My Experience with Dermatofibrosarcoma Protuberans (DFSP) Skin Cancer

Yes, the name of the cancer in the title above is a mouthful of a diagnosis to receive: Dermatofibrosarcoma Protuberans. I did a double take when my dermatologist carefully enunciated the syllables, because she, too, was having trouble - and I didn't know whether I should be crying or not. Any time you hear the word "cancer" it is terrifying. And I am experienced with cancer; many types - and some very rare - have affected family members. I am fair with blonde hair and blue eyes and lots of "beauty marks" (a prettier way of saying hundreds of irregular moles), so I always suspected that I might have to deal with basil cell carcinoma and needed to worry especially about melanoma. I am very careful with sunscreen. But, as it turns out, Dermatofibrosarcoma Protuberans (or DFSP) has nothing to do with the sun. Doctors don't know exactly what the cause may be, but there is the belief that DFSP tumors may occur where there was a prior injury.

I don't remember having any sort of injury. My tumor developed on the side of my face, on my cheek between the bottom of my ear and my jaw line. I first noticed a tiny bump in that area when I was in college, so that would have been ten years ago. I didn't pay any attention to it, and am actually surprised that I even remember when it appeared. Doctors have since told me that it was probably not cancerous at that point. I believe that within a period of the last three years it began to noticeably change, between my two daughters' births. And by the end of my second pregnancy and the first couple of months of my second daughter's life, it probably tripled in size and started to irritate me. Several doctors said, "Oh, don't worry about it. It is just a benign cyst. Get it taken off when you can." No one gave me the impression that it could be something malignant. My dermatologist actually told me that I could "live with it," but I wanted it removed because I knew it was growing rapidly. (And this is yet another example of how you should trust your own instincts!) By the time I had an initial surgery - with the doctor believing that she was removing a benign cyst - it was about the size of a large kidney bean and looked like a swollen mosquito bite. When we received the surprising biopsy results, it was evident that there were cancerous cells left on all margins of the tumor area.

Here are some facts about DFSP, mostly borrowed from an article by Chih-shan Jason Chen, MD, PhD entitled "Dermatofibrosarcoma Protuberans." I have found this article to be an informative and current description of DFSP, and it was also the one my dermatologist offered to me when she broke the news.

* Dermatofibrosarcoma Protuberans accounts for only 1% of all soft tissue sarcomas.

* Metastasis rarely occurs, but DFSP is a locally aggressive tumor with a high recurrence rate. The chance of metastasis is only 5%. Metastasis is more likely for those with recurrent tumors, and this is when the prognosis becomes poor.

* DFSP begins in the dermis (the second layer of our three layers of skin) and can invade fat, fascia (the dense tissue that covers muscle and bone), muscle, and bone. As is obvious in my case, it can grow very rapidly. Dermatofibrosarcoma Protuberans tumors invade tissue by sending out "roots" beneath the skin that makes complete removal difficult.

* These tumors are usually found in those between 20 and 50 years old. They most commonly occur on the trunk of the body, followed by extremities, and then the face and neck. (Lucky me!)

* There is no evidence of a genetic component contributing to the occurrence of Dermatofibrosarcoma Protuberans.

* The use of MRI may help determine how big the tumor is and how much area it has invaded. I had an MRI done of my head - and what a nerve-wracking experience that was - mainly to determine whether or not the cancer had invaded my parotid gland (salivary gland).

* Surgery is typically the method of choice for treatment of DFSP. Seldom is chemotherapy or radiation therapy used.

* Wide excision surgery had been used primarily to remove DFSP. However, more recently, Mohs micrographic surgery has been proven to have great results with Dermatofibrosarcoma Protuberans. This was the initial surgical treatment for my case; however, I had to have more extensive surgery as I describe later in this article.

* The recurrence rate is very high with the wide excision surgery (11-20%) and I believe that the scarring would be worse. With the Mohs procedure, however, more tissue can be saved and the recurrence rate shrinks to 0-6%.

* Recently, a leukemia drug called imatinib mesylate, or Gleevec, has been approved to treat Dermatofibrosarcoma Protuberans. It has had good success at shrinking or destroying these tumors.

* Most recurrences of DFSP occur three years following the initial surgical removal of the tumor. After five years with no recurrence of cancer, the prognosis is terrific.

When I was first diagnosed with DFSP, I avoided doing any internet research on this topic. I don't always believe what I read online (and, yes, there is irony in that I am writing this article) and the information that I came across scared me and seemed dated. A few pictures that I came across were troubling, to say the least.

Still, finding a simple list of the "facts" regarding this cancer would have been most helpful. I hope this article may be beneficial for anyone else who hears "dermatofibrosarcoma protuberans" and can't pronounce it, much less understand it.

These are the two pieces of advice that I have to offer:

1) Trust your own judgment. If something is changing in or on your skin, get it looked at right away. Chances are, it may be nothing, but you need to know for sure. Because the Dermatofibrosarcoma Protuberans occurred on my face, it was very noticeable and unattractive, so I went to the doctor as soon as I could after my daughter's birth. Don't wait too long; if I had waited, I am not sure what would have happened to my face and all that is below my skin (and as of now I am going to have some significant scars and some tolerable disfigurement of the shape of my face).

2) If you receive this diagnosis, talk to several doctors. Know that the Mohs procedure is considered the treatment of choice today. As I mentioned above, however, I have had more extensive treatment.

This is my specific experience, but I believe everyone diagnosed with DFSP has a different story:

I traveled to Johns Hopkins Hospital where a dermatologist was confident that she could remove all of the DFSP tumor with MOHs surgery. A later MRI of my head - which was recommended by the head and neck surgeon and reconstructive surgeon also working with me - showed that the tumor may have spread to my parotid gland.

We went on with the Mohs procedure as planned, and the tumor was bigger than anticipated. The large wound was left open for two weeks in order to continue pathology on the tissue. I had reconstructive surgery scheduled at the end of that two weeks if all the testing came back negative for cancerous cells. However, the night before my surgery the treatment plans changed. It is still not clear why the doctors waited until the last minute to drastically change the therapeutic strategy, other than our belief that there was some discordance between them. The testing of the tissue made it clear that all of the Dermatofibrosarcoma Protuberans tumor was gone (of course, this was something to celebrate!) but that there were still atypical cells in the fascia layer surrounding my parotid gland. This was troubling. The reconstructive surgeon and head and neck surgeon had seen this type of aggressive cancer repeatedly recur and emphasized that I was too young to leave any sort of potentially bad cells in my body. Additionally, the reconstructive surgery involved pulling skin up from my neck and moving a whole lot of tissue around, which would mean also relocating any of those atypical cells. So, I opted to have a "superficial parotidectomy," which meant I had the top half of my salivary gland removed. Unfortunately, I had to wait an additional two weeks with the open wound to get an appointment for this particular surgery. It was a tricky surgery because there is a main facial nerve that runs through the middle of the gland and controls the motor functioning of our faces.

Jump forward to five weeks post-op, and I am thrilled to be cancer-free. This hasn't been an easy road to say the least; it has taken about six months out of my life, and I am left with some nerve damage in my face that should resolve over time and some scars that should fade over time, as well. I will always remember this trying experience when I look in the mirror, but I guess I have gotten old enough to realize that scars are a part of life, whether on the inside or outside, and if I live my life like they are simply a part of me then everyone else around me won't be so concerned with them, either. I never wanted to be a supermodel, anyway.