From Big Medical Encyclopedia

FIBROMATOSES (fibromatosis, singular; lat. fibra fiber + - from + - osis) — the dysplastic processes in connecting fabric which do not have the signs of inflammatory reaction and features inherent in any certain tumor. T. meet at various age and can have different localization.

The concept «fibromatosis» was introduced in 1954 by Stout for the first time (And. P. Stout). The significant contribution to development of classification of fibromatoses was made Mackenzie (D. N of Mackenzie, 1970) and Allen (P. W. Allen, 1977).

Morphologically fibromatoses are characterized by the proliferation expressed in various degree (see) fibroblastichesky cells and infiltration of surrounding fabrics due to oppositional growth (see). At the same time unlike malignant tumors polymorphism of cells and their mitotic activity are less expressed.

T. it is accepted to divide on inborn (or juvenile) and mixed. The first meet hl. obr. at newborns, children of chest, preschool age and teenagers. Carry to them inborn fibrosarkomopodobny F., inborn generalized and multitsentrichny F., the inborn localized fibromatosis, a fibromatosis of a neck, a diffusion infantile fibromatosis, the fibrous gamartoma of babies recuring a fibrous tumor of fingers, juvenile aponeurotic fibroma, a juvenile angiofibroma of a nasopharynx, a multiple juvenile hyaline fibromatosis. Mixed F. can occur both at children, and at adults. Carry to them palmar and bottom F., abdominal and ekstraabdominalny desmoid (see), intraabdominal F. and hereditary gingivalny F.

The inborn fibrosarkomopodobny fibromatosis is allocated in 1977. Allene. The inborn tumors of various localization which are coming to light in the first 3 months of life are carried to it. Before them regarded as fibrosarcomas (see) in connection with a considerable morfol. looking alike this malignant tumor.

Inborn generalized and multitsentrichny F. are for the first time described in 1954 by Stout. Inborn generalized F. it is characterized by multiple nodes, to-rye are found more often at the birth of the child, localized in hypodermic cellulose, muscles, internals and bones. The number of nodes can reach fifty. They quickly increase in volume, new nodes appear. Skin over large nodes sometimes ulcerates. Wedge. the symptomatology is caused by defeat of these or those bodies. Most of children with inborn generalized F. dies soon after the birth. At inborn multitsentrichny F., unlike generalized, damage of internals is not observed that causes rather favorable a wedge, the course of process. However up to 5-year age new nodes can appear. Skin and hypodermic nodes, and also the centers of defeat in bones often (on average in 50% of cases) spontaneously regress. Inborn localized F. according to Allen (1977) proposal call cases of solitary defeats with single nodes, gistol. the picture to-rykh is similar that at inborn generalized F.

Fibromatoz of a neck — knotty education in grudino - clavicular sos-c-evidnoy to a muscle. It is microscopically formed by the connecting fabric poor in cellular elements, in a cut the set of the remained muscle fibers is defined (tsvetn. tab., Art. 384, fig. 1). In process of growth of a node, is more often after achievement by the child 3 — 4-year age, the wryneck can develop (see).

The diffusion infantile fibromatosis is allocated in 1965 to Entsingerom (F. M of Enzinger). Occurs preferential at boys of the first year of life. Process is localized generally in muscles of upper extremities, the heads and necks. Process does not meet in brushes and feet, does not happen multiple.

The fibrous gamartoma of babies is for the first time described in 1956 to R. D. Reye under the name «subepi-dermalny fibrous tumor of babies». It is found preferential in boys from the moment of the birth and up to 4 years (hl. obr. in the first 2 years of life). Defeat is localized in hypodermic cellulose of a forearm, shoulder, axillary and buttock areas, can be multiple. At microscopic examination idiosyncrasy — formation of islands from unripe cells of a mesenchymal look comes to light (tsvetn. tab., Art. 384, fig. 2).

The recurrent fibrous tumor of fingers is described to Reja in 1965. In 90% of cases this defeat arises on the first year of life, from them in 20% has inborn character. Cases of developing of a tumor at the age of St. 15 years are not described. More often fingers of brushes are surprised. Approximately at Ch3 of patients multiple defeats preferential on the same extremity are noted, at the same time the first fingers of brushes and feet are surprised extremely seldom. The tumor — the small knot which is a little towering over the surface of skin has, as a rule, several millimeters in the diameter, is located usually on the posterolateral surface of distal phalanxes of fingers. The tumor is located in a derma; characteristic morfol. feature of a tumor — existence in cytoplasm of cells of pironinofilny little bodies.

