Adamantinoma

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Adamantinoma
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Micrograph of an adamantinoma showing the biphasic histomorphology. H&E stain..

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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]
  • Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas, however they differ in histology and frequency of malignancy. The typically benign odontogenic tumor known as ameloblastoma was first recognized in 1827 by Cusack but did not yet have any designation.[2] In 1885, this kind of odontogenic neoplasm was designated as an adamantinoma by [3]and was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.[4][5]

Epidemiology and Demographics

Adamantinoma is a rare bone cancer, making up less than 1% of all bone cancers. 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[6][7] or a regressive phase of adamantinoma.[8]

Pathophysiology

Gross Pathology

The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones.

Microscopic Pathology

Islands of epithelial cells are found in a fibrous stroma.

Natural History, Complications, and 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).[9]

Diagnosis

X Ray

The tumor is typically well-demarcated, osteolytic and eccentric, with cystic zones resembling soap bubbles.[10]

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

References

  1. Fischer B. Uber ein primares Adamantinom der Tibia. 12. Frankfurt: Zeitschr. f. Path.; 1913:422-441.
  2. J.W. Cusack (1827). "Report of the amputations of the lower jaw". Dublin Hosp Rec. 4: 1–38. 
  3. L. Malassez (1885). "Sur Le role des debris epitheliaux papdentaires". Arch Physiol Norm Pathol. 5: 309–340 6:379–449. 
  4. R.H. Ivey, H.R. Churchill, (1930). "The need of a standardized surgical and pathological classification of tumors and anomalies of dental origin,". Am Assoc Dent Sch Trans. 7: 240–245. 
  5. Madhup, R; Kirti, S; Bhatt, M; Srivastava, M; Sudhir, S; Srivastava, A (Jan 2006). "Giant ameloblastoma of jaw successfully treated by radiotherapy". Oral Oncology Extra. 42 (1): 22–25. doi:10.1016/j.ooe.2005.08.004. 
  6. Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S (May 2006). "A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature". Tohoku J. Exp. Med. 209 (1): 53–9. PMID 16636523. doi:10.1620/tjem.209.53. 
  7. Springfield DS, Rosenberg AE, Mankin HJ, Mindell ER. (1994). "Relationship between osteofibrous dysplasia and adamantinoma.". Clin Orthop Relat Res. (309): 234–44. PMID 7994967. 
  8. 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. 
  9. Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (2008). "Adamantinoma: A clinicopathological review and update". Diagn Pathol. 3: 8. PMC 2276480Freely accessible. PMID 18279517. doi:10.1186/1746-1596-3-8. 
  10. 10.0 10.1 Ernest U. Conrad (2008). Orthopaedic Oncology: Diagnosis and Treatment. Thieme. pp. 143–145. 

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