Swelling dermatofibrosarcoma of skin and soft tissues: photo, symptoms, treatment, prognosis

Some skin diseases can be cancerous - dangerous for human life. For example, dermatofibrosarcoma swelling, although it is characterized by slow growth and in most cases does not give metastases, often has a tendency to relapse and aggressive growth deep into the skin.

To treat this disease should be at an early stage surgically.

What is bulging dermatofibrosarcoma?

Disease swelling dermatofibrosarcoma is a rare oncopathology, is a tumor of connective tissue localized on the skin of a person. Synonyms - progressive dermatofibrosarcoma, Darje-Ferran tumor.

The degree of malignancy is average, as the neoplasm tends to increase in size over the years and decades. Metastases are rarely diagnosed, in the overwhelming majority of cases, the tumor affects only surrounding tissues.

The photo shows the swelling dermatofibrosarcoma of the skin

The classification of education includes a simple and pigmented form. Histological examination of the tumor also reveals two types of its structure:

  • Similar to dermatofibroma
  • The fibrosarcoma

reminds statistical data on this pathology: it is rare, accounting for up to 0.01% in oncology, and among tumoral diseases of the skin - about 5%.More often observed in men, among races - in black people.

Typical age of skin lesions is 20-50 years( young age), in children education is observed in 10% of all clinical cases.

Reasons for the appearance of

The etiology remains unclear. Some authors point out that the disease belongs to collagenases, when collagen production fails.

For the enhancement of collagen production, fibroblast cells respond to the formation of the tumor. Other researchers note that the tumor originates from vascular anomalies that lie in the dermis.

Presumably, there are a number of risk factors that can cause the development of this pathology:

  • Injury of a specific part of the body
  • Burning of the skin with fire, chemical reagents
  • Contact with poisonous substances
  • Congenital gene mutations that occur in zone 17-22 of chromosomes
  • Transmission of "broken"genes by inheritance
  • Excessive frequency of X-ray examinations
  • Repeating operations

Symptoms of pathology

Localization area of dermatofibrosarcoma can be diverse. Most often it is on the body.

In most people dermatofibrosarcoma was found on the shoulders and chest, a little less often - on the legs and back. Even less often the neoplasm is diagnosed on the head, abdomen, in the groin, isolated cases are localization on the face, neck, nose, genitals, buttocks.

In the clinical course of the disease, two stages are distinguished:

  1. The first, involving the formation of a skin lesion zone. The tumor does not bulge above the surface, but grows in diameter. The skin gradually acquires a characteristic tuberosity, the color turns dark, from beige to brownish.
  2. Second, tumor. After a few months or years there is a bulging, inherent in this type of neoplasm.

As for the appearance of the disease, it can be quite unobtrusive for quite a long time. Therefore, a person seeks help only in the second stage, when the tumor begins to rise above the skin.

Neoplasm at the very beginning of development clearly stands out against the background of healthy tissues, without penetrating into them, not differing in invasive growth. But then it cools with the skin, with the fat layer.

At this stage, we can note the tension of the skin, its atrophy( thinning), the appearance of "vascular asterisks" around the tumor. In the absence of treatment, after 5-10 years, ulcers often appear on the tumor.

Visually signs of dermatofibrosarcoma at a late stage of development are:

  1. Color - red, bluish, purple.
  2. Surface - lacquered, glossy, smooth.
  3. Size - 2-5 or more centimeters.
  4. Mobility in relation to the skin - tightly welded, immovable.
  5. Consistency - dense, firm.
  6. Features of appearance - a large number of enlarged, convoluted vessels, ulcers, erosion, serous and blood crusts.
  7. Sensations of the person - occasionally soreness in a zone of a tumor.

The course of the disease is relatively benign, since the growth is slow. Only in very rare situations there was an active progression of the tumor. Unfortunately, almost 50% of tumors give relapses.

This development of the disease can lead to metastasis in regional lymph nodes, bones, muscles, fascia, brain, peritoneal and thoracic organs. Metastasis is observed in terms of 15-25 years from the beginning of the skin tumor.

Diagnostics of

Oncologists can make a preliminary diagnosis based on the data of the examination and the collection of anamnesis( clarifying the nature of the progression and the duration of the pathology).

But an accurate diagnosis is possible only after performing a biopsy of the tumor.

Since the main method of treatment is surgical, often the tumor is completely removed, and then sent for analysis. Methods of fine needle aspiration, open biopsy are also used, followed by a histological examination of the biopsy specimen.

To exclude the presence of tumor metastases, a number of instrumental examinations are mandatory:

  • lymph node ultrasound
  • CT, MRI of the abdominal cavity
  • Radiography, bone scintigraphy near the affected area

Chest radiograph

Differential diagnosis is made with fibrosarcoma, onycomicosis, certain forms of syphilis, dermatofibroma,leiomyosarcoma, neurofibroma, certain forms of melanoma.

Treatment of dermatofibrosarcoma

If possible, all neoplasms of this type are removed by surgery. The tumor from 3 cm of skin along its entire perimeter is necessarily cut out, which will allow to avoid relapse. Also, the adjacent fascia is excised and a method of intraoperative histological examination of the edges of the wound is used.

When a tumor is not more than 2 cm, perform an operation of micrographic surgery for Mohsa. If the defect was large, then further operations are performed from the field of plastic surgery.

After surgery, short courses of radiation therapy are sometimes recommended. Chemotherapy and radiotherapy can not completely eliminate the foci of tumors, therefore they are used only to enhance the effect of the operation, or in the presence of metastases with the inability to remove them( palliative therapy).

Sometimes the location of the neoplasm is such that it is not possible to make the operation possible, and in this case only radiation therapy is used.

Prognosis and prevention of

Prognosis of dermatofibrosarcoma is relatively favorable, but in different cases depends on the duration of the pathology, the number of relapses.

With distant metastases, the forecast is unfavorable, usually patients die within 1.5-2 years, but the risk of such an outcome does not exceed 6-8%.

To prevent illness, injuries, burns and wounds should be avoided, and all dermatological diseases should be treated at the earliest stage of their appearance.

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