What is the prognosis of dermatofibrosarcoma protuberans?

What is the prognosis of dermatofibrosarcoma protuberans?

The overall prognosis of dermatofibrosarcoma protuberans is good, with a 10-year survival rate of 99.1%. As metastasis is rare, morbidity due to local recurrence is a more common issue. Age older than 50 is a risk factor for local recurrence.

How do you treat dermatofibrosarcoma protuberans?

Dermatofibrosarcoma protuberans treatment typically involves surgery to remove the cancer. Other treatments may be used to kill cancer cells that might remain after surgery….Treatment options may include:

  1. Surgery to remove the cancer.
  2. Mohs surgery.
  3. Radiation therapy.
  4. Targeted therapy.
  5. Clinical trials.

Does DFSP metastasize?

Dermatofibrosarcoma protuberans (DFSP) is a tumor that develops in skin or subcutaneous tissue and is characterized by a protuberant growth pattern [1]. DFSP often recurs locally after surgery, but rarely metastasizes to distant sites and is classified as a sarcoma of intermediate-grade malignancy [2–7].

Is dermatofibrosarcoma protuberans curable?

The general prognosis for DFSP is excellent. In the past, recurrence rates were high, but with the introduction of Mohs surgery, those rates have decreased. Even with recurrent DFSP, Mohs surgery has a 98% cure rate. A poor prognosis is associated with metastasis.

Is dermatofibrosarcoma protuberans fatal?

Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon intermediate-grade fibrohistiocytic sarcoma. It occurs most often in adults aged 20-50 years and is associated with local invasion and a high recurrence rate. Uncontrolled local disease or metastases may result in death.

How fast does dermatofibroma grow?

Usually, they develop over time, growing slowly until they reach less than 1 cm (about ¼ inch) in size. On rare occasions, larger lesions do occur, but dermatofibromas larger than 1 cm should be evaluated by a doctor.

How do you get dermatofibrosarcoma protuberans?

Dermatofibrosarcoma protuberans results from a new mutation that occurs in the body’s cells after conception and is found only in the tumor cells. This type of genetic change is called a somatic mutation and is generally not inherited.

What does dermatofibrosarcoma protuberans look like?

When dermatofibrosarcoma protuberans (DFSP) first appears on the skin, a person may notice: A pimple-like growth or rough patch of skin. No pain or tenderness where the growth or patch forms. Little change in the growth or patch.

How common is dermatofibrosarcoma protuberans?

Frequency. Dermatofibrosarcoma protuberans is estimated to occur in 1 in 100,000 to 1 in 1 million people per year.

Can dermatofibroma become cancerous?

However, if a person has a growth that looks like a dermatofibroma but is rapidly growing or changing, they should seek medical advice. This growth may be a sign of a rare type of cancer called dermatofibrosarcoma protuberans.

What is inside a dermatofibroma?

What are dermatofibromas? Dermatofibromas are small, rounded noncancerous growths on the skin. The skin has different layers, including the subcutaneous fat cells, dermis, and epidermis. When certain cells inside the second layer of skin (the dermis) overgrow, dermatofibromas can develop.

What does DFSP feel like?

What is dermatofibrosarcoma protuberans?

Dermatofibrosarcoma protuberans (DFSP) is a very rare type of skin cancer that begins in connective tissue cells in the middle layer of your skin (dermis). Dermatofibrosarcoma protuberans may at first appear as a bruise or scar.

How do you test for dermatofibrosarcoma protuberans?

Tests and procedures used to diagnose dermatofibrosarcoma protuberans include: Skin examination. Your doctor will carefully inspect your skin to understand your condition. Skin biopsy. Your doctor will remove a small amount of tissue for testing. Imaging tests.

How effective is adjuvant radiation therapy for follicular deep tissue sarcoma (DFSP)?

Multiple studies have shown that DFSP is a radio-responsive tumor and adjuvant radiation therapy is effective to control tumor growth and reduce the incidence of postoperative recurrence [102,103,104,105,106].

What is conservative re-excision in revision surgery for dermatofibrosarcoma protuberans?

98. Snow H., Davies E., Strauss D.C., Smith M., Hayes A.J. Conservative Re-excision is a Safe and Simple Alternative to Radical Resection in Revision Surgery for Dermatofibrosarcoma Protuberans. Ann. Surg. Oncol.