Bone marrow tumors

Bone malignant tumors. Among primary malignant tumors, tumors from bone tissue (osteogenic sarcoma, parostal sarcoma, chondrosarcoma, malignant giant cell tumor) and non-native origin (Ewing's sarcoma, fibrosarcoma, chordoma, angiosarcoma, adamanthinum) are isolated from primary malignancies.

Osteogenic sarcoma occurs more often in the period of bone growth, at the age of 15 years, less often at a later age. Allocate osteoblastic, chondroblastic and fibroblastic sarcoma. Clinical symptoms develop rapidly: pain, difficulty in movement, anemia. A characteristic laboratory feature is an increase in alkaline phosphatase. Radiography shows a lesion of the metaphysis of long tubular bones and pelvic bones (97% of cases), less often the tumor is localized in other parts. Pre-tumor diseases (Paget's disease, osteomyelitis, etc.) precede osteogenic sarcoma in 5% of patients.

The diagnosis of osteogenic sarcoma, established by radiography of the bone, should be confirmed by biopsy of the tumor.

Osteogenic sarcoma has a significant propensity to metastasize to the lungs (70-90% of cases); Metastases develop rapidly within the first year after diagnosis. Early detection of metastases is facilitated by x-ray tomography and computed tomography of the lungs. Early diagnosis of the disease leads to a reduction in the volume of surgery and with additional chemotherapy provides a high chance of recovery.

Treatment should be conducted in specialized centers. In osteogenic sarcoma without metastases to the lungs, depending on the size and location of the tumor, limb amputation or bone resection is performed, and when the tumor is located in the pelvic bones or spine, radiation therapy is used. However, surgical treatment leads to the cure of only 20% of patients. The main problem is the development of pulmonary metastases. Therefore, postoperative chemotherapy is an obligatory component of complex treatment of osteogenic sarcoma, with a 5-year survival rate of 70%. If at the time of diagnosis, lung metastases have already developed, in some cases they are removed before chemotherapy begins. This approach significantly increases the effectiveness of chemotherapy, since micrometastases have the greatest sensitivity to cytostatic drugs. An important principle in the treatment of osteogenic sarcoma is the preoperative use of regional or systemic chemotherapy aimed at reducing tumor size, reducing the risk of additional dissemination during surgery, and exerting a cytostatic effect on possible distant metastases. A modern comprehensive treatment plan for osteogenic sarcoma can have several options. Preoperative chemotherapy is performed with adriamycin and / or cisplatin (regionally or systemically). In addition, local radiotherapy may be prescribed. After 2 weeks. After performing a radical operation, chemotherapy is started with a combination of adriamycin with cyclophosphamide, vincristine, sarcolysin or high doses of methotrexate with leucovorin, adriamycin.

The parostal sarcoma is characterized by slow growth, metastases rarely occur. They are ill at the age of 30-40 years. The distal metaphysis of the thigh is more often affected. After the operation, 70-80% of patients recover. With insufficient radicalism of the operation, a relapse of the disease is possible.

Chondrosarcoma occurs at the age of 30-50 years in the pelvic bones, proximal thigh, ribs, humerus. It grows slowly, reaches a large size. However, pain is not always noted, as well as restriction of movements. Metastases to the lungs occur in the late stages of the disease. More typical is the occurrence of relapses. After a radical operation, 50% of the patients recover.

Malignant giant cell tumor arises from benign (frequency 10-20%). It affects the epiphyses of long tubular bones. Surgical treatment is highly effective. Metastases in the lungs are rare, and a surgical technique can also be used. When the tumor is localized in the sacrum and pelvic bones, radiotherapy is performed, and cure is less often achieved.

Ewing's sarcoma is 15-20% of malignant tumors of the skeleton. Occurs at the age of 25 years. It affects primarily the diaphysis of long tubular bones, gives metastases to other bones and lungs. Clinical symptoms are pronounced: acute pain, fever, weight loss, leukocytosis, anemia. On the X-ray patterns, lytic destruction and reactive bone formation are visible. Radiation therapy is of primary importance in the treatment of primary tumors, the operation is used in contraindications to radiation therapy, incomplete effect and relapse. Meanwhile, radiation therapy, even in combination with surgery, and even more so without it, is cured by no more than 20% of patients, since the main problem is the formation of distant metastases. Therefore, treatment should always include chemotherapy, which increases the survival rate of patients to 70%. Chemotherapeutic regimens include adriamycin, dactinomycin, vincristine and cyclophosphamide. With metastases to the lungs, radiation therapy can additionally be used.

Fibrosarcoma is a rare variant of bone sarcoma. After surgery, 5-year survival is 15-40% (depending on tumor anaplasia).

Chordoma is a bone sarcoma of embryonic origin. Occurs at the age of 40-60 years, mainly in the terminal parts of the spine. It grows slowly, squeezes the nerve roots and spinal cord. Metastasizes late and rarely. After surgery, a tumor recurrence may occur.

Adamanthoma appears usually in the tibia (90%) in patients older than 30 years, growing slowly. The operation often leads to the cure of patients.

Primary malignant bone tumors must be differentiated from benign (osteoideostoma, giant cell tumor, osteoma, chondroblastoma, haemo-hyoma, etc.), non-tumor processes (hyperparathyroidism, Paget's disease, fibrotic dysplasia, bone cysts, histiocytosis, etc.), metastases of other tumors. Careful analysis of bone radiographs and biopsy are of paramount importance in diagnosis.