Disease sexually transmitted:


Osteogenic sarcoma

  • What is Osteogenic sarcoma
  • What triggers / Causes of osteosarcoma
  • Pathogenesis (what is happening) during Osteogenic sarcoma
  • The symptoms of osteosarcoma
  • Diagnosis of osteosarcoma
  • Treatment of osteosarcoma
  • Which doctors should be consulted if You have osteosarcoma

What is Osteogenic sarcoma -

Osteogenic sarcoma is a sarcoma, malignant cells which originate from the bone and produce this fabric. In some of these tumors are dominated by chondroblastoma or fibroblastic components. Radiographically divided into osteolytic, osteoplastic (sclerotic) and mixed forms.
Osteogenic sarcoma is an extremely malignant tumor. Arising directly out of the bone elements, it is characterized by rapid flow and a tendency to early to give metastases.
Osteosarcoma occurs at any age, but about 65 of all cases falls on the period from 10 to 30 years, and often the development of sarcomas is celebrated at the end of puberty. Men are affected twice as often than women. Favorite localization are the long tubular bones, the share of flat and short bones decreases not more than one-fifth of all osteogenic sarcomas. Bones of the lower limb 5-6 times more affected than the bones of the upper extremities, and 80 of all tumors of the lower extremities nest in the knee. The first number is the thigh, which falls half of all osteogenic sarcomas, followed by tibia, humerus, pelvis, fibula, shoulder girdle, elbow bone. Radial bone, where so often there is giant cell tumor, only rarely gives rise to osteogenic sarcoma. Almost never osteogenic sarcoma does not arise from the patella. Cranial lesion occurs mainly in childhood and in old age as disfiguring complications osteodystrophy. A typical localization of osteogenic sarcoma in long tubular bones is a meta - epiphyseal end, and the children and young people, before the onset of synostosis, the metaphysis of the bone. In the femur commonly affects the distal end, but about 10 of osteogenic sarcoma of the thigh nest in the shaft and leave the metaphyses intact. In the tibia osteosarcoma in only one case out of ten, you'll be in the distal end - a typical choice is the proximal medial condyle. The same typical choice for the humerus is the area of the roughness of the deltoid muscle.

What triggers / Causes of osteosarcoma:

Tumor development has some connection with the rapid growth of bones. Children suffering from osteosarcoma, usually taller compared to the age norm, and the disease affects the most rapidly growing parts of the skeleton.
The development of bone tumors is often associated with trauma, but trauma attracts the attention of a physician and forces to conduct x-ray examination
Sole agent in the external environment is known as a stimulator of bone sarcomas - ionizing radiation. Moreover, the interval between the impact factor and the occurrence of osteosarcoma can be from 4 to 40 years (average age 12 - 16).
Among those suffering from Paget's disease 2 develop osteosarcoma, often with multiple lesions of the bones.
The presence of benign bone tumors (osteohondromy, enchondroma, etc.) increases the risk of developing osteosarcoma.
Among patients treated for retinoblastoma, 50 secondary tumors account for osteosarcoma (retinoblastoma - tumor, often having hereditary), and in both diseases are the same changes in pair 13 chromosomes.

Pathogenesis (what is happening) during Osteogenic sarcoma:

Important role in the pathogenesis of osteosarcoma mutation plays a tumor suppressor gene P-53. In some cases, tumor development will also be affected by inactivation of other tumor suppressor genes - RBL (retinoblastoma suppressor gene that confirms a connection of these diseases), DCC gene, isolated from cells of colon-carcinoma (suggesting that this gene is localized in the long arm of chromosome 18, which is often missing in the cells of the human osteosarcoma).
At the present time the attention of researchers is attracted by the study of the activity of the MDR gene determining resistance to drug therapy, which is associated with the P-glycoproteins secreted by osteosarcoma cells. These proteins provide protection of tumor cells, reducing the accumulation of her chemotherapy.

