Adamantinoma

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Adamantinoma
Adamantinoma1.jpg
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]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2] [3] [4] [5]

Synonyms and Keywords:

Overview

  • Adamantinoma was first discovered by Fischer in 1913. Adamantinoma is classified into 2 distinct types: classic and differentiated (Osteofibrous dysplasia (OFD) - like). Adamantinoma is a low grade, malignant bone tumor. This tumor is predominantly located in tibia ( most in mid-portion of tibia). Most locations of the tumor include tibia, ipsilateral fibula, humerus, ulna, femur, fibula. It is a Yellow gray or grayish white tumor. In microscopic examination admixture of both epithelial and osteofibrous component can be seen. Adamantinoma may be caused by displacement of basal epithelium of skin during embryogenesis, traumatic implantation. Adamantinoma is a rare bone cancer ( 0.1–0.5% of all primary bone tumors ). Risk factors in the development of adamantinoma may include benign osteofibrous dysplasia. If left untreated, 15-30% of patients with adamantinoma may metastasize to other parts of the body such as lungs or nearby lymph nodes. The diagnosis of adamantinoma is based on the findings of radiologic studies such as x-ray, CT, and MRI. The most common symptoms of adamantinoma include swelling with or without pain. Less common symptoms of adamantinoma include pathological fracture. Adamantinoma may present with bowing deformity of the tibia. There is no medical therapy for adamantinoma. Radiation therapy and chemotherapy are not effective. Surgery is the mainstay of treatment for adamantinoma.

Historical Perspective

Adamantinoma was first discovered by Fischer in 1913.[1]

Classification

Adamantinoma is classified into 2 distinct types: classic and differentiated (Osteofibrous dysplasia (OFD) - like)

Features
Classic Differentiated
Age >20 years <20 years, Children
Radiology Soft tissue or intramedullary involvement Intra cortical
Histopathology Both epithelial and osteofibrous component, solid nests of basaloid cells OFD (Osteofibrous dysplasia) like pattern, Scattered positivity of epithelial elements for cytokeratin
Behavior Aggressive Relatively benign

Pathophysiology

Adamantinoma is a low grade, malignant bone tumor. This tumor is predominnatly located in tibia ( most in mid-portion of tibia). [2] It is a biphasic tumor including epithelial and osteofibrous components.[3].

Most locations of the tumor include:[4]

Gross pathology: [5]
  • Yellow gray or grayish white tumor
  • Fleshy or firm in consistency
  • Some OFD like adamantinomas are solid
  • Macroscopic cysts containing blood like fluid, occasionally
Microscopic examanination: [6]
  • Admixture of both epithelial and osteofibrous component, most commonly solid nests of basaloid cells in classic adamantinoma
  • Nuclear atypia in a few cases
  • Several patterns of growth including
    • Tubular
    • Basaloid
    • Squamous
    • Spindle-cell
    • Osteofibrous dysplasia-like variant
Immunohistochemistry:

Causes

Adamantinoma may be caused by: [10]

  • Displacement of basal epithelium of skin during embrogenesis
  • Traumatic implantation
  • Synovial origin

Differentiating Adamantinoma from Other Diseases

Adamantinoma must be differentiated from aneurrysmal bone cyst, unicameral bone cyst, fibrous dysplasia, chondromyxoid fibroma, eosinophilic granuloma, giant cell tumor, chondromyxoid fibroma, osteomyelitis, chondrosarcoma, epithelial metastasis, hemangioendothelioma, nonossifying fibromas, angiosarcoma.

Epidemiology and Demographics

Adamantinoma is a rare bone cnacer ( 0.1–0.5% of all primary bone tumors ).[11]

The prevalence of Adamantinoma is approximately 0.11 per 100000 individuals in Europe.

The incidence of Adamantinoma was estimated to be less than 300 cases worldwide.

Patients of all age groups may develop adamantinoma. The median age at diagnosis is 25 to 35 years.

Men are more commonly affected by adamantinoma than women. The men to women ratio is approximately 5 to 4.[12]

Risk Factors

Risk factors in the development of adamantinoma may include benign osteofibrous dysplasia. It maybe a precursor of adamantinoma.[13]

Screening

There is insufficient evidence to recommend routine screening for adamantinoma.

Natural History, Complications, and Prognosis

If left untreated, 15-30% of patients with adamantinoma may metastasize to other parts of the body such as lungs or nearby lymph nodes. [14]. Adamantinoma has mortality rate of 13%to 18%. [15]

Complications of adamantinoma include metastases to the lungs.

Prognosis is generally good. Male patint, short duration of symtoms, young age ( less than 20 years) and lack of squamous differentiantion of tumor are related with unfavorable clinical outcome. [16]

Diagnosis

Diagnostic Study of Choice

The diagnosis of adamantinoma is based on the findings of radiologic studies such as x-ray, CT, and MRI.

History and Symptoms

The initial symptoms of adamantinoma are often nonspecific. The most common symptoms of adamantinoma include swelling with or without pain. Less common symptoms of adamantinoma include pathological fracture( 23% of patients) [17] and neurological symptoms in spinal lesions.[18]

Physical Examination

Adamantinoma may present with bowing deformity of the tibia.[19]

Spinal lesions may be manifested by neurologic symptoms in addition to pain.

Laboratory Findings

Paraneoplastic hypercalcemia can be seen in tibial adamantinoma and pulmonary metastasis[20]

Electrocardiogram

There are no ECG findings associated with adamantinoma.

