Encondrom and encondromatoza

Encondroamele are benign cartilaginous neoplasms in bone intramedullary solitary. Significant morbidity of these lesions is related to their complications, pathologic fracture and malignant transformation In a smaller percentage.More often than not shows the consequences encondroamele and patients are asymptomatic. Are not life threatening, however painful malignant transformation can not be excluded, even in this benign appearance on radiography and imaging. Malignant transformation is virtually nonexistent in the hands and feet but is seen in long bones and flat.
In a patient with chondrosarcoma encondromatoza incidence is much higher than the other patients-50%. In Maffuci syndrome, hemangiomas suffer sarcomatous transformation. In the case of malignant transformation to chondrosarcoma chondrosarcoma patients with low grade of differentiation were 5-year survival rate of 65-85%, while patients with high degree of differentiation, survival is 15%.
PathogenesisEncondroamele scrap consist of hyaline cartilage in the intramedullary bone. The lesions replace normal bone with mineralized hyaline cartilage or nonmineralizat, generating a character on radiogafii lithic lithic ring or zone of calcification condroida springs. Injuries result from displaced remnants of the cartilage growth plate.Endosteal development can occur and does not imply malignant transformation in the hands and feet, while cell lesions appear.Although enlargement is not correlated with cellularity malignant transformation, mitotic figures are common lesions and their presence is correlated with malignancy.Pathological fracture through the thinning of the cortex is not associated with malignancy in the hands and feet, though in other areas such as long and flat bones, pathologic fracture is suggestive of malignant transformation.
When multiple coexisting encondroame encondromatoza diagnosis is suspected.Encondromatoza appears in three different conditions:Ollier's disease, non-heritable disorder, characterized by multiple encondroame predilection for unilateral distribution, these are large and disfiguring touchMaffuci-syndrome-non-heritable and less than Ollier disease is characterized by multiple hemangiomas and encondroameConsist of multiple-metacondromatoza osteocondroame encondroame and is inherited autosomal dominant.
Signs and symptomsEncondroamele solitary are often found in patients between 20-40 years. Ollier disease is found between 0-10 years. Encondroamele intramedullary lesions are solitary, although it may expand enough to cause the appearance of endosteal shell. Shows predilection for the small bones of the hand and foot. Of these, half occur in the proximal phalanx, followed in frequency of metacarpal and middle phalanx, distal phalanges and wrist. Other sites are the shoulder, pelvis and long bones. Encondroamele tend to occupy the region in the shaft of tubular bones metaphyses region in the short and long. Ollier's disease occurs with high frequency in long bones.50% of solitary encondroamele are discovered in his hands. Next in frequency are the distal femur and proximal humerus.
When patients experience pain or injury is suspected, rapid growth of malignant transformation, even in the absence of radiographs suggestive. Encondroamele are metabolically active and can continue to grow and evolve throughout the patient's life, therefore, progressive calcification over a period of time is not unusual. Loss of focal calcification intro region suggests malignant degeneration with destruction encondromului by sarcomatous tissue.Primary clinical complications include pathological fracture and malignant transformation, which may be concomitant. In particular, pathological fracture does not involve malignant transformation in the hands and feet.
DiagnosisInitial X-rays may not show calcifications extending tomography.When they are not currently being carried out magnetic resonance imaging that shows the classic appearance of hyaline cartilage noncalcificat. Classic appearance of calcification in the rings and arcs is pathognomonic when it is observed tomorrow. In long bones, calcifications are difficult to distinguish from those of myocardial dystrophic bone. In Ollier disease encondroamele appear large. Because young people are occurring in large, growth is affected MEMBER stopped and pathological fractures occur. The syndrome is seen hemangioane Maffuci associated soft tissue. They shine round central calcifications.
Nuclear scanning with technetium 99 is typically negative in uncomplicated cases, but the presence of pathological fractures cause intense activity at the fracture site. In patients with multiple endocondroame calcified or they are active, the radionuclide scans showed increased activity. Radionuclide scanning is a method used to evaluate lesions detected on MRI scan or suspected encondroame. If the scan is negative the possibility of malignancy is extremely remote. If the scan is positive biopsy is necessary.
Differential DiagnosisWhen the lesion shows calcifications, the first differential diagostice as heart and skeletal chondrosarcoma. When the lesion is purely lithic nonosificant differential diagnosis includes fibroma, bone cyst, fibrous dysplasia, granuloma Chondrosarcoma epzinofilic and clear cell.
TreatmentMost encondroame not require any treatment. When it is appropriate vary widely in technique, forcing debate even among orthopedic surgeons through the difficulty of determining aggressive lesion.
Surgical therapy:Therapy of choice is surgical curettage expanded plastic or artificial bone graft. Skeletally immature patients is recommended to fill the bone defect with polymethylmethacrylate, this skeletal growth while allowing the tumor cells are destroyed. In older patients where skeletal development is not a problem bone grafting is recommended for small lesions. For large lesions and cement reconstruction is necessary.Filling the defect with polymethylmethacrylate is recommended after removal of recurrent lesions. If an aneurysm secondary bone cyst is currently considered the use of phenol and cryosurgery, due to the high rate of local recurrence of these lesions.