Adamantinoma
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| Adamantinoma | |
|---|---|
 | |
| Micrograph (using H&E stain) of an adamantinoma showing the biphasic histomorphology. | |
| Specialty | Oncology, rheumatology  |
Adamantinoma (from Greek adamantinos 'very hard'[1]) is a rare bone cancer, making up less than 1% of all bone cancers.[2] It almost always occurs in the bones of the lower leg[3] and involves both epithelial and osteofibrous tissue.[4]
Patients typically present with swelling with or without pain. The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the tibia or fibula).[3]
Benign osteofibrous dysplasia may be a precursor of adamantinoma[4][7] or a regressive phase of adamantinoma.[8]
Histologically, islands of epithelial cells are found in a fibrous stroma. The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles.[2]
Diagnosis
Treatment
Treatment consists of wide resection or amputation. Metastases are rare at presentation but may occur in up to 30% of patients 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.[2]