Dermatofibrosarcoma bulging: causes, symptoms, diagnosis, treatment

Dermatofibrosarcoma swelling (synonym: dermatofibrosarcoma progressive and relapsing, Darje-Ferran tumor) - malignant connective tissue tumor.

The causes and pathogenesis of dermatosis have not been fully established. It is believed that the disease occurs from the vascular elements of connective tissue. Some clinicians see it as an intermediate form between dermatofibroma and fibrosarcoma.

Symptoms of dermatofibrosarcoma bulging. Clinically, it is characterized by the presence (usually in adults) of single scleroderm-like nodularis, initially flat, then to varying degrees protruding above the skin, with a smooth or tuberous surface and telangiectasias. Growth in most cases is slow, possibly ulceration, a tendency to recur. The most frequent localization is the skin of the trunk, especially the chest, abdomen and shoulder girdle. The hairy part of the head, face, and neck are rarely affected. Although the tumor is characterized by local-destructive growth, variants are possible with metastasis in the regional lymph nodes and internal organs.

Dermatofibrosarcoma bulging arises usually in men between the ages of 30 and 40 years, but it can also be in children. The tumor is often located in any part of the skin, but more often on the trunk. At the beginning of the disease, a dense fibrous focus (plaque) appears, with a smooth or slightly bumpy surface, a brownish or livid color. Gradually, the focus increases due to peripheral growth. After years or even decades comes the tumor stage of the disease. One (rarely - multiple) keloidlike tumor-shaped reddish-cyanotic color, with a smooth, shiny surface that reaches a few centimeters in diameter, significantly protrudes above the skin level and is permeated with telangiectasias. In the beginning, the skin over the tumor is thinned and tense, the tumor is dense to the touch, mobile. Then, due to infiltration and formation of adhesions, the tumor becomes immobile. Subjective sensations, as a rule, are absent, sometimes there is soreness. Over time, ulceration of the surface of the tumor, it is covered with serous-hemorrhagic crusts. Frequent redidivirovanie is considered a characteristic sign of the tumor. After a prolonged course, there is metastasis of the disease.

Histopathology. The tumor consists of intertwining bundles of monomorphic elongated cells. There are proliferations of young spindle-shaped cells, atypical mitoses. Depending on the degree of differentiation, the tumor can resemble either fibrosarcoma or dermatofibroma.

Pathomorphology of dermatofibrosacroma bulging. Tumor cells are usually differentiated, which resembles dermatofibroma, but the degree of differentiation in different parts of it is not the same. The presence of large atypical nuclei and pathological mitoses allows diagnosing dermatofibrosarcoma. In many areas, the formation of collagen; fibroblasts are arranged in the form of bundles going in different directions, often in the form of rings. Giant cells are few, sometimes they are absent. In the stroma of the tumor, places of mucus are locally identified. Usually the tumor occupies the entire dermis and penetrates into the subcutaneous fat layer. The epidermis is atrophic, sometimes with the phenomena of invasion by tumor cells and destruction. Differentiate dermatofibrosarcoma from dermatofibroma and atypical fibroxanthoma.

Histogenesis of dermatofibrosacroma bulging. Based on the electron microscopic study of this tumor, most authors consider her cells to be fibroblasts with active collagen synthesis in a well-developed endoplasmic reticulum. The tumor cells have cerebriform nuclei, some of them are surrounded by a material reminiscent of interrupted basal membranes. This picture indicates that the tumor originates from perineural or endoneural elements. AKApatenko (1977) considers knotty dermatofibrosarcoma as a malignant analog of angiofibroxanthoma and believes that it develops from adventitial cells.

Differential diagnosis. Differentiate the disease from dermatofibroma, fibrosarcoma, tumor forms of fungal mycosis, gummy syphilis. Differentiating from sarcoma of high degree of malignancy (fibrosarcoma, leiomyosarcoma), as well as from subcutaneous fibrosarcoma. Pay attention to the moire structure. Located in the cellular strands, and a relatively large amount of collagen fibers.

Treatment. Surgical excision of the tumor is performed within a healthy tissue.