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The dermatofibrosarcoma is eminating

Dermatofibrosarcoma eminating — a connective tissue tumor with destruiruyushchy growth, inclined to recurrence.

Epidemiology

The dermatofibrosarcoma eminating usually arises in 3 — the 4th decade of life, is slightly more often at men.

Classification

In addition to the main option, allocate a pigmental form of an eminating dermatofibrosarcoma.

Etiology and pathogeny

Most of authors consider as the main cells of a tumor fibroblasts with active synthesis of collagen in well developed cytoplasmic reticulum. Cells have tserebriformny kernels, and the part of them are surrounded with material, reminding the interrupted basal membranes that it does not exclude their genesis from perineural or endoneural elements.

Clinical signs and symptoms

The prevailing localization — skin of a trunk and hips. The pilar part of the head, a face, a neck are surprised seldom.
It is clinically characterized by existence of single sklerodermopodobny nodosity, flat in the beginning, then in various degree of speakers over skin level, with a smooth or hilly surface and teleangiectasias. Growth in most cases slow, the ulceration is possible, tendency to recuring is expressed (to 30 — 50%). Though of a tumor it is characteristic local destruiruyushchy growth, options with innidiation in regional lymph nodes and internals are possible.

The diagnosis and the recommended clinical trials

Are necessary for statement of the diagnosis a X-ray analysis of lungs, ultrasonography of a liver, a biopsy.

The tumor cells which are usually differentiated that reminds a deratofibroma, but differentiation degree in various sites unequal. Existence of large atypical kernels and pathological mitoses allows to diagnose a dermatofibrosarcoma. In many sites collagen forming is observed; fibroblasts are located in the form of the bunches going in various directions is frequent in the form of rings. It is not enough colossal cells, sometimes they are absent. In a tumor stroma sliming sites are defined by places. Usually the tumor occupies all derma and gets into a hypodermic fatty layer. Epidermis is atrofichny, sometimes with the invasion phenomena cells of a tumor and destruction.

Differential diagnosis

Fibrosarcoma, deratofibroma, diffusion form of a neurofibroma, leiomyosarcoma, fusocellular melanoma.

General principles of treatment

Radical surgical excision of a tumor is necessary.

Forecast

Forecast of an eminating dermatofibrosarcoma rather favorable.
 
"Fungoid mycosis   High-quality fibrous histiocytoma"