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December 09, 2016 00:05

Dermatofibrosarcoma protuberans .Causes.Symptoms .Diagnostics.Treatment

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

causes and pathogenesis of dermatosis finally installed.It is believed that the disease occurs because vascular connective tissue elements.Some clinicians consider it as an intermediate form between dermatofibromas and fibrosarcoma.

Symptoms dermatofibrosarcoma protuberans.Clinically is characterized by the presence of (usually adult) single sklerodermopodobnyh knobby spines, initially flat, then, to varying degrees protruding above the skin, with a smooth surface and go lumpy telangiectasias.Growth in most cases slow, possibly ulceration, expressed a tendency to recur.The most frequent localization - the skin of the body, especially the chest, abdomen, and the shoulder girdle.Scalp, face, neck are rarely affected.Although the tumor is characterized by locally destruirujushchego growth, possible options with metastasis to regional lymph nodes and inte

rnal organs.

dermatofibrosarcoma protuberans usually arises from the rank of the husband at the age between 30 and 40 years, but can be in children.The tumor is often located in any part of the skin, but most often on the torso.At the beginning of the disease there is a dense fibrotic lesions (plaque) with a smooth or slightly uneven surface, brownish or lividnogo color.Gradually, the focus is increased by peripheral growth.Over the years, or even decades, there comes a stage of tumor disease.Formed one (rarely - plural) keloidopodobny tumor UzA reddish-bluish in color, with a smooth, shiny surface that is several centimeters in diameter, significantly above the level of the skin is penetrated and telangiectasias.At the beginning of the skin above the tumor thinned and tense swelling on the feel dense, mobile.Then, due to the formation of adhesions and infiltration of the tumor becomes fixed.Subjective feelings are usually absent, sometimes there is soreness.Over time, there is ulceration of the surface of the tumor, it is covered with serous-hemorrhagic crusts.Redidivirovanie often considered a characteristic feature of the tumor.After a long course of metastatic disease noted.

Histopathology.The tumor is composed of interlacing bundles of elongated cells monomorphic.There have been young proliferation of spindle cells, atypical mitosis.Depending on the degree of differentiation of the tumor may resemble a fibrosarcoma or dermatofibromu.

Pathology dermatofibrosakromy protuberans.Tumor cells are usually differentiated that resembles dermatofibromu, but the degree of differentsrovki in different parts of it differently.The presence of large atypical nuclei and mitoses to diagnose pathological dermatofibrosarkomu.In many areas there is the formation of collagen;fibroblasts are arranged in the form of beams extending in different directions, often in the form of rings.Giant cell is small, sometimes it does not.The tumor stroma places defined areas mucilaginized.Usually the tumor occupies the entire dermis and penetrate into the subcutaneous fat layer.Atrophic epidermis, sometimes with symptoms of tumor cell invasion and destruction.Differentiate from dermatofibrosarkomu dermatofibromas and atypical fibroksantomy.

Histogenesis dermatofibrosakromy protuberans.Based on electron microscopic examination of the tumor, most authors believe it fibroblast cells with an active collagen synthesis in the endoplasmic reticulum is well developed.tserebriformnye tumor cells have a nucleus, some of which are surrounded by a material resembling the basal membrane discontinuous.This pattern indicates that the tumor is derived from, or perineural endoneural elements.A.K.Apatenko (1977) examines how gnarled dermatofibrosarkomu malignant analogue angiofibroksantomy and believes that it develops from adventitial cells.

differential diagnosis.Differentiate from the disease need to dermatofibromas, fibrosarcoma, tumor forms of mycosis fungoides, gummy syphilis.Differentiating from the high-grade sarcomas (fibrosarcoma, leiomyosarcoma), as well as subcutaneous fibrosarcoma.Pay attention to the moiré patterns.strands arranged in the cell, and a relatively large amount kollatenovyh fibers.

treatment.Spend surgical excision of the tumor within the healthy tissue.