Osteosarcoma

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An osteosarcoma ( OS ) or osteogenic sarcoma ( OGS ) is a bone cancer tumor. In particular, it is an aggressive malignant neoplasm arising from primitive cells transformed from the mesenchymal origin (and thus sarcoma) and which exhibits osteoblastic differentiation and produces malignant osteoid.

Osteosarcoma is the most common histologic form of primary bone cancer. This is most common in adolescents and young adults.

Video Osteosarcoma

Signs and symptoms

Many patients first complain of pain that may worsen at night, may be intermittent and the intensity varies and may have occurred for some time. Teenagers who are active in sports often complain of pain in the lower thighbone, or directly below the knee. If the tumor is large, it may appear as a clear local swelling. Sometimes a sudden fracture is the first symptom, because the affected bone is not as strong as normal bone and may break abnormally with mild trauma. In the case of a deeper tumor that is not as close to the skin, as it comes from the pelvis, local swelling may not be visible.

Maps Osteosarcoma

Cause

Several research groups are investigating cancer stem cells and their potential for causing tumors along with genes and protein causes in different phenotypes. Radiotherapy for unrelated conditions may be a rare cause.

Family cases where chromosome removal of 13q14 inactivates retinoblastoma genes is associated with a high risk of developing osteosarcoma.
Bone dysplasia, including Paget's bone disease, fibrous dysplasia, enchondromatosis, and some hereditary exostoses, increase the risk of osteosarcoma.
Rothmund-Thomson syndrome (ie, an autosomal recessive relationship in bone defects, hair and skin dysplasia, hypogonadism, and cataracts) is associated with an increased risk of this disease.
Large doses of Sr-90 emissions from nuclear reactors, seekers of nicked bones increase the risk of bone cancers and leukemia in animals, and are considered to do so in humans.

Although there are rumors that suggest otherwise, there is no clear relationship between water fluoridation and cancer or cancer deaths, both for cancer in general and also specifically for bone and osteosarcoma cancer. The series of studies concluded that the concentration of fluoride in water was not associated with osteosarcoma. Confidence about the association of fluoride and osteosarcoma exposure comes from a study of the US National Toxicology program in 1990, which shows uncertain evidence of fluoride and osteosarcoma associations in male rats. However, there is still no strong evidence of fluoride tendency that causes cancer in mice. Water fluoridation has been practiced throughout the world to improve people's dental health. It is also considered a major health success. The fluorine concentration levels in the water supply are regulated, as the United States Environmental Protection Agency regulates fluoride levels of no more than 4 milligrams per liter. Actually, the water supply already has natural fluoride, but many communities choose to add more fluoride to the point that it can reduce tooth decay. Fluoride is also known for its ability to cause new bone formation. However, further studies suggest no osteosarcoma risk from human fluoride water. Most studies involve counting the number of cases of osteosarcoma patients in a particular area that have a difference in the concentration of fluoride in drinking water. Statistical analysis of the data showed no significant difference in the incidence of osteosarcoma cases in different fluoride areas. Another important study involving the collection of bone samples from osteosarcoma patients to measure fluoride concentrations and compare them with bone samples from malignant newly diagnosed bone tumors. The result was that median fluoride concentrations in bone samples of osteosarcoma patients and tumor control did not differ significantly. Not only the concentration of fluoride in bone, the fluoride exposure of osteosarcoma patients also proved not to differ significantly from healthy people.

Osteosarcoma (PBL) - by Nathan Nam [Infographic]

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Mechanism

Osteosarcomas tend to occur in places of bone growth, probably because proliferation makes osteoblastic cells in this region susceptible to acquire mutations that can cause cell transformation (the RB gene and p53 gene are commonly involved). Because of this tendency, the high incidence of osteosarcoma is seen in some large breeds of dogs (St. Bernards and Great Danes). The tumor may be localized at the end of long bones (usually in metaphysis). Most often it affects the proximal end of the tibia or the humerus, or the distal end of the femur. Osteosarcoma tends to affect the area around the knee in 60% of cases, 15% around the hips, 10% in the shoulder, and 8% in the jaw. The tumor is solid, hard, irregular ("fir-tree," "moth-eaten", or "sunlight" appearance on X-ray examination) because of tumor spicules from calcified bone radiating in right angles. This right angle forms what is known as the Codman triangle, which is a characteristic but not an osteosarcoma diagnostic. The surrounding tissue is infiltrated.