The symptoms of osteosarcoma:

The major clinical symptom of osteosarcoma is pain over the affected area. The pain is a dull, consistent with a gradual increase in intensity. The characteristic symptom is night pain. In 3/4 patients can present soft-tissue component. The limb is increased in volume, often looks swollen. The pain and the increase in lead to dysfunction. The duration of history is on average 3 months.
Characteristic lesions of the metaphyses of long bones. The most frequent localization (about 50 cases) - the knee joint is the distal part of the femur and the proximal tibia. Often also affects the proximal part of the humerus and femur, and the middle third of the femur. The lesions of flat bones, especially the pelvis occurs in children less than 10 cases.
Osteosarcoma has a great tendency to the development of hematogenous metastases. By the time of diagnosis 10-20 patients have macro metastases in the lungs detected radiographically. But about 80 of patients at diagnosis have micrometastases in the lungs, not detected radiographically, but visible during computed tomography. Because bones do not have a well-developed lymphatic system, the early proliferation of osteosarcoma in regional lymph nodes is rare but if it occurs, is a poor prognostic sign. Other areas of metastasis of the bone, pleura, pericardium, kidneys, CNS.
Osteosarcoma has a local aggressive growth, can spread to the epiphysis and the nearby joint (most often affects the knee and shoulder joints), extending along the intra-articular structures, through the articular cartilage, through pericapsular space, or, in a direct way, because of pathological fracture, and not to form the adjacent pockets satellites - the "skip"metastases.
The disease
Onset of the disease cannot always be accurately determined. Vague dull pain appear near the joint, as the primary tumor is localized mostly near metaphyseal Department of the tubular bones. There is a pain without objective evidence of effusion in it, often after an injury in the past. With the expansion of the boundaries of the tumor and involvement of adjacent tissues, the pain intensified. Appear distinct thickening metadiaphyseal of the division of bone, expressed pastoznost tissues, venous network of the skin is clearly defined. By this time contracture occurs in the joint, lameness increases. Palpation - a sharp pain. Strong nocturnal pain which does not respond to aspirin, not related to the function of limbs and persisting even during fixation in plaster. The tumor is spreading rapidly to neighboring tissues, quickly fills the medullary canal, grows muscles, very early gives extensive hematogenous metastases, especially to the lungs, brain - bone metastases are extremely rare.
Rare variants of osteosarcoma.
Telangiectatica - radiographically resembles aneurysmal bone cyst and giant cell tumor, manifested by the presence of lytic lesions with poorly pronounced sclerosis. The course of disease and response to chemotherapy is almost no different from the standard variants of osteosarcoma.
Juxtacortical (periosteal) - proceeds from cortical layer of the bone, tumor tissue from all sides to surround the bone, but generally does not penetrate into the medullary canal. Soft tissue component is missing, so radiographically the tumor difficult to distinguish from osteoid. Generally, this tumor is low-grade, flows slowly, almost not give metastases. However, periosteal osteosarcoma requires adequate surgical treatment, virtually the same as the standard variants of the tumor. Otherwise, the tumor recurs and tumor component changes the degree of malignancy to a higher, which determines the prognosis of the disease.
Periodcally - as well as periosteal, is located at the surface of the bone and has a similar flow. The tumor is a soft tissue component, but does not penetrate into the medullary canal.
Interscalene tumors with low malignancy, well differentiated, with minimal cellular atypia, can be regarded as a benign tumor. But they also have a tendency to local recurrence with changes in tumor component at a more malignant variant.
Multifocal - manifested in the form of multiple foci in the bones, similar to each other. It is not clear to the end, whether they appear immediately, or is rapid metastasis from the same site. Prognosis fatal.
Extraskeletal osteosarcoma is a rare malignant tumor characterized by the production of osteoid or bone tissue, sometimes along with cartilage in the soft tissues, most often the lower limbs. But there are lesions to other areas such as the mouth, kidneys, esophagus, intestines, liver, heart, bladder, etc., the Prognosis remains poor, sensitivity to chemotherapy is very low. Diagnosis extraskeletal osteosarcoma can only be set after exclusion of the presence of bone tumor foci.
Small cell osteosarcoma - vysokokachestvennaya tumor, according to their morphological structure differs from the other options that determines its name. Most often the tumor is localized in the femur. Small cell osteosarcoma (differential diagnosis with other small cell tumors) necessarily produces osteoid.
Osteosarcoma of the pelvis - despite the improvement in overall survival in osteosarcoma, has a very poor prognosis. The tumor is characterized by rapid and extensive distribution into tissues and along them, because of the anatomical structure of the pelvis, not encounters significant and anatomical fascial barriers.
Staging of osteosarcoma (Enneking WF, Spanier SS, Goodman MA, 1980, USA)
Stage IA well-differentiated tumor. The lesion is limited to a natural barrier against the spread of the tumor. The absence of metastases
Stage IB - well-differentiated tumor. The lesion extends beyond the natural barrier. The absence of metastases
Stage IIA - poorly-differentiated tumor. The lesion is limited to a natural barrier. The absence of metastases.
Stage IIB - poorly-differentiated tumor. The lesion extends beyond the natural barrier. The absence of metastases.
Stage III - the Presence of regional and distant metastases, regardless of the degree of tumour differentiationОстеогенная саркома

