KSS. Osteoarticular system. Benign tumors from the bone tissue itself. Osteoblastoclastoma (differential diagnosis)

Differential diagnosis of cancer upper jaw with ameloblastoma(adamantinoma) is caused by the fact that when it is localized in the alveolar process, there is a thickening of this part of the maxillary bone. When ameloblastoma appears, the first clinical sign

There is also swelling of the affected area of ​​the alveolar process of the upper jaw. From this moment, as N. M. Mikhelson and

L. O. Varshavsky (1955), there is a need for differentiation malignant tumor from ameloblastoma.

The difference between cancer of this location and ameloblastoma is expressed in rapid growth cancer, pain in the teeth and their looseness. Ameloblastoma grows extremely slowly and sometimes takes years to reach large sizes. There are no pain symptoms with ameloblastoma. Loose teeth are observed extremely rarely, usually after tumor suppuration. When ameloblastoma is large, its bone wall becomes thinner and bends under finger pressure, which is accompanied by the appearance of a “parchment crunch.” There is no involvement of the lymph nodes. The X-ray picture of ameloblastoma is also significantly different from that of a cancerous lesion, as already mentioned earlier.

The need for differential diagnosis of alveolar cancer with osteoblastoclastoma is caused by the same general characteristics, i.e. the presence of deformation of the alveolar process.

The difference between alveolar cancer and osteoblastoclastoma is that, in addition to those already mentioned distinctive features with ameloblastoma, which are also characteristic of osteoblastoclastoma, localized in the alveolar process, it usually occurs in children and individuals young and much less often in older people.

At the same time, the long course of the disease, the absence of nasal discharge, pain symptom distinguishes osteoblastoclastoma from cancer. Important differential diagnosis includes x-ray examination.

The final diagnosis is established based on the data

pathomorphological examination.

Differential diagnosis of cancer of the upper jaw with odontogenic cysts carried out due to the presence of them common feature- deformation of the alveolar process, which, as these formations grow and fill with them, maxillary sinus may manifest itself as protrusion of the anterior wall of the sinus and the corresponding lateral wall of the nose.

The difference between a cancerous tumor of the alveolar process and odontogenic cysts is that a cancerous tumor does not cause significant thickening of this part of the upper jaw, causes pain in the teeth, and quite quickly leads to the destruction of bone tissue and loose teeth with subsequent formation of ulcers. When spreading towards the orbit

the tumor causes exophthalmos and blurred vision. There is nasal discharge mixed with ichor.

Odontogenic cysts are manifested by a more distinct swelling of the alveolar process, which, with further development of the cyst, spreads to the anterior wall of the maxillary sinus and protrudes it. But even with a significant size, the cyst, as a rule, does not spread towards the orbit, does not cause exophthalmos and does not lead to visual impairment.

In addition, with an odontogenic cyst, there is no pain in the teeth, their looseness, or bloody discharge from the nose. The mucous membrane of the alveolar process has normal color. A test puncture through the anterior wall of the maxillary sinus in the area of ​​the transitional fold of the vestibule of the oral cavity with an odontogenic cyst allows one to obtain a viscous amber-colored liquid. If there is a cancerous tumor, the punctate will contain either blood or no contents.

On radiographs, both with a cyst and with cancer, shading of the maxillary sinus is noted, but with a cyst it will have clear boundaries and connection with the tooth - the presence of a tooth root facing the cyst (with a radicular cyst) or a crown (with follicular cyst). With cancer, the entire maxillary sinus is obscured, and destruction of its walls is revealed.

Differential diagnosis of cancer of the upper jaw from osteoma is carried out because the latter is often found on the upper jaw, localized on its anterior surface and on zygomatic bone. Less commonly, it is located in the maxillary sinus. With the development of osteoma as a result of thickening of the affected part of the upper jaw, its deformation occurs.

Located in the maxillary sinus, with further growth towards the orbit it can cause displacement eyeball outward and impair vision; if the nose grows towards the side, it can lead to protrusion outer wall, and with growth towards the palatine processes - to protrusion hard palate. Symptoms mentioned also found in cancer

upper jaw.

