Monday, January 12, 2015

Different Types of Fibromas

Hey there everyone! Thank you to everyone for your kind words after I posted my last blog past last week on eating disorders and body image, it truly means a lot to me! This week I am going to share information on different types of fibromas commonly seen in patients with NF. There are 3 types of fibromas:

1. Cutaneous Neurofibromas
2. Plexiform Neurofibromas
3. Spinal Neurofibromas

Alright then, lets start with cutaneous fibromas (which is pictured below)

Cutaneous neurofibromas occur when the covering of a nerve below the skin begins to form a slow-growing tumor. This occurs, because people with Neurofibromatosis are unable to make a protein called neurofibromin, which is responsible for "monitoring" the body for tumor growth... kind of like how cops monitor for crimes... However, people with Neurofibromatosis do not make this specific protein, which is why tumors can rapidly grow in the body.

Cutaneous neurofibromas begin to develop right around puberty, it is very uncommon that these types of tumors to develop in infancy or early childhood. Typically, as an individual gets older there is an increase in the number of cutaneous fibromas...this is especially true of women who are pregnant (there is speculation that tumor growth in NF is aggravated by hormones and hormonal changes). These types of tumors hardly ever cause problems, and are extremely unlikely to ever become cancerous. The most commonly reported "problems" of cutaneous fibromas are: an intense itching sensation, pain when touched or bumped and cosmetic deformities.

In terms of treatment for cutaneous neurofibromas, the size and location of the tumor depends on which course of treatment a doctor will take, Most commonly the tumors will be removed during surgery, but radiation and chemotherapy are also methods to remove tumors if they can't be accessed by surgery. Also, sometimes doctors can prescribe creams that will help to stop the itching!

Next we will discuss plexiform neurofibromas!

Plexiform neurofibromas are present in about 30% of individuals living with NF, and unlike cutnaneous fibromas these tumors are often found in early infancy and sometimes even at birth. Plexiform fibromas grow similarly to that of a cutaneous fibromas, except it involves tumors growing on multiple nerves instead of a tumor growing on just one nerve. Usually these types of tumors grow around the eye socket, face, arm, leg, back, chest or abdomen. Typically these tumors grow at a very slow rate, and are unlikely to become cancerous. When these tumors do become cancerous however (in 5-10% of plexiform neurofibromas) they are called "malignant peripheral nerve sheath tumors"

The main complications that plexiform neurofibromas cause are: pain, loss of function and cosmetic deformity. Another symptom that commonly occurs in these types of tumors is called "Hyperpigmentation Overlying a Plexiform Neurofibroma".... sounds super complicated eh? All it really means is that it is common for a tumor to become an orange-like colour, grow more hair than normal and develop an uneven surface (like the picture above!)

Treatment options for plexiform neurofibromas is very similar to the treatment of cutaneous neurofibromas (surgery, radiation or chemo). With this type of tumor, it is very common that parts of the tumor will grow back after the procedure if they entire nerve is not taken out... so surgery does not pose a 100% cure!

Lastly, we are going to discuss spinal neurofibromas! The  yellow arrow in the MRI is pointing to the spinal cord, and the red arrow is pointing to the spinal neurofibroma. Signs and symptoms of spinal neurofibromas depend on where the tumor is located. Sometimes these tumors produce no symptoms, and sometimes they can cause complications such as weakness in the arms and legs, back pain and numbness. Typically these tumors grow slowly, and rarely become cancerous. Treatment usually includes surgical excision.

Thanks for reading, I hope you all learned something!


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