From Big Medical Encyclopedia

DERMATOFIBROSARCOMA EMINATING (dermatofibrosarcoma protuberans; Greek derma, dermat[os] + fibrosarcoma; synonym: the progressing and recurrent deratofibroma, dermatofibrosarcoma to Darya — Ferrana, a hilly fibrosarcoma of skin) — malignant mesenchymal tumor of skin. P. U other in 1897 is for the first time mentioned. In an independent form the progressing and recurrent deratofibroma, or a dermatofibrosarcoma is allocated in 1924 to Zh. Darya and Ferran (M. of Ferrand) under the name. Afterwards began to be called a dermatofibrosarcoma to Darya — Ferrana. The term «dermatofibrosarcoma eminating» was offered by E. Goffmann in 1925. From domestic dermatologists the histopathology of this disease was studied by V. O. Lushitsky.

D.'s histogenesis of century is not clear. There is an opinion on its vascular origin and communication with (E. Goffmann) sclerosing an angioma, however there are proofs its fibroblastichesky genesis.

Macroscopically the tumor has an appearance of a dense node of violet or bluish-brown color, fibrous structure, to dia. 7 — 8 cm. The microscopic structure is similar to well differentiated fibrosarcoma (see). Tumor cells of a spindle-shaped form, without the expressed signs of atypia, form bunches which create so-called moire structures. Usually normal formation of collagen is found that confirms the fibroblastichesky nature of a tumor. In certain sites of a tumor the mucous degeneration of collagen can be observed. Figures of mitoses are rare. Sometimes it is difficult for D. to distinguish century from deratofibromas (see). D.'s diagnosis of century is spoken well by infiltriruyushchy growth.

The tumor meets seldom, equally affects persons men's and female. D.'s development by century begins with emergence of the plaques consisting of 2 — 3 small knots covered with not changed skin. Gradually small knots become more and they increase in sizes. Nodes are painful and very dense, are not soldered to the subject fabrics, have quite clear boundary. Integuments are, as a rule, not changed, however at long existence nodes can be eroded and bleed. D. preferential in skin of a chest and abdominal wall, usually single is located with century. The etiology is unknown. Growth of a tumor slow, metastasises are rare.

Treatment — operational. The forecast is favorable though a local recurrence even after radical excision is possible.

Bibliography: Lushitsky V. O. Clinic and histopathology of a dermatofibrosarcoma to Darya, Vestn, veins. and dermas., No. 11, page 22, 1939; Olkhovskaya I. G. and Rabena. S. O to the kliniko-morphological characteristic of a dermatofibrosarcoma to Darya — Ferrana, Klien, medical, t. 54, No. 6, page 75, 1976; Olkhovskaya I. G., Probatova N. A. and Kazantseva I. A. About an eminating dermatofibrosarcoma, Arkh. patol., t. 38, No. 12, page 17, 1976; Darier J. et Ferrand M. Dermato-fibromes progressifs et recidivants ou fibrosarcomes de la peau, Ann. Derm. Syph. (Paris), ser. 6, t. 5, p. 545, 1924; Petkov I. u. Andreev Y. G. Dermatofibrosarcoma protuberans in seltener Lokalisation, Hautarzt, S. 508, 1972, Bibliogr.; Srivastava Y. K. a. Bhar-gava K. S. Dermatofibrosarcoma protuberans, Indian J. Cancer, v. 9, p. 257, 1972, bibliogr.

A. A. Klimenkov.