Adamantinoma
| Adamantinoma | |
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| Micrograph of an adamantinoma showing the biphasic histomorphology. H&E stain.. | |
| ICD-O: | 9310/0 |
| DiseasesDB | 31676 |
| MeSH | D050398 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Adamantinoma is a rare bone cancer, making up less than 1% of all bone cancers. It predominantly arises in bone in a subcutaneous location (85% are in the tibia). Most commonly, patients are in their second or third decade, but it can occur over a wide age range.
Historical Perspective
The condition was first described by Fischer in 1913.[1][2]
Prior name for ameloblastoma
The typically benign odontogenic tumor known as ameloblastoma was first recognized in 1827 by Cusack but did not yet have any designation.[3] In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by Malassez[4] and was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.[5][6] Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas, however they differ in histology and frequency of malignancy.
Epidemiology and Demographics
Most commonly, patients are in their second or third decade, but adamantinoma can occur over a wide age range.
Risk Factors
Benign osteofibrous dysplasia may be a precursor of adamantinoma[7][8] or a regressive phase of adamantinoma.[9]
Pathophysiology
Gross Pathology
The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles.[10]
Microscopic Pathology
Islands of epithelial cells are found in a fibrous stroma.
Natural History,Complications,Prognosis
Complications
Metastases are rare at presentation but may occur in up to 30% of patients during the disease course.
Prognosis
Prognosis is excellent, with overall survival of 85% at 10 years, but is lower when wide surgical margins cannot be obtained.
History and Symptoms
Patients typically present with swelling in long bones with or without pain.
Physical Examination
Extremities
The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the tibia or fibula).[11]
Treatment
Treatment consists of wide resection or amputation.This tumor is insensitive to radiation so chemotherapy is not typically used unless the cancer has metastized to the lungs or other organs.[10]
See also
- ameloblastoma
- osteofibrous dysplasia
References
- ↑
- ↑ Fischer B. Uber ein primares Adamantinom der Tibia. 12. Frankfurt: Zeitschr. f. Path.; 1913:422-441.
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- ↑ Springfield DS, Rosenberg AE, Mankin HJ, Mindell ER. (1994). "Relationship between osteofibrous dysplasia and adamantinoma". Clin Orthop Relat Res. (309): 234–44. PMID 7994967.
- ↑ Gleason, Briana C., Liegl-Atzwanger, Bernadette. "Osteofibrous Dysplasia and Adamantinoma in Children and Adolescents: A Clinicopathologic Reappraisal". American Journal of Surgical Pathology. 32 (3): 363–376. doi:10.1097/PAS.0b013e318150d53e.
- ↑ 10.0 10.1
- ↑
- Campbell's Operative Orthopedics (10th edition ed.). 2003.