Diagnosis of osteosarcoma:

The diagnosis of osteosarcoma is based on histopathological criteria in conjunction with x-ray data. Standard histological variants of osteosarcoma - osteoblastic bone (50), chondroblastoma (25) and fibroblast - less than 20
1. Full x-ray examination.
This study allows to suspect the presence of osteosarcoma in a patient, and detects the presence of a soft tissue component, pathological fracture, determines the size of the tumor, and the optimal level of biopsy.
Radiological signs of osteosarcoma:
- metaphyseal localization in long tubular bones-
- the presence of sclerotic and lytic lesions in bone, the presence of vascularization-
- the pathological foci of osteopathy in the soft tissues-
- violation of the integrity of the periosteum to form a "canopy" or "triangle Colmena"-
- needle periostitis - "spicules" (the proliferation of the periosteum in the form of needles arranged perpendicular to the surface of the bone)-
- chest x-ray reveals the macro metastases.
But many x-ray findings can mislead:
- well-differentiated tumor-
lytic lesions or lesions with minimal sclerosis
- limiting lesion of the medullary canal
- atypical periosteal reaction
- rare intraorally localization
- rare skeletal localization (soft tissue, skull, rib, etc.).
2. Morphological examination of the tumor.
One of the common causes is impossible for limb-sparing surgery is poorly performed biopsy with local colonization of tumor cells, pathological fracture. Therefore, a biopsy should be performed by a surgeon and it is preferable to bone marrow biopsy, rather than a knife biopsy (for maximum protection adjacent to the tumor tissues from contact with the biopsy).
3. Bone scan (OSG) with Te-99 - allows you to identify other lesions in the bones, while increasing the accumulation of the isotope is not specific. When conducting OSG in the dynamics of the change in the percentage accumulation of the isotope in the hearth before and after chemotherapy, it is possible to accurately judge the effectiveness of chemotherapy. A significant decrease in the percentage accumulation of the isotope in the hearth accurately correlates with good histologic response of the tumor to chemotherapy.
4. Computed tomography (CT) focus - enables you to identify the precise localization of the tumor, its size, the relation of the tumor to the surrounding tissues, spread it on the joint. Lung CT scan allows you to detect micrometastases not detected radiographically.
5. Magnetic resonance imaging (MRI).
The most precise method of contrast enhancement of the tumor, revealing its relation to the surrounding tissues, the neurovascular bundle, and also allows to determine the dynamics of the process in the case of chemotherapy, its efficacy and, accordingly, to plan the operation. Currently MRI scan performed with contrast agent containing gadolinium, which is accumulated on the periphery of the tumor, clearly otgranichivaya her.
In the largest cancer clinics around the world use advanced method - DEMRI - dynamic capture of a contrast agent as determined by MRI. Using a computer is performed quantification (b) tumor cells accumulating contrast before and after chemotherapy, thereby defining histological tumor response to treatment in the preoperative period.
6.Angiography is performed before surgery. This method reveal free or not vessels from the tumor, which determines the volume of transactions. In the presence of the vascular tumor emboli, holding limb-sparing surgery is not possible.
The differential diagnosis of osteosarcoma is carried out between chondrosarcoma, eosinophilic granuloma, cartilaginous exostosis, osteoblastoclastoma.
Остеогенная саркома
Osteogenic sarcoma

