Adamantinoma affects young adults usually over 20 years of age. It usually affects the tibia. It is a low grade malignant tumor. When it is a low grade bony tumor, you treat it by wide excision alone, because chemotherapy and radiation therapy will not work for low grade malignant bony tumors such as adamantinoma and parosteal osteosarcoma.
Chemotherapy and radiation therapy will not work with chondrosarcoma. Treatment of chondrosarcoma is wide resection. For the treatment of adamantinoma, parosteal osteosarcoma and chondrosarcoma, do a wide resection.
There is doubt that the adamantinoma tumor arises from the osteofibrous dysplasia (OFD). Osteofibrous dysplasia (OFD) occurs in children and is benign. The treatment of osteofibrous dysplasia is observation and not wide resection. Osteofibrous dysplasia (OFD) looks similar to fibrous dysplasia, but it has an osteoblastic rimming.
Presentation of Adamantinoma:
Usually is in pain for a long time.
There may be tenderness over the tibia or a mass.
There may be bowing of the tibia.
It occurs within the tibial diaphysis.
X-rays:
Classic with multiple lucent and sclerotic defects.
Soap bubble appearance.
No periosteal reaction.
Differential Diagnosis:
Chondromyxoid fibroma.
Fibrous dysplasia.
Osteofibrous dysplasia.
Pathology:
Adamantinoma is a biphasic tumor.
There will be a cell nest of epithelial cells in a fibrous stroma.
Keratin-positive.
Treatment:
Wide resection and reconstruction.
Lung metastasis occurs in about 25% of the patients.
Need to do patient follow-up for a long period of time.
In the exam, they may give you an x-ray of a tibia and the lesion is located in the diaphysis of the tibia. The slides may show epithelium-like tissue embedded in fibrous stroma.
What is the treatment?
The treatment of adamantinoma is wide resection.