The difference between cancer of the upper jaw and osteoma is the faster growth of the tumor, the absence of clear boundaries, the appearance of nasal discharge mixed with blood, and loose teeth. Osteoma grows extremely slowly.

Located at a wide base on the surface of the bone, it is clearly demarcated from the surrounding bone tissue. Bloody-purulent discharge is not observed with it. No loose teeth are noted. Even with a significant size of osteoma, the mucous membrane of the upper jaw does not change, does not grow with a tumor and does not ulcerate.

An x-ray of osteoma reveals newly formed bone tissue that has a homogeneous structure with clear contours.

The need for differential diagnosis of cancer of the upper jaw with fibroma sometimes occurs due to the presence of such general symptoms as deformation of the upper jaw, impaired nasal breathing, copious discharge from the nose.

The difference between cancer of the upper jaw and fibromatous growths of the nasal cavity and maxillary sinus is that with cancer of the upper jaw, spontaneous pain and loosening of the teeth are usually observed, and nasal discharge is purulent in nature. X-ray reveals

intense shading of the maxillary sinus without clear boundaries and destruction of bone tissue. With fibromatous growths in the nasal cavity and in the maxillary sinus, pain and loose teeth are not observed. Discharge from the nose is mucopurulent in nature without admixture of blood. The x-ray shows a smooth, clearly defined border of the tumor, separating it from the walls of the maxillary sinus.

In difficult cases, the question of the nature of the tumor is decided on the basis of biopsy data.

Differential diagnosis of cancer of the upper jaw with chronic inflammation of the maxillary sinus is determined by the following general symptoms: duration of the disease, presence in the upper jaw aching pain, difficulty in nasal breathing, purulent discharge from the nose.

The difference between cancer of the upper jaw and chronic inflammation of the maxillary sinus is that with cancer there is deformation of the upper jaw as a result of protrusion of the anterior wall of the maxillary sinus, loose teeth, and discharge of pus from the nose mixed with ichor.

At chronic inflammation In the maxillary sinus there is no deformation of the upper jaw, there is no looseness of the teeth, pus is released in greater quantities than with cancer and without the admixture of ichor. A test puncture indicates the presence of pus in the sinus. The x-ray shows no signs of destruction of the bone walls of the maxillary sinus, which is usually observed with cancer.

The need for differential diagnosis of hard palate cancer with syphilitic gumma determined by the presence of a number of similar characteristics. Thus, in the infiltrative form of cancer emanating from the mucous membrane of the hard palate, the neoplasm initially appears as a painless infiltrate, covered with normal mucous membrane. With gummous lesions of the hard palate, a limited painless infiltrate of bluish-red color is also initially noted. Subsequently, both with cancer and with tertiary syphilis, the infiltrates disintegrate with the formation of ulcers. Moreover, the edges of the ulcers in both cases are dense.

The difference between a cancerous ulcer of the hard palate and a syphilitic gumma is that a cancerous ulcer, as already indicated, has crater-shaped, torn edges, covered with granular, easily bleeding tissue. It quickly spreads along the length, capturing the soft palate and alveolar process, causing loosening of the teeth.

A gummous ulcer has a regular round or oval shape, surrounded by a ridge of bluish-red infiltrate. Its bottom is covered with a yellow-gray coating. Probing reveals necrotic bone, which is subsequently sequestered to form a perforation opening connecting the oral cavity with the nasal cavity.

A gummous ulcer does not tend to spread along its length and does not cause loosening of the teeth. There is usually no nasal discharge. The x-ray shows signs of thickening and compaction of bone tissue due to periosteal layers.

Osteoblastoclastoma(osteoblastoclastoma, giant cell tumor, osteoclastoma, gigantoma).

The term "osteoblastoclastoma" was given to wide use in the Soviet Union over the past 15 years. First detailed description this tumor belongs to Nelaton (1860). Over the years, its teaching has undergone significant changes. In the second half of the 19th century, osteoblastoclastoma (giant cell tumor) was included in the group of fibrous osteodystrophies. In the works of S. A. Reinberg (1964), I. A. Lagunova (1962), S. A. Pokrovsky (1954), giant cell tumor is considered as local fibrous osteodystrophy. V. R. Braitsov (1959) expressed his view of a “giant cell tumor” of bones as a process of embryonic disorder of bone development, which, however, was not further confirmed. Currently, most researchers do not doubt the tumor nature of this process (A.V. Rusakov, 1959; A.M. Vakhurkina, 1962; T.P. Vinogradova, Bloodgood).

