Dermatofibrosarcoma protuberans (DFSP)
Dermatofibrosarcoma protuberans (DFSP) Is a type of skin cancer that develops in cells of connective tissue in the dermis of the skin (middle layer). It is a very rare cancer that usually affects the skin only.
Initially, you may observe dermatofibrosarcoma protuberans appearing as a scar or a bruise. As the tumor grows, you develop lumps of tissue called protuberans near the surface of your skin.
Usually, cancer develops on certain organs of the body, like the legs, arms, and trunk. However, dermatofibrosarcoma protuberans very rarely spreads beyond the skin and grows very slowly.
It is not exactly clear what leads to dermatofibrosarcoma protuberans. However, researchers already know that the tumor results because of mutations that occur in the cells of the body.
These mutations occur after the birth of an individual and present only in tumor cells. Such mutations are called somatic mutations. Additionally, you do not inherit these mutations.
You may not notice your skin cancer for months or even years as it grows very slowly. However, when a dermatofibrosarcoma protuberans (DFSP) appears on your skin, you may notice the symptoms like:
A rough patch or a pimple-like outgrowth on your skin.
The patch or growth that results shows no tenderness or pain.
Your patch shows no or little change.
However, when cancer grows further inside the middle layer of your skin, you have other signs. The tumor will now push on the top layer of your skin. A lump, called protuberan, may develop, which is rubbery or hard.
After that, the tumor stretches your skin and now you show symptoms like:
The skin becoming tender
Bleeding and cracks results
The lump hardens in the skin and becomes cement-like.
In a pregnant woman, dermatofibrosarcoma protuberans grows more quickly. More lumps may appear with time. Once the lumps appear, the tumor tends to grow faster. They can be violet or reddish-brown in adults. However, in young ones, it tends to be red or blue.
There are several ways by which your doctor may diagnose dermatofibrosarcoma protuberans which include:
Skin examination. To understand your condition in a better way, your doctor may carefully observe your skin. He/she will look for skin changes and other patches.
Skin biopsy. In this procedure, your doctor will take a small amount of tissue from your skin. He/she will look for the changes in the cells that cancerous cells show. This is a very promising method to detect dermatofibrosarcoma protuberans.
Imaging tests. In many cases, your doctor may order various imaging tests to diagnose your dermatofibrosarcoma protuberans. These tests such as an MRI give them more details about the tumor.
Moreover, these tests are particularly helpful in getting the cancers of soft tissues like dermatofibrosarcoma protuberans. These also help in the better planning of the tumor.
The treatment for dermatofibrosarcoma typically involves surgery to remove the entire tumor. Your doctor may also use other tests after surgery to kill the cancerous cells.
Treatment options include:
Surgery. For several tumors, the treatment options include surgery. Thus, your doctor may recommend surgery wherein he/she removes the tumor along with some healthy tissue around it. This way your doctor makes sure whether the cancerous cells are completely removed during surgery.
Mohs surgery. This is a special form of surgery wherein your doctor removes thin layers of cancer-containing skin progressively. He/she will do it until only tissue with no cancer cells remains. After the removal of each skin layer, your doctor will examine it under a microscope for signs of a tumor.
For treating larger tumors, Mohs surgery may be helpful. This is because the dermatofibrosarcoma protuberans grow in an irregular shape which can be difficult to remove completely.
Radiation therapy. This procedure uses powerful energy beams such as X-rays and protons to kill the tumor cells. In case your doctor is not able to remove the tumor surgically, he/she may recommend radiation therapy.
Targeted therapy. This method uses drugs that help to kill the tumor cells. The drugs focus on some specific traits of a tumor that make them vulnerable to death.
For instance, individuals with dermatofibrosarcoma protuberans may have cancerous cells that show the presence of a protein in excess. Thus, a drug called imatinib (Gleevec) helps target and kill these cells. Moreover, your doctor may recommend this procedure only if the tumor shows recurrence.
An option can be clinical trials to test new forms of treatment. Thus, whether you can participate in a clinical trial or not, you should ask your doctor.
Coping and support
When you get diagnosed with dermatofibrosarcoma protuberans, it could be frightening for you. The condition will be no different for your family.
However, you may learn to cope with the uncertainty and distress related to cancer with time. Thus, you need to follow these instructions until then;
Call on for medical support:
Various things are going to help you. For instance, the knowledge and understanding of a medical social worker, or any other mental health professional is necessary. They will help you in understanding your cancer.
Furthermore, if your child or other family member suffers from cancer, you need to ask health care professionals for advice. They will provide you with options for medical health support. This will also provide you with emotional and social support.
In addition to this, you can check various online services that will provide you support to combat cancer.
- Gain more knowledge about dermatofibrosarcoma protuberans to make decisionsabout control and care:
Ask your doctor about various treatment options related to dermatofibrosarcoma protuberans. Little knowledge is dangerous. Therefore, more confidence in understanding and making decisions about treatment options will be there with you. So you should always learn more about the disease.
Ask the health care team for guidance if your child has cancer. Therefore, get more and more information for appropriately caring for the patient.
Be close to your friends and family:
A close and strong relationship with your family and friends is necessary. It will help you deal with dermatofibrosarcoma protuberans.
You need practical support, moral support from your friends and relatives. Thus, someone should be there for you to look after the family. Emotional support from them is going to matter most. Thus, a healthy and happy person will ultimately efficiently fight the disease.
Prepare for your appointment
You are likely to start making an appointment with your primary care doctor if some signs and symptoms worry you. Ask for a referral to an experienced specialist if your doctor suspects dermatofibrosarcoma protuberans.
A team of specialists typically can treat. For instance;
Tumor surgeons who have specialization in operating soft tissue cancers.
Doctors who have a specialization in treating cancers with systemic medications or chemotherapy.
Pathologists diagnose the specific type of cancer by analyzing a tissue.
Rehabilitation specialists who after surgery help in the recovery of a tumor.
What you should expect from your doctor:
You will face several questions from your doctor. Thus, you should be ready to answer these. So, give more time to your doctor to address them. Your doctor may ask;
What signs and symptoms concern you more?
Have your symptoms been occasional or continuous?
When did you start to notice the symptoms?
The severity of your symptoms?
Is there anything that improves your symptoms?
Is there anything that worsens your symptoms?
Do you have any family or personal history of cancer?