Monday, June 1, 2015

Chemotherapy/Radiation and Neurofibromatosis

In terms of Neurofibromatosis, chemotherapy and radiation treatments can be extremely useful for those pesky tumors that may be growing at a fast rate, growing in a location that may be deemed inoperable or used to treat tumors that are causing all kinds of unpleasant symptoms. The "theory" behind using chemotherapy for Neurofibromatosis is the same as it is for cancer... it destroys cells and prevents further growth of unwanted tumors or masses. The use of chemotherapy has been shown to be very effective in controlling the progression of optic nerve gliomas (which are usually benign) as well as malignant peripheral nerve sheath tumors. 

Now, lets talk about how these different therapies work! 

1. Chemotherapy

Chemotherapy is a medication that is most effective on cells that actively growing and dividing. Chemo drugs (which are called cytotoxic drugs) interrupt the cell cycle and slow down or sometimes completely stop rapidly growing cells ability to reproduce. Sometimes only one cytotoxic drug is needed to treat the tumor, but combining drugs will often lead to better and more long term outcomes. The goals of combining different kinds of chemo are: 

a) Killing as many cancer (or NF cells) as possible without harming the other healthy cells that are growing in the body 
b) Increases the chances of killing NF cells 
c) Decreases the chances that NF cells will mutate

Chemotherapy is most often given through an IV, which causes the medication to go directly into the bloodstream. This way, the drug can be carried around the entire body and can be delivered to body parts/systems that need it to fight tumor cells. The type of chemo drug that the physician decides to prescribe depends on factors such as: the person's age, the person's overall health, and the location of the tumor. These drugs may not kill all the tumor cells the first time they are given, and often chemotherapy cycles are continued for periods up to 6 months to ensure that the amount of "tumor death" is optimized!

2. Radiation Therapy 

 Radiation therapy is delivered by using highly specialized equipment, and works by damaging the genes in cells (which are responsible for controlling how fast cells grow and divide). Radiation damages genes by making small breaks in the DNA inside cells, which prevents these cells from growing and dividing. 

As previously mentioned chemotherapy usually exposes the entire body to the cancer drugs, however, radiation is a "local treatment", meaning it treats and affects only the parts of the body that need to be treated. The total dose of radiation given to a patient is typically divided into a number of smaller doses called factions, this is done in order to minimize damage to normal "healthy cells" while maximizing the death of NF cells. 

In order for a doctor to plan radiation treatment, a detailed imaging scan (usually a CT scan) must be obtained so that the tumor can be clearly outlined, and also to map out where healthy cells and tissues are located. During the treatment, it is VITAL that the patient is positioned in the exact same position in order to deliver the radiation to the same location each time. Apparatuses such as head molds, face masks, temporary skin tattoos and skin marks are all used to not only help position the patient, but to also keep them still during the procedure. 

I hope you've all learned something new today, comment below on what you would like to learn about in the future!




  1. We hear about chemotherapy or radiation all the time but I don't know much about either one of them. Now I know. Is NF one specific condition or is it part of a family of conditions.? Are there other things like NF?

    1. I guess you could say that NF encompasses 3 different conditions: Neurofibromatosis Type 1, Neurofibromatosis Type 2 and Schwannomatosis. Each condition is caused by a mutation in a different gene, but they all cause tumors to grow on the ends of nerves. Proteus syndrome is semi similar to NF. This condition causes an overgrowth of bones, tissues and skin. I'm sure you've heard NF referred to as the Elephant Man's Disease? The man who was the "Elephant Man" didn't actually have NF... he had Proteus Syndrome!

    2. I should have gone back to see your reply. Thanks for the info.

  2. So well written!!! Love the pictures too! Easy to understand....great education for everyone!