Osteoblastoclastoma is one of the most frequent tumors bones. There are no noticeable sex differences in the incidence of osteoblastoclastoma. Cases of familial and hereditary disease have been described.

The age range of patients with osteoblastoclastomas ranges from 1 year to 70 years. According to our data, 58% of cases of osteoblastoclastoma occur in the second and third decades of life.

Symptoms of Osteoblastoclastoma

Osteoblastoclastoma is usually a solitary tumor. Its double localization is rarely observed and mainly in adjacent bones. Long tubular bones are most often affected (74.2%), less often - flat and small bones.

In long tubular bones, the tumor is localized in the epimetaphyseal region (in children - in the metaphysis). It does not grow into articular cartilage and epiphyseal cartilage. IN in rare cases diaphyseal localization of osteoblastoclastoma is observed (according to our data, in 0.2% of cases).

Clinical manifestations of osteoblastoclastoma largely depend on the location of the tumor. The first sign is pain in the affected area, bone deformation develops, and pathological fractures are possible.

Benign osteoblastoclastoma can become malignant.

Causes of malignancy A benign tumor has not been precisely determined, but there is reason to believe that trauma and pregnancy contribute to this. We observed cases of osteoblastoclast malignancy of long bones after multiple series of external beam radiation therapy.

Signs of malignancy of osteoblastoclastoma: rapid growth of the tumor, increasing pain, an increase in the diameter of the focus of destruction or the transition of the cellular-trabecular phase to the lytic phase, destruction of the cortical layer over a large area, unclear contours of the focus of destruction, destruction of the endplate that previously limited the entrance to the medullary canal, periosteal reaction.

The conclusion about the malignancy of osteoblastoclastoma based on clinical and radiological data must be confirmed morphological study tumors.

In addition to malignancy, the benign form of osteoblastoclastoma can also be primary malignant osteoblastoclastomas, which, essentially (T.P. Vinogradova) are a type of sarcoma of osteogenic origin.

The localization of malignant osteoblastoclastomas is the same as that of benign tumors. An X-ray examination reveals a focus of bone tissue destruction without clear contours. The cortical layer is destroyed over a large area, the tumor often grows into soft fabrics. There are a number of features that distinguish malignant osteoblastoclastoma from osteogenic osteoclastic sarcoma: older age of patients, less pronounced clinical picture and more favorable long-term outcomes.

Diagnosis of Osteoblastoclastoma

X-ray picture of osteoblastoclastoma of long bones.
The affected bone segment appears asymmetrically swollen. The cortical layer is unevenly thinned, often wavy, and can be destroyed over a long area. At the break point cortical layer It can be disaggregated or sharpened in the form of a “sharpened pencil”, which in some cases imitates the “periosteal visor” in osteogenic sarcoma. The tumor, destroying the cortical layer, can extend beyond the bone in the form of a soft tissue shadow.

There are cellular-trabecular and lytic phases of osteoblastoclastoma. In the first case, foci of bone tissue destruction are identified, as if separated by partitions. The lytic phase is characterized by the presence of a focus of continuous destruction. The focus of destruction is located asymmetrically with respect to the central axis of the bone, but as it increases, it can occupy the entire diameter of the bone. Characteristic is a clear delineation of the focus of destruction from the intact bone. The medullary canal is separated from the tumor by the endplate.

Diagnosis of osteoblastoclastoma long tubular bones sometimes seems difficult. The greatest difficulties arise in the radiological differential diagnosis of osteoblastoclastoma with osteogenic sarcoma, bone cyst and aneurysmal cyst.

Clinical and radiological indicators such as the patient’s age, medical history, and localization of the lesion become important in differential diagnosis.

