Adamantinoma
Encyclopedia
Adamantinoma is a rare bone cancer
Bone tumor
A bone tumor refers to a neoplastic growth of tissue in bone. Abnormal growths found in the bone can be either benign or malignant .-Classification:...

, making up less than 1% of all bone cancers.

It includes epithelial components.

The condition was first described by Fischer in 1913.

Presentation

It predominantly arises in bone in a subcutaneous location (most are in the tibia
Tibia
The tibia , shinbone, or shankbone is the larger and stronger of the two bones in the leg below the knee in vertebrates , and connects the knee with the ankle bones....

). Most commonly, patients are in their second or third decade, but it can occur over a wide age range.

Histologically, islands of epithelial cells are found in a fibrous stroma
Stroma (animal tissue)
In animal tissue, stroma refers to the connective, supportive framework of a biological cell, tissue, or organ.The stroma in animal tissue is contrasted with the parenchyma.Examples include:* Stroma of iris...

.

Treatment

Treatment consists of wide resection or amputation
Amputation
Amputation is the removal of a body extremity by trauma, prolonged constriction, or surgery. As a surgical measure, it is used to control pain or a disease process in the affected limb, such as malignancy or gangrene. In some cases, it is carried out on individuals as a preventative surgery for...

. 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.

Prior name for ameloblastoma

The typically benign odontogenic tumor known as ameloblastoma
Ameloblastoma
Ameloblastoma is a rare, benign tumor of odontogenic epithelium much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusack...

 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
Ameloblastoma
Ameloblastoma is a rare, benign tumor of odontogenic epithelium much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusack...

in 1930 by Ivey and Churchill. Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas, however they differ in histology and frequency of malignancy.
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