any abnormality noticed should be treated immediately. Serious complications can also lead to premature death. Good medical care can lead to a better quality of life. The symptoms should be monitored with regular medical checkups.