An aneurysmal cyst in long bones, unlike osteoblastoclastoma, is localized in the diaphysis or metaphysis. With an eccentric location of an aneurysmal bone cyst, local swelling of the bone, thinning of the cortical layer, and sometimes the location of the bone crossbars perpendicular to the length of the cyst are determined. An aneurysmal bone cyst, unlike osteoblastoclastoma, in these cases is predominantly elongated along the length of the bone and may have calcareous inclusions (A. E. Rubasheva, 1961). With a central aneurysmal cyst, there is a symmetrical swelling of the metaphysis or diaphysis, which is not typical for osteoblastoclastoma.

The monoosseous form of fibrous osteodysplasia of the long tubular bone may be mistaken for osteoblastoclastoma. However, fibrous osteodysplasia manifests itself, as a rule, in the first or early second decade of a child’s life (M.V. Volkov, L.I. Samoilova, 1966; Furst, Schapiro, 1964). Bone deformation manifests itself in the form of its curvature, shortening, less often lengthening, but not pronounced swelling, which occurs with osteoblastoclastoma. For fibrous osteodysplasia pathological process, as a rule, is localized in the metaphyses and diaphysis of tubular bones. There may be thickening of the cortical layer (compensatory), the presence of zones of sclerosis around the foci of destruction, which is not typical for osteoblastoclastoma. In addition, with fibrous osteodysplasia, there is no pronounced pain symptom inherent in osteoblastoclastoma, a rapid progression of the process with a growth inclination towards the joint, or a breakthrough of the cortical layer with the tumor emerging into the soft tissue.

Of the flat bones, changes are most often observed pelvic bones and shoulder blades. The lower jaw is affected in approximately 10% of cases. Solitary and isolated lesions are also characteristic. Bone swelling, thinning, waviness or destruction of the cortical layer and a clear delineation of the pathologically altered area of ​​​​the bone are determined. During the lytic phase, destruction of the cortical layer prevails, during the cellular-trabecular phase - thinning and waviness of the latter.

The greatest differential difficulties arise when osteoblastoclastoma is localized in lower jaw. In these cases, osteoblastoclastoma is very similar to adamantinoma, odontoma, bone fibroma and true follicular cyst.

Treatment of Osteoblastoclastoma

Treatment of benign osteoblastoclastomas carried out by two methods - surgical and radiation. Great importance in the assessment of the treatment carried out belong to x-ray examination, which makes it possible to establish anatomical and morphological changes in the affected part of the skeleton during therapy and in the long term after it. In these cases, in addition to multiaxial radiography, direct magnification radiography and tomography may be recommended. Certain structural features of osteoblastoclastoma are known at various times after external beam radiation therapy. On average, after 3-4 months, with a favorable course of the process, trabecular shadows appear in place of previously structureless areas of the tumor; gradually the trabeculae become more dense. The lesion acquires a fine-meshed or coarse-meshed structure. The thinned or destroyed cortical layer is restored; tumor size may decrease. The formation of a sclerotic shaft between the tumor and the unchanged part of the bone is noted. The duration of reparative bone formation varies from 2-3 months to 7-8 or more months. In cases of the development of the “paradoxical reaction” phenomenon, first described by Herendeen (1924), 2-8 weeks after radiation therapy, pain in the affected area increases, foci of destruction increase, trabeculae resolve, and the cortical layer becomes thinner. The paradoxical reaction subsides after approximately 3 months. However, a paradoxical reaction may not be observed during radiation therapy for osteoblastoclasts.

An important criterion for the effectiveness of osteoblastoclast therapy is the severity of remineralization of the former lesion. Relative concentration minerals at various times after radiation and surgical methods osteoblast treatment is determined by the method of relative symmetric photometry of radiographs.

Differential diagnosis

Central osteoblastoclastomas are difficult to differentiate from solid adamantine and sarcomas. Only pathohistological examination can reject the diagnosis of solid adamantinoma.

When a solid adamantinoma turns into a cystic one, then it can be relatively easily differentiated according to radiography (polycystic pattern) and puncture (cholesterol crystals). The difference from sarcoma is the significant duration of development of osteoblastoclastoma (3-5-10 or more years) and root resorption.