Juvenile aponeurotic fibroma is described for the first time in 1953 by L. E. Keasbey. It occurs preferential at children aged from 3 up to 15 years and is localized on brushes and feet, to a thicket is located in a fatty tissue, is connected with an aponeurosis or a fascia. For morfol. pictures are typical deposits of salts of calcium and focuses of cartilaginous transformation.

The juvenile angiofibroma of a nasopharynx is allocated in 1954 by S. S. Sternberg. Occurs preferential at persons of a men's yole aged from 10 up to 20 years. Has the expressed infiltrative growth, it is microscopically formed by connecting fabric and the vessels of cavernous type which are evenly located in it. Klien, symptomatology is connected with the sizes of the center of defeat and extent of its distribution on the neighboring areas.

Juvenile multiple hyaline F. E. Drescher et al. in 1967 is in detail described. Occurs at newborns and children of chest age. It is characterized by emergence of multiple nodes in hypodermic cellulose, especially pilar part of the head. Also various bones can be surprised, damage of joints leads to disturbance of their function. Macroscopically on skin papules of whitish color are found, to-rye during the progressing take a form of rough dense eminating nodes. Histologically process is characterized by existence of plentiful deposits of homogeneous amorphous acidophilic substance, among to-rykh small accumulations of spindle-shaped fibroblastichesky cells are scattered.

Palmar and bottom fibromatoses are described by G. Dyupyuitren (1832) and to Ledderkhoza (L. Ledder-hose, 1897). Are observed preferential at men aged 40 years are more senior, quite often can have heredo-familial character. The disease is expressed in emergence of one or several slowly growing nodes in the field of a palmar or bottom aponeurosis leading to development of a contracture (see Dyupyuitren a contracture; Fascia, pathology). Cases of simultaneous emergence palmar and bottom F are known., and also their combination with Peyroni's disease (see Induratio penis plastica).

From intraabdominal it is most known mezenterialny F., expressed in emergence of knotty fibroblastichesky growth in a mesentery of a small bowel. Can be an independent disease or be manifestation of a syndrome of Gardner (see Gardner a syndrome).

Hereditary gingivalny F. it is characterized tumorous utolshchbniyekhm gums and the accruing shaking and dedentition (see Desna, new growths). It can arise in the period of first dentition, and sometimes since the birth. More often is hereditary though can have sporadic character. It can sometimes be combined with a hypertrichosis (see), drink-rofibromatozom (see), dementia.

Diagnosis F. it is difficult and has to be under construction on the careful analysis a wedge, yielded and results gistol. researches of biopsy and operational material. Differential diagnosis, napr, at inborn generalized and multitsentrichny F. carry out with inborn neurofibers omatozy (see), multiple inborn geman-gioperitsitomy (see), multiple inborn leiomyomas (see), an inborn leiomyosarcoma (see) or a fibrosarcoma (see) with multiple metastasises.

Treatment of hl. obr. operational also consists in excision of a fibromatous node, sometimes with the subsequent radiation therapy (e.g., at a juvenile angiofibroma of a nasopharynx). At inborn generalized F. treatment is directed to reduction (where it is possible) mechanical impact of fibromatous nodes on adjacent fabrics that is especially important in the first 5 weeks after the birth since further process can regress.

The forecast more often favorable, however idiosyncrasy of fibromatoses is their tendency to a local recurrence after excision.

At a number of fibromatoses, napr, diffusion infantile, a recurrent fibrous tumor of fingers cases of spontaneous regression of process are observed.

Bibliography: In and x e r A. M. t, etc.

Tumorous formations and tumors of connecting fabric, page 60, M., 1977;

Pathoanatomical diagnosis of tumors of the person, under the editorship of N. A. Krayev-sky, etc., page 18. M, 1982; E N of c and N-g e r F. M. Histologic classification of tumors of soft tissues, the lane with English, M., 1974; Allen P. W. The fibromatoses. Amer. J. surg. Path., v. 1, p. 255, 1977; Enzinger F. M. Fibrous tumors of infancy, in book: Tumors of bone and soft tussue, p. 375, Chicago, 1965; Mackenzie D. H. The differential diagnosis of fibroblastic disorders, p. 67, 105, Oxford — Edinburgh, 1970; S h n i t-k and T. K., As p. D. M of a. H about r-n e r R. H. Congenital generalized fibromatosis, Cancer, v. 11, p. 627, 1958;

Simpson R. D., Harrison E. G. a. M and at about Page W. Mesenteric fibromatosis in familial polyposis, ibid., v. 17, p. 526, 1964; Sternberg S.

S. Pathology of juvenile nasopharyngeal angiofibroma — a lesion of adolscent males, ibid., v. 7, p. 15, 1954. V. M. Blinov.