Microscopic: The hallmark of osteosarcoma is the presence of osteoid (bone formation) in the tumor. The tumor cells are very pleomorphic (anaplastic), some are gigantic, many atypical mitoses. These cells produce osteids describing irregular trabeculae (amorphous, eosinophilic/pink) with or without central calcification (hematoxylinophilic/blue, granular) - tumor bone. Tumor cells are included in the osteoid matrix. Depending on the features of existing tumor cells (whether they resemble bone cells, cartilage cells, or fibroblast cells), tumors can be subclassified. Osteosarcomas can show giant osteoclast-like multinucleic cells.

chondroblastic osteosarcoma - Humpath.com - Human pathology

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Diagnosis

Family and orthopedic doctors rarely see malignant bone tumors (most bone tumors are benign). The route of diagnosis of osteosarcoma usually begins with X-rays, followed by a combination scan (CT scan, PET scan, bone scan, MRI) and ends with a surgical biopsy. The characteristic often seen on X-rays is the Codman triangle, which is essentially a subperiosteal lesion that develops when the periosteum is removed by a tumor. The film is suggestive, but a bone biopsy is the only definitive method to determine whether the tumor is malignant or benign.

Often, early signs of osteosarcoma are caught on X-rays taken during routine dental examinations. Osteosarcoma often develops in the lower jaw (lower jaw); Therefore, Dentists are trained to look for signs that may indicate osteosarcoma. Although radiographic findings for these cancers vary widely, a person usually sees a symmetrical dilation of the periodontal ligament space. If the dentist has reason to suspect osteosarcoma or other underlying disorder, he or she will refer the patient to Oral & amp; Maxillofacial surgeon for biopsy. Osteosarcoma suspect biopsies outside the face area should be performed by qualified orthopedic oncologists. The American Cancer Society states: "Perhaps no other cancer is important to perform this procedure properly. An incorrect biopsy may make it difficult to save affected limbs from amputation." It may also be metastasized to the lungs, especially appearing on chest x-rays as solitary or solitary nodules some of the most common in the lower regions.

Variant

Conventional: osteoblastic, chondroblastic, fibroblastic OS
OS Telangiektatic
Small cell OS
Low class center OS
The Periosteal OS
The Paraosteal OS
Secondary OS
high-quality surface OS
Extraskeletal OS

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Treatment

Mifamurtide is used after the patient undergoes surgery to remove the tumor and along with chemotherapy to kill the remaining cancer cells to reduce the risk of cancer recurrence. Also, the option to have rotationplasty after a tumor is taken there.

Patients with osteosarcoma should be managed by a medical oncologist and an orthopedic oncologist with experience in dealing with sarcomas. The current standard treatment is to use neoadjuvant chemotherapy (chemotherapy administered before surgery) followed by surgical resection. The percentage of tumor cell necrosis (cell death) seen in tumors after surgery provides an overview of the prognosis and also allows the oncologist to know if the chemotherapy regimen should be changed after surgery.

Standard therapy is a combination of limb-orthopedic surgery if possible (or amputation in some cases) and high-dose methotrexate combinations with leucovorin rescue, intra-arterial cisplatin, adriamycin, ifosfamide with mesna, BCD (bleomycin, cyclophosphamide, dactinomycin), etoposide, and muramil tripeptida. Rotationplasty can be used. Ifosfamide can be used as adjuvant treatment if the necrotic level is low.

Despite the success of chemotherapy for osteosarcoma, it has one of the lowest survival rates for childhood cancer. The best reported 10-year survival rate was 92%; the protocol used is an aggressive intra-arterial regimen that adjusts therapy based on an arteriographic response. The 3-year free incidence of free survival ranged from 50% to 75%, and five-year survival ranged from 60% to 85% in some studies. Overall, 65-70% of patients treated five years ago will live today. This survival rate is on average overall and varies greatly depending on the level of individual necrosis.