The experience of our clinic staff shows that a radioindication diagnostic method using radioactive phosphorus (P32) can play a certain supporting role in differentiation from sarcoma. When performing a biopsy, it is necessary to take into account that osteoblastoclastoma tissue is heterogeneous in its structure and therefore must be examined at different depths.

Treatment and outcomes

In accordance with the literature data (A. A. Kolesov, 1964, etc.) and the experience of our clinic (O. V. Babashev, 1966), we can recommend surgical intervention as the main method of treatment for osteoblastoclastoma. Excochleation of small cellular or cystic forms of tumors must be supplemented by curettage of the adjacent bone.

For small lytic tumors, as well as for extensive cellular and cystic osteoblastoclastomas, we recommend particularly careful excochleation with curettage to healthy bone or resection of the lower jaw.

"Fundamentals of surgical dentistry",
Yu.I. Bernadsky

This type of tumor is classified as a primary tumor. osteogenic origin.

There are both malignant and benign forms. There are many synonyms for this neoplasm: brown tumor, OBC, brown tumor, local fibrous osteitis, giant cell osteodystrophy, giant cell fibroma, gigantoma and others.

Clinical forms

There are two clinical forms of OBC: lytic and cellular-trabecular. The latter, in turn, is divided into two types: active-cystic and passive-cystic. Lytic the form is characterized by rapid growth and large destruction of lytic bone. Active cystic is a growing, spreading tumor without clear boundaries, with signs of the formation of new cells at the border of healthy and tumor tissue. Passive cystic the form has clear boundaries with healthy tissue, surrounded by a band of osteosclerosis and without a tendency to spread.

Gigantoma under the influence of stressful situations, especially during times of hormonal changes (the beginning of regular menstruation, pregnancy, etc.) can become malignant.

This type of tumor is more common in children and adults under the age of 30. Affects the epiphyses and metaphyses. The favorite localization in children is the proximal metaphysis of the humerus; in adults, the epimetaphysis of the bones forming knee-joint. Males are more often affected.

Clinic

The diagnosis of a neoplasm presents certain difficulties, especially in the early stages of development of the tumor process. The course of the disease at this time is asymptomatic. The exception is the lytic form of the tumor. The first sign of this form of the disease is pain, swelling, increased local temperature, deformation of the affected segment, and dilation of the saphenous veins. All these signs appear 3-4 months after the onset of pain. It should be noted that deformation of the segment occurs quite quickly due to the rapid growth of the tumor. With significant thinning of the cortical layer, pain becomes constant both at rest and with movement, and intensifies with palpation. With large destruction of the articular surface, joint contractures occur.

In cystic forms of OCD, the course is asymptomatic. The first manifestation of the disease is most often a pathological fracture or deformation of a segment in the absence of pain even with palpation of the tumor. Fractures with osteoblastoclastoma, as a rule, heal well, but after this active tumor does not stop growing, and may even increase. If the tumor is located in areas with a “deficiency of integumentary tissues” with significant thinning of the cortical bone layer, then palpation can reveal the symptom of bone crunching (crepitus), which occurs due to damage to the thinned cortical plate by the fingertips.

Almost all patients note the presence of trauma to the affected limb, which occurred several months ago, before the tumor was diagnosed. It should be noted that after the injury there was a “bright period” of asymptomatic disease for several months. Some authors try to connect the fact of injury with the cause of the tumor. Most orthopedists do not adhere to this point of view.

Diagnostics

On an x-ray, osteoblastoclastoma looks like an endosteal lesion of clearing in the bone, thinning the cortical layer and, as it were, swelling the bone from the inside. The bone around the tumor is not changed, its pattern corresponds to this location. Only in the passive-cystic form of the neoplasm is a “corolla” of osteosclerosis characteristic. The structure of the lesion depends on the shape of the tumor: in the lytic form it is more or less homogeneous, and in the cystic form it is cellular-trabecular and resembles “ bubble", swelling the bone from the inside. In lytic forms, the epiphyseal cartilage is affected by the tumor, and it grows into the epiphysis; the articular cartilage is never damaged by the tumor. Despite the preservation of the epiphysis, these forms of tumor, due to their close approach to the growth zone and disruption of its nutrition, subsequently cause significant shortening in the growth of the limbs.

