What are bone sarcomas
Sarcomas are heterogeneous groups of tumors that encompass three main groups: soft tissues, bones, and GIST (gastrointestinal stromal tumor). They are tumors that originate from the uncontrolled growth of bone cells and can affect any bone in the body.
They are rare tumors, with an incidence of around 0.2% of all cancers in adults. In Brazil, there are few epidemiological studies on the disease, but the comparison of regional data with international databases shows that the incidence and prognosis are similar to the rest of the world. Initiatives of cooperation between doctors and patients from various parts of the globe are extremely important for the better understanding and management of this disease.
The causes of sarcomas are unknown in most patients, although a small percentage is related to factors such as hereditary genetic syndromes (such as neurofibromatosis or von Recklinghausen disease, Gardner syndrome, Li-Fraumeni syndrome, retinoblastoma, Werner syndrome, Gorlin syndrome, and tuberous sclerosis), exposure to radiation, and chemical agents.
Subtypes of Bone Sarcomas
The classification of bone tumors takes into account the tissues produced by the tumors. The main types are:
- Osteosarcoma – This is the most common type of bone sarcoma. It affects children, adolescents, and young adults more frequently. It usually occurs in long bones, such as the femur, bones of the arms, and pelvis.
- Ewing’s Sarcoma – This is the second most common malignant bone tumor and affects children more frequently. It typically occurs in the femur and pelvis regions. Highly aggressive, it can also develop in soft tissues such as muscles and cartilage.
- Chondrosarcoma – The fundamental tissue of this type of bone tumor is formed by cartilage. It more commonly arises in long bones and the pelvis.
In addition to the three most common types, this heterogeneous group includes other subtypes such as pleomorphic sarcoma of the bone, fibrosarcoma, giant cell tumor of bone, and chordoma.
Symptoms and Signs of Bone Sarcomas
Pain, swelling in the affected area, and fractures are the most commonly found symptoms in patients with bone sarcoma. Fever may accompany the condition.
Some cases can be asymptomatic, with the tumor being an incidental finding in a routine examination.
Diagnosis of Bone Sarcomas
The diagnosis of bone tumors begins with a clinical examination and imaging tests such as X-rays, computed tomography (CT) scans, magnetic resonance imaging (MRI), and bone scintigraphy.
After the imaging analysis, the diagnosis is confirmed through a biopsy, which involves the removal of a piece of the tumor for laboratory analysis to confirm the subtype, aggressiveness grade, and molecular characteristics that may help in treatment.
Treatment for Bone Sarcomas
The low incidence, wide spectrum of subtypes, biological behaviors, and ubiquity make the management of osteosarcomas challenging. Therefore, treatment should be carried out in a multidisciplinary environment, with teams of experienced specialists.
Treatment depends on the type of bone tumor, size, and location of the tumor and may use the three most common modalities in oncology:
- Surgery;
- Radiation therapy;
- Chemotherapy.
In addition to disease control, preserving the limb with good motor function is one of the main treatment goals, with an individualized approach for each patient.
Prevention of Bone Sarcomas
Most bone sarcomas do not have a known risk factor, making prevention difficult. There is no routine test that can help detect the disease.
For those who have hereditary genetic syndromes related to bone tumors, regular screening tests can help with early diagnosis and result in a greater chance of cure in treatment.