Treatment of osteosarcoma:

Treatment of osteosarcoma includes the following stages:
1. Preoperative chemotherapy to suppress micrometastases in the lungs, reducing the size of the primary lesion of tumor and histological evaluation of tumor response to chemotherapy, which determines the further course of treatment. For the treatment of osteosarcoma currently uses the following drugs: high-dose methotrexate, adriamycin, ifosfamide, platinum drugs (carboplatin, cisplatin), etoposide.
2. Mandatory operation. If you have previously resorted to major surgery, often involving amputation of the entire limb, it is currently limited sparing surgery. Removing only part of the bone to replace it with an implant made of plastic, metal or cadaveric bone. From conserving surgery refuse in cases when the tumor invades the neurovascular bundle, if there was a pathological fracture, and the larger size of the tumor and its spreading into the soft tissues. The presence of metastases is not a contraindication to limb-sparing surgery. Large metastases in the lungs are also removed surgically.
3. Postoperative chemotherapy based on the results of preoperative chemotherapy.
Radiation therapy for the treatment is ineffective due to the fact that the cells of osteosarcoma insensitive to ionizing radiation. Radiation therapy is carried out in the event that if for any reason the operation is not possible.
The emergence of new approaches that adjuvant and neoadjuvant chemotherapy and radiotherapy in addition to surgery and the development of gentle methods significantly increase the survival of patients with osteogenic sarcoma. Significantly increased the chances for the treatment of patients with pulmonary metastases.
Radical surgery with preservation of the limb (perhaps more than 80 patients) in conjunction with preoperative and postoperative chemotherapy gives better results. In the treatment of patients with localized osteogenic sarcoma 5-year survival exceeds 70. The survival of patients with sensitive to chemotherapy tumors varies in the range of 80-90.

Prognostic factors (according to research COSS-77-91)
1. Determining the volume of tumor mass.
Is made prior to treatment.
For ellipsoidal tumors: length x width x thickness x 0,52
For tumors of the discrete type: length x width x thickness x 0,785
Patients are divided into groups, depending on the volume of tumor mass:
Group 1 - 70 ml and less - favorable.
Group 2 - 71 - 150 ml - intermediate.
Group 3 - 150 ml - high risk.
When the tumor volume more than 200 ml of half of patients develop secondary metastases.
2. Histological tumor response to chemotherapy.
This indicator is expressed as the percentage of surviving tumor cells throughout the tumor tissue that is removed during surgery after preoperative chemotherapy (CT). Histological tumor response to chemotherapy determines the prognosis and further treatment tactics. Depending on the number of live tumor cells are defined by 6 degrees of histological response (Grad.n.Salzer - Kuntschik, Wien):
I - complete absence of viable tumor cells
II - a single living tumor cells or a site with live tumor cells < 0.5 cm-
III - IV - 10 - 50 live tumor cells
V - >50 live tumor cells
VI - no response to chemotherapy.
Auxiliary prognostic factors:
- the level of alkaline phosphatase (high level worsens the prognosis of the disease)
- localization of the tumor (Central localization and proximal part of the thigh is the most unfavorable localization).
Distribution of patients according to risk groups (depending on the degree of the intensity of treatment) - (COSS-96).
The low-risk group - patients with tumor volume is 70 ml or less, regardless of the degree of histological tumor response to chemotherapy (survival rate in this group with adequate treatment is 97).
High risk group - tumor volume of more than 150 ml, with 5 and 6 degrees of histological response (survival with standard treatment is 17).
Standard risk all others (survival of 67)
Each risk requires a certain therapy - longer and more intense at high risk, and less intense in the low-risk group.

Which doctors should be consulted if You Osteogenic sarcoma:

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