Macroscopically, foci of the lytic form of OBC have the appearance of brown blood clots filling the entire tumor space. When the periosteum is destroyed, it becomes gray-brown in color, and the tumor penetrates the soft tissues, growing into them. In active cystic forms, a denser surrounding cortical layer is observed. The contents of the tumor are located among complete and incomplete bone septa and consist of a more liquid jelly-like mass, reminiscent of blood clots, but it contains a lot serous cysts. In the passive-cystic form, the lesion consists of serous fluid enclosed in a dense bone box or fibrous membrane. As with the active cystic form, cells and trabeculae may persist.

Treatment

First place in treatment osteoblastoclast is given away operative method. In lytic forms - extensive, segmental resection of the bone with removal of the periosteum, and sometimes part of the soft tissue. In cystic forms, the tumor is removed subperiosteally. The attitude towards the growth zone of the epiphyseal cartilage should be careful. After tumor removal it is necessary bone grafting(auto- or allo-). For pathological fractures, it is better to operate after a month, waiting for the formation of the primary callus.

The prognosis, even with benign forms, must be determined very carefully. This is due to the possible occurrence of tumor recurrence, its malignancy, the development of limb shortening in children after surgery, the formation false joint and resorption of the allograft.

Osteodysplasia is pathological condition one or many bones, caused by a congenital disorder of their development. Osteodysplasia has several forms, depending on the phase in which the developmental disorder occurred.

With osteodysplasia, excessive development of bone substance occurs in the bone, or instead of bone there is cartilaginous or fibrous tissue. As a result, bone deformations develop, which can lead to dysfunction.

Osteodysplasia is detected in the first years of life, in adolescence and less often in older age. Recognition of osteodysplasia is based on data x-ray examination. On radiographs, depending on the form of osteodysplasia, areas of osteosclerosis (see) and thickening of the cortical layer, single or multiple cysts, thinning of the cortical layer, as well as areas devoid of bone structure are visible. With fibrous osteodysplasia, in contrast to fibrous osteodystrophy (see), there is no osteoporosis in areas of the bone adjacent to the lesion.

Osteodysplasia (from the Greek osteon - bone + dysplasia) is a pathological condition of the skeleton caused by a congenital disorder of the osteogenesis process.

Osteodysplasia can be clinically detected in the first years of life or much later - in adolescence and adolescence, depending on the degree of skeletal damage and mainly on the deformations with which it is accompanied. Changes in the skeleton with osteodysplasia can be localized within one, two or many bones; they can be generalized. When many bones are affected, the changes are usually unilateral or predominantly unilateral. The bones of only the upper or only the lower limb may be selectively affected.

Osteodysplasia has many varieties, depending on what phase of osteogenesis the disorders occurred. If these disorders relate to the phase of bone tissue formation, melorheostosis, osteopoikilia, the so-called genuine osteosclerosis, generalized hyperostosis, or Engelmann's disease occur (with all these forms there is excessive development of normally formed bone tissue), osteogenesis imperfecta or the so-called increased fragility of bones, marble disease and osteomyelodysplasia (in which the ratio in the formation of bone and hematopoietic tissue is disrupted) and a number of other diseases.

Impaired osteogenesis in osteodysplasia can occur during the formation phase cartilage tissue, and then diseases arise that are combined into the group of skeletal chondrodysplasias (multiple cartilaginous exostoses, enchondromas, Ollier's disease). Finally, a violation of osteogenesis can occur during the formation of fibrous tissue, and then fibrous osteodysplasia develops. Fibrous dysplasia as an independent nosological form was identified by Lichtenstein [Lichtenstein (L. Licbtenstein)] and then Jaffe [Jaffe (N. Jaffe)]. Previously, it was described under the names fibrous osteodystrophy, fibrocystic disease, fibroosteoma.

Fibrous osteodysplasia has clear clinical and radiological characteristics and certain patterns of progression.

Pathological anatomy. There are two forms of fibrous osteodysplasia - monoostotic (damage to one bone) and polyostotic (damage to several bones), often unilateral. In tubular bones, the process develops in the metaphyses and diaphysis, rarely in the epiphyses.