Filgrastim or pegfilgrastim helps the number of white blood cells and the number of neutrophils. Blood transfusions and epoetin alfa help to overcome anemia. Computational analysis on the Osteosarcoma cell line tubes identifies newly divided and specific therapeutic targets (proteomics and genetics) in Osteosarcoma, while the phenotype shows an increased role of tumor microorganisms.

Osteosarcoma (Conventional) : Bone Tumor Cancer

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Epidemiology

Osteosarcoma is the most common form of cancer of children aged eight, comprising 2.4% of all malignancies in pediatric patients, and about 20% of all primary bone cancers.

The incidence rate for osteosarcoma in US patients under the age of 20 is estimated at 5.0 per million per year in the general population, with slight variations among individuals of black, Hispanic, and white ethnicity (6.8, 6.5, and 4.6 per million per year). , each). This is slightly more common in men (5.4 per million per year) than in women (4.0 per million per year).

It originated more frequently in the metaphyseal region of long tubular bone, with 42% occurring in the femur, 19% in the tibia, and 10% in the humerus. About 8% of all cases occur in the skull and jaw, and the other 8% are in the pelvis.

About 300 of the 900 people diagnosed in the United States will die each year. The second peak in the incident occurs in the elderly, usually associated with underlying bone pathology such as Paget's bone disease.

Osteosarcoma - conventional (histology) | Radiology Case ...

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Prognosis

The prognosis is separated into three groups.

Stage I osteosarcoma is rare and includes parosteal osteosarcoma or low grade central osteosarcoma. It has an excellent prognosis (& gt; 90%) with wide resection.

Stage II

Initial presentation of osteosarcoma stage III with pulmonary metastases depends on resection of primary and pulmonary nodules, the degree of primary tumor necrosis, and possibly the number of metastases. The overall survival prognosis is about 30%.

Deaths due to malignant neoplasms of bone and joints cause an unknown child mortality rate. The mortality rate due to osteosarcoma has decreased by about 1.3% per year. The long-term survival survival for osteosarcoma has increased dramatically during the late 20th century and is estimated to reach 68% in 2009.

Pathology Outlines - Periosteal osteosarcoma

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Animal

Risk factors

Osteosarcoma is the most common bone tumor in dogs and usually affects large and giant middle-aged dogs such as Irish Wolfhounds, Greyhounds, German Shepherds, Rottweilers, mountain breeds (Great Pyrenees, St. Bernard, Leonberger, Newfoundland), Doberman Pinschers and Great Danes. It has a 10-fold greater incidence in dogs than humans. The hereditary base has been shown on the dog St. Bernard. The smoked/castrated dog has twice the risk of developing osteosarcoma. Infestation with parasites Spirocerca lupi can cause osteosarcoma esophagus.

Clinical presentation

The most commonly affected bones are the proximal humerus, the distal radius, the distal femur, and the tibia, following the basic premise "away from the elbow, close to the knee". Other sites include ribs, lower jaw, spine, and pelvis. Rarely, osteosarcoma can arise from soft tissue (osteosarcoma extracelleletal). Metastatic tumors involving leg bones are very common, usually to the lungs. Tumors cause a lot of pain, and can even cause an affected fracture. Like human osteosarcoma, bone biopsy is the definitive method for achieving final diagnosis. Osteosarcoma should be distinguished from other bone tumors and other lesions, such as osteomyelitis. The differential diagnosis of osteosarcoma in the skull typically includes, inter alia, chondrosarcoma and multilobular bone tumors.

Treatment and prognosis

Amputation is the initial treatment, although this alone will not prevent metastasis. Chemotherapy combined with amputation increases survival time, but most dogs still die within a year. Surgical techniques designed to save the feet (limb-saving procedure?) Do not increase the prognosis.

Several current studies suggest that osteoclast inhibitors such as alendronate and pamidronate may have beneficial effects on quality of life by reducing osteolysis, thereby reducing the level of pain, as well as the risk of pathologic fractures.

Cat

Osteosarcoma is also the most common bone tumor in cats, although not commonly encountered, and most often affects the hind legs. Cancer is generally less aggressive in cats than in dogs, so amputation alone can lead to significant survival time in many affected cats, although post-amputation chemotherapy is recommended when high levels are confirmed in histopathology.