Tumorous defeats

Description:

Anevrizmalny cyst of a bone. J. Jaffe and L. Lichtenstein is allocated in an individual disease in 1942. A long discussion about the place of this disease among damages of a bone and the extensive literature devoted to various aspects of an anevrizmalnoa of a cyst allow to consider now that it possesses inherent only it clinicoradiological and morphological features which cornerstone local disturbance of blood circulation of a bone is.

Defeat of children, teenagers and persons of young age is most characteristic of an anevrizmalny cyst.

Ekzostoztny chondrodysplasia. At the heart of this disease, according to T. P. Vinogradova, disturbance of an endostosis lies. According to M. V. Volkov (1974), this disease makes 27% of all primary tumors and opukholepodobny displaziya of a skeleton at children, and according to Adler (1983), osteoarticular exostoses occur among benign tumors of bones in 40% of cases. The disease is well studied in clinical, radiological, morphological and genetic aspects. Fact in evidence of its hereditary transfer to 75% of all observations. There is a statement that for development of an ekzostozny chondrodysplasia ответствен a segment of a long limb 8.

The disease meets in two forms: multiple ekzostozny chondrodysplasia and solitary osteoarticular exostosis. Both solitary, and multiple osteoarticular exostoses can affect any bone. Favourite localization are metaphyses of long tubular bones. In 48% of all osteoarticular exostoses defeats of a distal metaphysis of a femur, proximal metaphyses of humeral and bolypebertsovy bones come to light. The disease usually comes to light at children's and teenage age, is the most frequent - in the second decade of life. Considerable dominance of males is noted.

Fibrous dysplasia. L. Lichtenstein is allocated in an independent nosological form from group of osteodystrophies in 1938. A. V. Rusakov, T. P. Vinogradova carry a fibrous dysplasia to diseases, boundary with tumors. M. V. Volkov holds the same opinion. The bone formation perversion at a connective tissue stage of a skeletogeny is the cornerstone of a disease. Frequency of a fibrous dysplasia among primary tumors and a dysplasia of a skeleton, according to M. V. Volkov, makes 2,5%.

The fibrous dysplasia - a disease of children's and youthful age, but is descriptions of observations when the disease was for the first time diagnosed for adults. On prevalence of pathological process distinguish poliossalny - a unilateral and bilateral fibrous dysplasia - and monoossalny.

Eosinophilic granuloma. In the histologic WHO classification the eosinophilic granuloma is regarded as not tumoral defeat of an unknown etiology which is characterized by intensive proliferation of reticulohistocytosis elements.

History of studying of this disease is connected with a name H. I. Tara-tynova which in 1913 described clinical observation under the name "pseudotuberculous granuloma".

As on not tumoral process and reference it to group of retikulogistiotsitoz most of researchers adhere to a view of an eosinophilic granuloma. M. V. Volkov (1974) allocates three forms of a disease: a solitary and multiple eosinophilic granuloma and a bone xanthomatosis, combining them in the general name "bone эозинофилез".

The eosinophilic granuloma - the most high-quality form also makes 50-60% of all forms of a Christian's disease. Children and teenagers suffer preferential. In rare instances the disease occurs at adults.

By data I. A. Kasymova (1994), a solitary form meets twice more often than multiple. Localization of an eosinophilic granuloma is various, but the backbone, a femur, pelvic bones are surprised more often.

Pathogeny:

Preferential localization of the pathological center are proximal departments of femoral, bolyiyebertsovy bones and pelvic bones. However the most frequent localization is the backbone. According to Dominok, Knoch (1971), anevrizmalny cysts affect a backbone in 23,4%, and in M. V, Volkov and A. P. Berezhny's observations this indicator is even higher - 60%.

Symptoms of tumorous diseases:

Pain and swelling are the main clinical symptoms of this disease. Pains, as a rule, happen non-constant, low-intensive, amplify at exercise stresses. With development of process of pain amplify, become constants. At damage of a backbone of pain have progressively amplifying character. Quite often the course of a disease is complicated by a compression of a spinal cord or its roots. The swelling, as a rule, corresponds to localization of a disease. Integuments often have the increased temperature, expanded venous vessels. The palpation of a swelling is painful, the crunch is sometimes noted.

Radiological excentricly located unstructured lytic center of destruction with sharp thinning of a cortical layer comes to light most often. Sometimes the cellular nature of structure of the center takes place. Many authors in the analysis of clinicoradiological displays of anevrizmalny cysts allocate process development phases:

The I phase - ossifluence;
II - an otgranicheniye;
III - recovery, emphasizing with that dynamics of a disease and compliance of clinical and radiological manifestations.
Duration of clinical symptoms is from 1 month up to 3 years.

Ekzostoztny chondrodysplasia
The clinical symptomatology depends on a form of a disease, localization, the sizes of exostoses, their form and relationship with surrounding bodies and fabrics.

At solitary defeats bone density, motionless in relation to a bone, various sizes and a form tumorous educations, as a rule, come to light; integuments over them are usually not changed. Osteoarticular exostoses of the big sizes can put pressure upon vessels or nervous trunks, causing pain. The arrangement of exostoses in a backbone with their growth towards the vertebral channel can cause a compression of a spinal cord.

At a multiple form of an ekzostozny chondrodysplasia quite often into the forefront such symptoms as it is undersized an awn, talipomanus, valgus deformation of knee joints act.

The sizes and form of an osteoarticular exostosis are various. The main signs are localization in metafizarny or in a metadiaphyseal zone, the osteoarticular exostosis can have the wide or narrow leg which is continuation of a cortical layer of the bone and marrowy cavity; the bulk of an exostosis represents bone structure, its outside surface can be equal or with awl-shaped outgrowths.