The affected bone is very easy to saw. When cut, its cortical layer is thinned. The dilated medullary canal contains whitish-pink tissue with a fibrous appearance (Fig. 1). When you run your finger along the cut surface, small bone inclusions are identified in the tissue. Single or multiple smooth-walled cysts containing serous or hemorrhagic fluid may occur in the affected areas. Less common are cartilage inclusions, which are usually located close to the epiphyseal plate. Due to the thinning of the cortical layer and the proliferation of fibrous tissue, the affected areas of the bone take on a swollen, deformed appearance. Thinning of the cortex leads to decreased bone strength and pathological fractures. Flat Bones in the affected areas they are swollen, when cut, the cortical plates are thin, the bone substance of the spongy layer is replaced by fibrous tissue.

Microscopically, growth of cellular-fibrous tissue with foci of formation of bone structures in it is found. Osteogenic tissue may consist predominantly of immature elongated cells, among which there are a few bone beams (Fig. 2). Such multicellular areas sometimes make it possible to differentiate the process from sarcoma. In other cases, collagen fibers predominate (Fig. 3), arranged in the form of intertwined bundles, sometimes hyalinized. Bone formations represent different stages of bone development, from primitive fibrous and osteoid rays to more mature bone structures. The bone beams are characterized by a disordered afunctional arrangement. Sometimes the bone beams form bizarre interlaces, reminiscent of the structure of cancellous osteoma (Fig. 4). When the bones of the skull and especially the jaw bones are affected, bone inclusions may take the form of spherical layered formations resembling cementicles. Often in pathological tissue There are foci consisting of mononuclear and giant multinucleated cells, the origin of which A.V. Rusakov explains by overexcitation of bone-forming tissue. Cartilage inclusions may microscopically resemble chondroma or have the structure of mature hyaline cartilage.

Rice. 1. Tumor-like focus of osteogenic tissue in the diaphysis of the tubular bone. (Drug T. F. Ganzhulevich.)
Rice. 2. Osteogenic tissue with a primitive bone beam.
Rice. 3. A network of delicate collagen fibers in the tissue of fibrous osteodysplasia.
Rice. 4. Areas with big amount bone beams.

Fibrous osteodysplasia may be associated with extraskeletal lesions. Its combinations with multiple fibromyxomas of soft tissues, neurofibromatosis of the skin, etc. are described. Sometimes in patients with fibrous osteodysplasia, areas of pathological skin pigmentation and premature puberty. This triad of symptoms is called Albright syndrome. More often, Albright's syndrome is observed in the polyostotic unilateral form of fibrous osteodysplasia. Skin pigmentation manifests itself in the form of landcard-shaped or freckled spots of brown color on the skin of the back of the head, neck, back, lower back, buttocks, and thighs. The location of pigment spots may not correspond to the side of the bone lesion. Dark spots are noted at birth or appear in the first years of life. Microscopically, melanin deposits are found in the basal layer of the epidermis. Precocious puberty with Albright syndrome occurs predominantly in girls, manifesting itself in the form of early menstruation and early development secondary sexual characteristics. In boys, it is rarely observed and consists of rapid growth, early deepening of the voice, and premature pubic hair growth. Others are often observed hormonal disorders(persistent thymus, adenoma thyroid gland, hyperthyroidism).

Clinical picture(signs and symptoms). Fibrous osteodysplasia is benign, the prognosis is good (ossification is slow, much later than usual, but still occurs). The disease is detected most often in childhood or adolescence, occasionally in older late age, developing very slowly and stabilizing when skeletal growth stops. It occurs 3-4 times more often in women than in men.

With fibrous osteodysplasia, bones of both cartilaginous and connective tissue origin can be affected. Lesions of the bones of the lower extremity (femur and tibia) predominate, and of the flat ones - the pelvic bones. Often, varying degrees of pain appear, usually occurring after an injury or significant stress. Sometimes the first manifestation is pathological fracture followed by consolidation. With large thickenings, especially in the peripheral bones, a swelling of one size or another is detected, painless on palpation.

Growth disturbances are also possible: true elongation of the limb, accompanied by its arched curvature. When the epiphyseal zone is involved in the process, bone shortening occurs. In this case, movement restrictions and rapid fatigue are possible. Significant deformities can be detected by eye, especially hip deformities that cause lameness.

