Chondrosarcoma is the second most common malignant bone, representing 25% of primary bone neoplasms. Chondrosarcomas represents a group of tumors with different characteristics, ranging from slow-growing lesions without metastases highly aggressive metastasizing tumors up.
Chondrosarcomas incidence is dependent on patient age. In children the incidence is low. Most tumors occur at ages over 40 years. The risk is increased in patients with chondrosarcoma syndromes encondromatoza: Ollier disease, syndrome Maffuci, metacondromatoza) and those with hereditary multiple exostoza.Patients with these conditions are generally young.
For patients with chondrosarcoma, prognosis is generally correlated with lesion size, anatomical location and histological grading of the site. Patients with axial lesions were most negative prognosis from those with lesions of the appendicular skeleton. 5-year survival rate for patients with grade 1 lesions is 90%, the rate dropped to 29% for those with grade 3 tumors.
Pathogenesis chondrosarcomaChondrosarcoma is a malignant tumor of origin in the formation of cartilaginous tumor matrix is likely condroida. Chondrosarcomas are classified as central (originating in the intramedullary canal) or peripheral. Juxtacorticale lesions rarely appear. Occur when lesions are primary or secondary de novo occur when a preexisting lesion or as a encondrom osteocondrom.Grade 1 lesions do not metastasize. Metastatic extension typically is most frequently associated pulmonary lesions of grade 3.Extending through the lymph nodes is more frequent in chondrosarcoma than in other bone neoplasms. Tumor Recurrence after surgery occurs in 5-10 years. Recurrent chondrosarcoma is more aggressive than the primary, and the histological grading is increased.Tumors are characterized by the degree of differentiation. Grade 1 is the least aggressive in terms of histology, and grade 3 is the most aggressive tumor. Most condrosarcoame are classified pathologically as conventional clear cell, myxoid, mesenchymal and undifferentiated.
Signs and symptomsAge of occurrence of Chondrosarcomas is over 40 years.Chondrosarcomas side affects patients between 20-40 years.Chondrosarcomas are rare in children, and when everybody is more aggressive. Tumors are predominantly axial location is the most common pelvic bones, femur, humerus, ribs, scapula, sternum or spine. The tubular bones of metaphysics is most affected. Metaphysics is the most frequently affected proximal than distal. Impaired distal humerus is unusual. Chondrosarcomas are rare hand and foot, and if complications are discovered are multiple encondromatozei syndrome.
The most common symptom at presentation is pain, which is present for several months and is of medium intensity. It may worsen at night. Local swelling may be present. When the tumor is adjacent to a joint may be present epansamentele or restrict movement. The average duration of symptoms before presentation is 1-2 years. The tumor occurs occasionally as a pathological fracture.
There are three additional types condrosarcoame:Mesenchymal chondrosarcoma:A rare variant with histological bimorph and undifferentiated cells resembling Ewing's sarcoma. Shows predilection for the spine, ribs and jaw. It is found in the third decade of life. It is more common in women and can grow extremely large. Spread to lungs, lymph nodes and other bones.Clear cell chondrosarcoma:It is a malignant cartilage tumor that may be the adult version of chondrosarcoma. It is a rare tumor, with less grading and better prognosis. It detects the femur and humerus epiphysis.Histologically soft tissue invasion is rare. Shows the clear cell cytoplasm vacuole. Calcified cartilage matrix shows trabeculae and giant cells.Chondrosarcoma didiferentiat:It is the most malignant form of chondrosarcoma. This tumor is a mixture of low-grade Chondrosarcoma and highly fibrous sarcoma, in which an aspect fibrocitele cartilage. Didiferentiate portion of the lesion may be malignant fibrous histiocitom histological data, undifferentiated sarcoma or osteosarcoma. It has a 10% survival rate at 5 years.
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