What is the treatment of Dermatofibrosarcoma?
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: Surgery to remove the cancer.
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.
Does DFSP spread?
DFSP tumors occur in the dermis layer of the skin and can invade deeper subcutaneous tissue, such as fat, fascia, and muscle. They are slow growing, and spread laterally or sideways. They may increase in size more rapidly during pregnancy. If left untreated they can come through the skin, ulcerate and become painful.
Is DFSP malignant?
Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue neoplasm of intermediate- to low-grade malignancy. Metastasis rarely occurs. DFSP is a locally aggressive tumor with a high recurrence rate.
Is DFSP 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.
How is DFSP diagnosed?
If your dermatologist suspects you have DFSP, you will need a skin biopsy. This is the only way to diagnose skin cancer. Your dermatologist can safely perform a skin biopsy during an office visit. To perform a skin biopsy, your dermatologist will remove some of the tumor.
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.
Is DFSP hereditary?
The cause of dermatofibrosarcoma protuberans (DFSP) is unknown. Laboratory studies have shown that chromosomal aberrations may contribute to the pathogenesis of DFSP; however, no evidence of hereditary or familial predisposition exists. In 10-20% of patients with this tumor, trauma at the site seems to be incriminated.
Is DFSP life threatening?
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.
How are myxoid lesions differentiated from low-grade myxofibrosarcoma?
Predominantly myxoid lesions must be differentiated from low-grade myxofibrosarcoma (which is lobulated, shows significant nuclear atypia, and contains typical curvilinear vessels). 3. Myxoid neurofibroma Myxoid neurofibromas are usually solitary lesions, occuring in adults, on the extremities.
What are the myxoid lesions of nodular fasciitis?
Nodular fasciitis usually contains myxoid foci and approximately 5% of fasciitis are predominantly myxoid. Predominantly myxoid lesions must be differentiated from low-grade myxofibrosarcoma (which is lobulated, shows significant nuclear atypia, and contains typical curvilinear vessels). 3. Myxoid neurofibroma
How can I confirm the presence of myxoid lymph nodes?
Its presence can be confirmed by special stains such as alcian blue and only chondroid substance (sulfated GAG) will remain positive after treatment by hyaluronidase. Limited myxoid areas may be encountered in most soft tissue tumors and pseudotumors.
What are the diagnostic elements of benign myxoma?
Vascularity is another important diagnostic element. Most benign lesions, specially ” myxomas ” are poorly vascularized , whereas myxofibrosarcoma and myxoid liposarcoma are characterized by a prominent vascular tree. Nuclear atypia is often indicative of malignancy and allows the separation of myxofibrosarcoma from benign myxomas.