What is Dermatofibroma Protuberans?
Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. This condition is a type of soft tissue sarcoma , which are cancers that affect skin, fat, muscle, and similar tissues.
What does factor XIIIa positive mean?
The cells are located predominantly in the papillary dermis at the dermo-epidermal junction, also in association with skin appendages, but normally not within the epidermis. Elevated numbers of FXIIIa positive cells can be found in a host of cutaneous inflammatory conditions, including lichen planus and psoriasis.
How serious is dermatofibrosarcoma protuberans?
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.
What is factor XIIIa?
Factor XIIIa (FXIIIa) (fibrinoligase, plasma transglutaminase, fibrin stabilizing factor, E.C. 2.3. 2.13) is a glutaminyl-peptide γ-glutamyl transferase functioning in the final stages of the coagulation cascade, stabilizing the fibrin clot by crosslinking the α and γ chains of fibrin to form homopolymers (1-4).
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.
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.
What is cellular Dermatofibroma?
The cellular dermatofibroma is a morphologic variant of the ordinary dermatofibroma. It presents a higher frequency of subcutaneous extension and a higher rate of local recurrence (25%). Under these circunstances, the ideal is to perform a local excision of the lesion, with free surgical margin.
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.
Where is factor 13 found?
The factor XIII A chain gene is found on chromosome 6. Circulation of plasma factor XIII occurs in association with its substrate, fibrinogen. Thrombin cleavage of the Arg37 -Gly38 bond in the A chain, causing the release of an Mr 4500 activation peptide, is the key step in the activation of plasma factor XIII.
What is factor 13 disorder?
Summary. Listen. Factor XIII deficiency is an extremely rare inherited blood disorder characterized by abnormal blood clotting that may result in abnormal bleeding. Signs and symptoms occur as the result of a deficiency in the blood clotting factor 13, which is responsible for stabilizing the formation of a blood clot.
How is DFSP treated?
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.