No changes in other organs and systems, including the endocrine system, are detected in fibrous osteodysplasia, with the exception of Albright's syndrome (see above). The calcium content in the blood serum is not increased. Only isolated cases of malignancy of fibrous osteodysplasia are known.

X-ray picture Fibrous osteodysplasia is characterized by a number of features. In the affected areas, the cortical layer is thinned from the inside, but is not interrupted anywhere. In some places, on the contrary, it can be compensatory thickened. Its internal contour is scalloped. The bone structure appears diffusely or focally sparse, resembling in shape cystic changes(Fig. 5). Against the background of rarefaction there may be various types bone crossbars - from barely visible to rough, creating a trabecular or cellular pattern (Fig. 6). The diaphyseal sections of long tubular bones are mainly affected. Changes in the structure of the epiphyses and apophyses, which have independent ossification nuclei, are rarely observed. Even in defeat femur, where the predominant localization is the proximal part, the head and trochanters remain unchanged. The zones of growth cartilages are like the boundaries of the affected area. When the process passes beyond the epiphyseal growth line, it may change articular surface bones.

Along with structural changes the affected parts of the bones are swollen to varying degrees, and variously expressed curvatures are noted. When the hip is affected, this is a typical coxa vara (Fig. 7). At the tops of the curvatures, zones of restructuring may appear. The morphological substrate of radiographically detectable diffuse or focal cyst-like clearings is the same diffuse or nodular accumulations of dense fibrous tissue (and not true cysts filled with liquid contents).

Rice. 5. Single cyst-like foci of rarefaction in the diaphysis of long tubular bones. Mild swelling at the level of these lesions.
Rice. 6. Large-cell and fine-cell restructuring of the structure over a significant extent of the diaphyses of long tubular bones without deformation and with significant arcuate deformation.
Rice. 7. Focal lesion of the right half of the pelvis and femur with its arched curvature and a decrease in the neck-shaft angle (coxa vara).

Changes are observed in the skull, which is affected alone or in combination with other bones. In the vast majority of cases, bone growths in the brain and facial areas skulls are one-sided, causing more or less pronounced asymmetry of the head (elefantiasis cranii). In this case, compression of the brain, its membranes, and exophthalmos (due to deformation of the orbit) occur as secondary phenomena. When the upper jaw is affected, obliteration of the air cavities occurs. When changes are localized in the area of ​​the base of the skull, narrowing of the openings may occur with compression of the vessels and nerves passing through them. However, such complications are rare.

Differential diagnosis fibrous osteodysplasia with widespread skeletal lesions does not represent special difficulties. The X-ray picture allows us to exclude hyperparathyroid osteodystrophy based on the absence of systemic osteoporosis, the presence of unaffected bones and areas with unchanged bone structure in the affected bones, not to mention completely different clinical manifestations of this disease. It is often possible to distinguish fibrous osteodysplasia from bone chondromatosis, and in the absence of deformity from multiple eosinophilic granuloma, only after a biopsy. In the case of single-bone lesions, significant differential diagnostic difficulties are possible, which also require histological examination(puncture, biopsy).

The dynamics of the process during X-ray observation, even after long periods of time, is very slow. Reparative changes are reduced to eburnation and gross trabecular reconstruction of the bone (Fig. 8).

Rice. 8. Reparative changes in the form of filling of areas of rarefaction, eburnation and coarse trabecular bone restructuring.

Cases of fibrous osteodysplasia, accompanied by some extraosseous malformations (underdevelopment or absence of one kidney, coarctation of the aorta, arteriovenous aneurysms, etc.), as well as several cases of a combination of fibrous osteodysplasia with multiple myxomas, have been described.

Albright syndrome is also classified as fibrous osteodysplasia (see Albright syndrome).

Treatment fibrous osteodysplasia in cases with advanced deformation is reduced to conservative orthopedic measures. To active surgical intervention should be treated with caution, since extensive resections and curettages occur with sluggish bone regeneration (fibrous tissue is replaced not by bone, but by fibrous tissue).