Growth of an exostosis continues usually in a growth period of bones, but sometimes increase in its sizes is noted also after closing of regions of growth.

One of terrible complications of a current of osteoarticular exostoses is their ozlokachestvleniye. According to S. T. Zatsepin, L.P.Kuzmina (1971), transformation of exostoses into a chondrosarcoma is noted at 12,5% of patients, according to Adler (1983), - about 20%. V. V. Balberkin (1994) notes that among 29 cases of the ozlokachest-curling osteoarticular exostoses in 25 observations the chondrosarcoma and in 4 - an osteosarcoma was diagnosed. More often the ozlokachestvleniye of exostoses takes place at patients with a multiple form of an ekzostozny chondrodysplasia (72%). The prevailing localization of an ozlokachestvleniye of exostoses are pelvic bones, the shovel, edges, a backbone is more rare.

In clinical practice for the purpose of the characteristic of exostoses and forecasting of their current several classifications are offered. S. I. Lipkin and A. V. Balberkin (1994) offered classification which basis data of a histologic research are.  

Usual exostoses with the balanced growth of the bone basis and cartilaginous covering. The majority of exostoses belongs to this type.
The exostoses beginning grow quickly after the end of growth of a skeleton. Their growth is caused still by an osteogenesis on the basis of a cartilage.
Exostoses with the hondromny growth of a cartilaginous covering. Bystry increase in weight exostoses happens at the expense of a cartilaginous covering.
Exostoses are ozlokachestvlenny.
Fibrous dysplasia

The clinical picture of a disease differs in big variety and steady progressing.

Deformations of bones of a skeleton, quite often pathological changes leading to sharp curvatures and shortenings of extremities, heavy functional disturbances. Most often bones of the lower extremities are subject to deformations. Deformation as a shepherd's stick is characteristic of femurs. Shins quite often have the acinaciform form in combination with a valgus curvature.

Eosinophilic granuloma

The clinical picture has no specific characters: patients are disturbed most often by pains, sometimes fervescence, restriction of movements, a hypotrophy of muscles.

The radiological symptomatology consists of existence of the centers of destruction of rounded or irregular shape, with quite accurate contours, sometimes the centers merge with each other. At localization in a backbone lytic destruction and a collapse of a body with formation of a flat vertebra - a typical X-ray pattern, however can be observed also the centers of destruction in a vertebra arch.

A. P. Berezhnaya, G. N. Berchenko, I. A. Kasymov on the basis of kliniko-X-ray morphological data allocate three phases in development of an eosinophilic granuloma: ossifluence, reparation and outcome.

Diagnosis:

The differential diagnosis is carried out with an osteoblastoclastoma, chondromyxoid fibroma and a telangiectatic osteosarcoma.

One of widespread types of treatment is puncture with introduction to a cavity of the center of steroid hormones.

The regional resection of tubular bones together with the pathological center and plastics of defect cortical allotransplants, as a rule, provides recovery. At localization of an anevrizmalny cyst in a backbone the resection of the struck department of a vertebra is considered the most rational.

Fibrous dysplasia
The X-ray pattern at a poliossalny form of a fibrous dysplasia is characterized by a sharp thickening of a bone, swelling and thinning of a cortical layer, sometimes disturbance of its continuity. The structure of a bone within the struck segment is, as a rule, not identical, sites of consolidation alternate with sites of depression. Ulotnenny sites have the greased appearance of "opaque glass".

At a monoossalny form the centers of destruction of a bone have the rounded or oval shape and are located in metadiaphyseal departments. The fibrous dysplasia as anomaly of development is sometimes combined with vneskletny frustration.

Example of similar defeat is Albright's syndrome. Multiple bone defeats and extra skeletal are characteristic of it (pathological skin pigmentation in the form of coffee spots, premature puberty).

M. V. Volkov, A. L. Arenberg allocate the mixed poliossalny fibrocartilage osteodysplasia. Sometimes perhaps ozlokachestvleny a fibrous dysplasia, both poliossalny, and mo-noossalny, with its transformation into an osteosarcoma.

Treatment of tumorous diseases:

Treatment of osteoarticular exostoses only surgical. Operative measures concerning uncomplicated exostoses - a regional resection of a bone together with the exostosis basis. Need for bone plastics is extremely rare.

Operative measures concerning secondary chondrosarcomas have to be adequate to the nature of a disease: at defeats of pelvic bones - resections of a basin or mezhpodvzdoshno-belly amputation; at localization in long tubular bones - an osteoarthrotomy with endoprosthesis replacement or plastics the allogenic joint end.

Fibrous dysplasia
Treatment of a fibrous dysplasia has to consist in surgical intervention.

At a monoossalny form the regional resection of a bone with removal of pathological fabric and plastics of defect cortical allotransplants is shown. At the expressed deformations of bones the regional resection with removal of pathological fabric and an alloplasty of defect has to be combined with corrective osteotomies with bone or nailing.

Eosinophilic granuloma
Treatment of an eosinophilic granuloma has no unambiguous decisions. Surgical intervention consists in a regional resection of a bone with defect plastics. Some authors apply radiation therapy, polychemotherapy.

By A. P. Berezhnaya and I. A. Kasymov (1994) at a solitary eosinophilic granuloma by method of the choice consider puncture treatment with intra focal introduction of corticosteroids. Damage of a backbone treat conservatively, an operative measure is shown at defeats with neurologic disturbances.