Dermatofibrosarcoma bulging: causes, symptoms, diagnosis, treatment

Dermatofibrosarcoma protuberans (synonym: dermatofibrosarcoma progressive and recurrent, Darier-Ferrand tumor) is a malignant connective tissue tumor.

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

Symptoms of dermatofibrosarcoma protuberans. Clinically characterized by the presence (usually in adults) of single scleroderma-like nodules, initially flat, then protruding above the skin to varying degrees, with a smooth or bumpy surface and telangiectasias. Growth is slow in most cases, ulceration is possible, there is a tendency to recurrence. The most common localization is the skin of the trunk, especially the chest, abdomen and shoulder girdle. The scalp, face and neck are rarely affected. Although the tumor is characterized by locally destructive growth, variants with metastasis to regional lymph nodes and internal organs are possible.

Dermatofibrosarcoma protuberans usually occurs in men between the ages of 30 and 40, but can also occur in children. The tumor is often located on any part of the skin, but most often on the trunk. At the onset of the disease, a dense fibrous lesion (plaque) appears, with a smooth or slightly bumpy surface, brownish or livid in color. Gradually, the lesion increases due to peripheral growth. After years or even decades, the tumor stage of the disease occurs. One (rarely multiple) keloid-like tumor-like lesion of a reddish-bluish color is formed, with a smooth, shiny surface, which reaches several centimeters in diameter, significantly protrudes above the skin level and is penetrated by telangiectasias. At first, the skin above the tumor is thinned and tense, the tumor is dense to the touch, mobile. Then, due to infiltration and the formation of adhesions, the tumor becomes immobile. Subjective sensations are usually absent, sometimes painfulness is noted. Over time, ulceration of the tumor surface appears, it is covered with serous-hemorrhagic crusts. Frequent redivision is considered a characteristic sign of the tumor. After a long course, metastasis of the disease is noted.

Histopathology. The tumor consists of intertwined bundles of monomorphic elongated cells. Proliferation of young spindle-shaped cells and atypical mitoses are noted. Depending on the degree of differentiation, the tumor may resemble either fibrosarcoma or dermatofibroma.

Pathomorphology of dermatofibrosacromas protuberans. Tumor cells are usually differentiated, which resembles dermatofibroma, but the degree of differentiation in different areas is not the same. The presence of large atypical nuclei and pathological mitoses allows diagnosing dermatofibrosarcoma. Collagen formation is observed in many areas; fibroblasts are located in the form of bundles going in different directions, often in the form of rings. Giant cells are few, sometimes they are absent. In some places, mucus areas are determined in the tumor stroma. Usually, the tumor occupies the entire dermis and penetrates the subcutaneous fat layer. The epidermis is atrophic, sometimes with phenomena of invasion by tumor cells and destruction. Dermatofibrosarcoma is differentiated from dermatofibroma and atypical fibroxanthoma.

Histogenesis of dermatofibrosacromas protuberans. Based on electron microscopic examination of this tumor, most authors consider its cells to be fibroblasts with active collagen synthesis in a well-developed endoplasmic reticulum. The tumor cells have cerebriform nuclei, some of which are surrounded by material resembling discontinuous basement membranes. This picture indicates that the tumor originates from perineural or endoneural elements. A.K. Apatenko (1977) considers nodular dermatofibrosarcoma to be a malignant analogue of angiofibroxanthoma and believes that it develops from adventitial cells.

Differential diagnosis. It is necessary to differentiate the disease from dermatofibroma, fibrosarcoma, tumor forms of mycosis fungoides, gummatous syphilis. Differentiating from high-grade sarcoma (fibrosarcoma, leiomyosarcoma), as well as from subcutaneous fibrosarcoma. Pay attention to moire structures located in cellular cords, and a relatively large number of collaten fibers.

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