Adamantinoma of long bones
|Adamantinoma of long bones|
|X-ray (side and front views) showing an adamantinoma in the large bone of lower leg, near the ankle.|
|Symptoms||Swelling, with or without pain in the lower leg|
|Diagnostic method||X-ray, MRI|
|Frequency||Rare, 0.4% of primary bone tumors|
Adamantinoma of long bones is a cancerous bone tumor, that almost always occurs in the bones of the lower leg. Swelling over the affected bone with or without pain are the most frequent clinical features.
Treatment is by surgically removing the tumor.
Signs and symptoms
Swelling over the affected bone with or without pain are the most frequent clinical features. The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the tibia or fibula).
Metastases are rare at presentation but may occur in up to 30% of people during the disease course. Prognosis is excellent, with overall survival of 85% at 10 years, but is lower when wide surgical margins cannot be obtained. This tumor is insensitive to radiation so chemotherapy is not typically used unless the cancer has metastasized to the lungs or other organs.
The typically benign odontogenic tumor known as ameloblastoma was first recognized in 1827 by Cusack but did not yet have any designation. In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by Malassez and was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill. Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas, although they differ in histology and frequency of malignancy.
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