Adamantinoma () is a rare

bone cancer A bone tumor is an neoplastic, abnormal growth of tissue in bone, traditionally classified as benign, noncancerous (benign) or malignant, cancerous (malignant). Cancerous bone tumors usually originate from a cancer in another part of the body su ...

, making up less than 1% of all bone cancers. It almost always occurs in the bones of the lower leg and involves both

epithelial Epithelium or epithelial tissue is a thin, continuous, protective layer of cells with little extracellular matrix. An example is the epidermis, the outermost layer of the skin. Epithelial ( mesothelial) tissues line the outer surfaces of man ...

and osteofibrous tissue. The condition was first described by Fischer in 1913.

Presentation

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 The tibia (; : tibiae or tibias), also known as the shinbone or shankbone, is the larger, stronger, and anterior (frontal) of the two Leg bones, bones in the leg below the knee in vertebrates (the other being the fibula, behind and to the outsi ...

fibula The fibula (: fibulae or fibulas) or calf bone is a leg bone on the lateral side of the tibia, to which it is connected above and below. It is the smaller of the two bones and, in proportion to its length, the most slender of all the long bones. ...

). Benign osteofibrous dysplasia may be a precursor of adamantinoma or a regressive phase of adamantinoma. 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.

Diagnosis

X-rays An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...

of the affected area show a well defined tumour in bone, with multiple lobules giving a "soap bubble" appearance.

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...

can provide a more useful guide to its severity.

Treatment

Treatment consists of wide resection or

amputation Amputation is the removal of a Limb (anatomy), limb or other body part by Physical trauma, trauma, medical illness, or surgery. As a surgical measure, it is used to control pain or a disease process in the affected limb, such as cancer, malign ...

. 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 Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

is not typically used unless the cancer has metastasized to the lungs or other organs.

History

The typically benign odontogenic tumor known as

ameloblastoma Ameloblastoma is a rare, benign or cancerous tumor of odontogenic epithelium ( ameloblasts, or outside portion, of the teeth during development) much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusa ...

was first recognized in 1827 by Cusack. Still, it did not yet have any designation. In 1885, this kind of odontogenic neoplasm was designated as an ''adamantinoma'' by Malassez. It was finally renamed to the modern name ''

'' in 1930 by Ivey and Churchill.

References

External links

{{Osseous and chondromatous tumors Rare cancers Osseous and chondromatous neoplasia