Where is dermatofibrosarcoma protuberans located?
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. As it grows, lumps of tissue (protuberans) may form near the surface of the skin.
Who classification of DFSP?
In the current World Health Organization classification, DFSP is defined as a superficial, low-grade, locally aggressive fibroblastic neoplasm (1,2).
What does DFSP look like?
DFSP most often starts as a small, firm patch of skin, approximately one to five centimetres in diameter. The skin is occasionally flat or depressed. It can be purplish, reddish or flesh-coloured. The tumor typically grows very slowly (over months to years) and can become a raised nodule.
What causes DFSP?
The cause of DFSP is unknown, but injury to the affected skin may be a predisposing factor. Treatment usually involves surgically removing the tumor. If the tumor is unable to be removed completely, additional therapy may be needed. Regular follow-up is important to monitor for recurrence.
How fast does DFSP grow?
The tumor typically grows very slowly (over months to years) and can become a raised nodule. DFSP tends to affect people between the age of 20 and 50, but it has been diagnosed in people of all ages.
Is DFSP graded?
Approximately 85 to 90 percent of DFSPs are low grade, while the remainder contain a high-grade sarcomatous component (which is usually a fibrosarcoma, designated DFSP-FS) and are considered to be intermediate-grade sarcomas [1].
Can DFSP be benign?
The limitation on developing imatinib clinically for the treatment of DFSP is that it is a benign to intermediate grade tumor in which complete surgical resection obviates the need for systemic therapy.
Is DFSP malignant?
Dermatofibrosarcoma protuberans (DFSP) is a rare dermal malignancy affecting the deep dermis and subcutaneous tissues. It was first reported by Darier and Ferrand in 1924. This tumor is locally aggressive with high recurrence rates, but rarely metastasizes.
How serious is DFSP?
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.
Can DFSP Spread To Brain?
Dermatofibrosarcoma protuberans (DFSP) is a low-grade tumor with rare metastasis. A 26-year-old male presented with multiple cutaneous nodular lesions of DFSP since 3 months along with distant metastasis to the brain, pleura, and muscles that were detected on investigations.
Can DFSP be cured?
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.
How do you prevent DFSP?
To reduce the risk of DFSP returning after surgery, your dermatologist may include a second treatment. The second treatment helps to kill cancer cells. Dermatologists also are studying new treatment options. One way to reduce DFSP from returning may be to treat patients with both excision and Mohs surgery.