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Osteosarcoma

Believe that development of an osteosarcoma correlates with growth of bones. As a rule, the disease begins during rapid growth at the teenager. Middle age at the time of diagnosis makes 15 years.

Within the first 13 years of life at boys and girls the frequency of development of a tumor is identical, but further it raises at males, and at female persons remains stable. Results of one of researches demonstrate that children with an osteosarcoma differ in higher growth, than children of control group with other types of malignant new growths. Besides, the osteosarcoma meets at large breeds of dog more often (for example, the Danish mastiff). As a rule, process is localized in long tubular bones, in their metaphyses, points of the most active growth and reorganization of a bone. The distal department of a femur, quite often proximal departments of humeral and tibial bones serve more often the place of primary localization. The osteosarcoma can proceed from any bone.
At children with a bilateral retinoblastoma the incidence of an osteosarcoma is raised. In the past considered that it develops generally in the areas which underwent radiation, but there are more and more messages on its development in the bones which are out of its field and are shown in the form of multifocal defeat. The gene connected with a retinoblastoma can contribute also to development of an osteosarcoma.
Certain diseases of bones, the part of which is genetically determined, can also contribute to development of an osteosarcoma. Treat them a multiple chondromatosis (Ollye's illness) which can coexist with hemangiomas (Maffucci syndrome); multiple hereditary dysostosis; imperfect bone formation and Pedzhet's illness. The osteosarcoma (osteosarcoma) usually differs in family character. It meets and as a secondary tumor after treatment of the patient with the long period of a survival after Ewing's sarcoma; stage of latency makes 4 — 20 years.
Pathology. Understand primary malignant tumor of a bone which neoplastic cells create osteoids as an osteosarcoma. In classical cases it begins from a wall of the marrowy channel and, getting through all thickness of a bone, destroys a cortical layer with formation of soft weight which can reach the considerable sizes. The tumor can extend also on the marrowy channel. In the same center of defeat it can be shown osteo - hondro-and a fibrosarcoma. Its characteristic histologic signs are presented on fig. 1. It is important to distinguish osteosarcoma subtypes. The Parostalny osteosarcoma represents the differentiated extramedullary tumor with a low metastatic potential. Surgical treatment without use of other methods is considered adequate. On the contrary, a periosteal osteosarcoma with the same localization — histologically more polymorphic education with an aggressive clinical current.

Fig. 1. Diagnostic histologic characters of an osteosarcoma.
Kernels of cells of the different size with chromatin of different density are visible (more often it dark). Cells "are immured" in the main amorphous substance representing osteoid.
гистологические признаки остеосаркомы

The telangiectatic osteosarcoma represents cystous education with rich blood supply. At the same time on roentgenograms again formed bone tissue is not defined that reminds a picture of an aneurysmal bone cyst. The forecast can be adverse.
The osteosarcoma of mainly osteoblastic type (multiple sclerosing osteosarcoma) can arise at the same time in several places.
Clinical manifestations. Pain in the place of formation of a tumor belongs to the most frequent first signs. The patient and members of his family usually consider it as result of an injury. Later restriction of the movement and the tumor palpated or seen approximately can join. At its localization in bones of legs or a pelvic cavity lameness and disturbances of gait can develop. Morbidity, a local erythema and a hyperthermia belong to late manifestations. Most often the tumor metastasizes in lungs. In the beginning process in them proceeds usually asymptomatically, but respiratory insufficiency can develop later. The pleural exudate or pheumothorax can join. Metastasises can meet also in bones, inguinal lymph nodes and TsNS.
Diagnosis. At persistent inexplicable bone pain, especially at the palpated education, the radiological survey is required. Typical changes at the same time are presented on fig. 2. The bone is usually sclerosed, education in a periosteum of a new bone tissue attracts attention. Before carrying out primary surgery the minimum researches are necessary for definition of a stage of an illness to which radio isotope scanning, a X-ray analysis (fig. 3) and a computer tomography of a thorax belong. The last allows to reveal more damages in comparison with a X-ray analysis that is especially important concerning patients on whose roentgenogram changes were not found.

Fig. 2. Roentgenogram of distal department of a femur. The bast layer of a bone is destroyed by a tumor in which the calcification centers are visible.
Корковый слой кости разрушен опухолью
On the other hand, about a half of the nodes found by means of a tomography at adults are high-quality granulomas or near pleural lymph nodes that comes to light at a thoracotomy.
The contrast computer tomography of an extremity helps to determine extent of distribution of a tumor by the marrowy channel that has essential value when planning operation. If it is supposed to keep an extremity, it is necessary to make arteriography for the purpose of definition of a possibility of functioning of department, distal in relation to a tumor. Possible increase of activity of an alkaline phosphatase can serve as an indicator of efficiency of treatment. Diagnosis. it is necessary to confirm with results of histologic research at an open biopsy of the center of damage.
Treatment. Amputation of an extremity or broad local excision of a flat bone is recommended to patients without signs of innidiation of a tumor if it is possible. Amputation is made according to extent of distribution of the tumor on the marrowy channel revealed at a computer tomography. Himiopreparata were not used yet, only one amputation provided a survival during 5 flyings of 17% of patients. Metastasises in the lungs developing during 2 flyings at patients at whom at the time of diagnosis of a tumor their signs were absent were a usual cause of death. In this regard small uncontrolled researches with use of one himiopreparat are conducted.

Fig. 3. Multiple metastasises of an osteosarcoma.
Множественные метастазы остеосаркомы
Introduction of a methotrexate or doxorubicine in high doses allowed to increase survival level without aggravations approximately to 40%. The schemes of treatment providing use of several drugs (high doses of a methotrexate, Bleomycinum, Cyclophosphanum, Dactinomycinum, doxorubicine and platinum) can provide to 80% survival without aggravations. In one of supervision at patients, treated only by a surgical method, it reached 40%. The last regulated controlled research conducted by Pediatric Oncological Group allowed to compare results of the combined chemotherapy and only surgical treatment. They demonstrate that the complex chemotherapy serves as a choice Method for postoperative maintaining patients. The osteosarcoma is insensitive to influence of a X-ray.
For patients with the deleted pulmonary metastasises surgical treatment is recommended that survival level without aggravations brings closer to 20%. However quite often at small number of the centers on the usual roentgenogram find a large number at a computer tomography in the patient and bigger at operation.
Attempts to keep the affected extremity by carrying out before operation of chemotherapy with the subsequent resection of a bone and internal prosthetics were made. The choice of a method demands a careful assessment of relative functionality of an extremity with an internal prosthesis which will be much less strong, than healthy. In general the more active than the patient, the higher it adaptation after amputation of an extremity, than after introduction of an internal prosthesis. The last is more reasonable for a hand if it is possible to keep a brush, and axial loadings are small.
After amputation much attention should be paid to rehabilitation of the patient. In many cases postoperative stump neuralgias can disturb him. After operation the stump usually bulks up with the subsequent wrinkling that creates certain difficulties at prosthetics. Long psychological support of the patient is necessary.
Forecast. The forecast at tumors of low degree of a zlokachestvennost is optimum, for example at a parossalny osteosarcoma. Only surgical treatment at a classical osteosarcoma provides a long survival of only 20% of patients. Its level after intensive chemotherapy is still unknown, but it is not lower than 50%. Cases of a long survival after excision of the metastatic center in lungs are described, but cases of a survival of patients with diffusion metastatic process in lungs or a bone are not known.

 
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