What Is a Dermatofibrosarcoma Protuberans?
Sudden dermal fibrosarcoma (DSFP) is derived from fibroblasts or histiocytes and originates from the dermis, a slow-growing tumor. The origin of the tumor is unknown.
- English name
- dermatofi-brosarcoma protuberan
- Visiting department
- dermatology
- Multiple groups
- Middle-aged
- Common locations
- Torso and limbs
- Common symptoms
- Hard plaques on the trunk and limbs, skin color or dark red, etc.
Basic Information
Causes of bulging dermal fibrosarcoma
- The etiology and pathogenesis are unknown.
Clinical manifestations of cutaneous fibrosarcoma
- Patients are usually middle-aged. The tumor can occur in any part of the body, but it is more common in the trunk and limbs, more on the ventral side than on the dorsal side, more on the proximal side than on the distal side, and it is rare in the head, face, and neck. 10% to 20% of patients reported a history of trauma before onset. The course of the disease progresses slowly, starting with hard plaques, skin color or dark red, the skin is slightly concave and atrophic, and the skin around the tumor is bluish red, and later red, dark red or purple blue single nodules or different sizes Adjacent multiple nodules grow with a bulging appearance, ranging in size from 0.5 to 2 cm, and can suddenly accelerate growth with surface ulceration. A small number of tumors have a little pigmentation, which is called pigmented cutaneous fibrosarcoma or Bednar tumor. Pain is noticeable as the tumor grows. The disease is localized, occasionally widespread, but rarely metastatic.
Pathological manifestations of cutaneous fibrosarcoma
- Tumor cells and collagen fibers are often arranged in the shape of mats, wheels, braids, swirls or bundles. The above special cell arrangement methods are helpful for the differential diagnosis of DFSP, but they are not unique to DFSP and cannot be used as a basis for diagnosis. Giant cells, yellow tumor cells, foam cells, inflammatory cells, and hemorrhagic necrosis are occasionally seen in DFSP. At present, DFSP can be generally classified into general type, myxoid type, fibroid type, melanin type, giant cell fibroblastoma type, atrophic type, and mixed type. Because the tissue structure of DFSP is usually atypical and the morphological diversity is common in heavier or recurrent DFSP, the pathological diagnosis, especially early diagnosis, is more complicated and difficult.
Diagnosis of Carina cutaneous fibrosarcoma
- Histological characteristics and clinical data are the main basis, and immunohistochemical examination is helpful for differential diagnosis. In immunohistochemical staining, DFSP tumor cells showed a strong and diffuse positive response to vimentin; CD34 generally showed a strong and diffuse positive response, with a positive rate of 72% to 92%. Lysozyme showed a focal positive response; the expression of smooth muscle actin (SMA) in DFSP was 50% to 95%, but its expression was often unstable and often focal.
Carina cutaneous fibrosarcoma treatment
- Mohs microsurgical resection, the recurrence rate is 2%; the recurrence rate of patients with extensive resection can reach 11% to 50%. For surgical resection, the surgical margin should be 3 cm outside the tumor area and a deep fascial resection can reduce the recurrence rate.