X-ray

An x-ray may be helpful in the diagnosis of adamantinoma. Findings on an x-ray suggestive of adamantinoma include central or eccentric, multilocular lesion in tibia. The tumor is found in the diaphyseal location. Metaphyseal extention or only involvement of metaphysis is seen occationally. The lesions are well circumscribed surrounded ring-shaped densities ( soap -bubble appearance). The lesion are more intra-cortical, but if they destroy cortex, extracortical soft tisuue invasion can be seen. The periosteal reaction can be minimal to prominnet.[21]

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with adamantinoma.

CT scan

CT scan may be helpful in the diagnosis of adamantinoma. Findings on CT scan suggestive of adamantinoma include cortical involvement and the soft tissue extension if it exist.

Chest CT scan can detect pulmonary metastasis. [22]

MRI

MRI is helpful in the diagnosis of adamantinoma. It is useful in detection distant cortical foci, soft tissue, and intramedullary extension. MRI is a very important diagnostic study for stating of tumor and tumor-free margins. Findings on MRI suggestive of adamantinoma include a solitary lobulated focus or multiple small nodules in one or more foci. Tumors demonstrate low signal intensity on T1-weighted images and high signal on T2-weighted images.[23]

Other Imaging Findings

Bone scan may be helpful in the diagnosis of adamantinoma. Findings on a nuclear medicine suggestive of adamantioma include:[24]

  • Increased blood flow in the region of the tumor
  • Increased accumulation of technetium-99m methylene diphosphate in the area of the tumor

Other Diagnostic Studies

There are no other diagnostic studies associated with adamantinoma.

Treatment

Medical Therapy

There is no medical therapy for adamantinoma. Radition therapy and chemotherapy are not effective. [25]

Surgery

Surgery is the mainstay of treatment for adamantinoma.

Primary Prevention

There are no established measures for the primary prevention of adamantinoma.

Secondary Prevention

There are no established measures for the secondary prevention of adamantinoma.

References

  1. Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. PMC 2276480Freely accessible. PMID 18279517. doi:10.1186/1746-1596-3-8. 
  2. Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266. 
  3. Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M (October 2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatr Radiol. 36 (10): 1068–74. PMID 16906392. doi:10.1007/s00247-006-0272-5. 
  4. Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266. 
  5. Unni KK, Dahlin DC, Beabout JW, Ivins JC (November 1974). "Adamantinomas of long bones". Cancer. 34 (5): 1796–805. PMID 4426036. 
  6. Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266. 
  7. Donner R, Dikland R (February 1966). "Adamantinoma of the tibia. A long-standing case with unusual histological features". J Bone Joint Surg Br. 48 (1): 138–44. PMID 5909059. 
  8. Weiss SW, Dorfman HD (March 1977). "Adamantinoma of long bone. An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes". Hum. Pathol. 8 (2): 141–53. PMID 852865. 
  9. Maki M, Saitoh K, Kaneko Y, Fukayama M, Morohoshi T (October 2000). "Expression of cytokeratin 1, 5, 14, 19 and transforming growth factors-beta1, beta2, beta3 in osteofibrous dysplasia and adamantinoma: A possible association of transforming growth factor-beta with basal cell phenotype promotion". Pathol. Int. 50 (10): 801–7. PMID 11107052. 
  10. Ryrie BJ (December 1932). "ADAMANTINOMA OF THE TIBIA: AETIOLOGY AND PATHOGENESIS". Br Med J. 2 (3752): 1000–1020.1. PMC 2522231Freely accessible. PMID 20777206. 
  11. Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. PMC 2276480Freely accessible. PMID 18279517. doi:10.1186/1746-1596-3-8. 
  12. Moon NF, Mori H (March 1986). "Adamantinoma of the appendicular skeleton--updated". Clin. Orthop. Relat. Res. (204): 215–37. PMID 3514033. 
  13. 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. 
  14. Moon NF, Mori H (March 1986). "Adamantinoma of the appendicular skeleton--updated". Clin. Orthop. Relat. Res. (204): 215–37. PMID 3514033. 
  15. Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266. 
  16. Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. PMC 2276480Freely accessible. PMID 18279517. doi:10.1186/1746-1596-3-8. 
  17. Qureshi AA, Shott S, Mallin BA, Gitelis S (August 2000). "Current trends in the management of adamantinoma of long bones. An international study". J Bone Joint Surg Am. 82–A (8): 1122–31. PMID 10954102. 
  18. Dini LI, Mendonça R, Adamy CA, Saraiva GA (August 2006). "Adamantinoma of the spine: case report". Neurosurgery. 59 (2): E426; discussion E426. PMID 16883154. doi:10.1227/01.NEU.0000223497.06588.4A. 
  19. Qureshi AA, Shott S, Mallin BA, Gitelis S (August 2000). "Current trends in the management of adamantinoma of long bones. An international study". J Bone Joint Surg Am. 82–A (8): 1122–31. PMID 10954102. 
  20. Altmannsberger M, Poppe H, Schauer A (1982). "An unusual case of adamantinoma of long bones". J. Cancer Res. Clin. Oncol. 104 (3): 315–20. PMID 7161313. 
  21. Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M (October 2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatr Radiol. 36 (10): 1068–74. PMID 16906392. doi:10.1007/s00247-006-0272-5. 
  22. Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M (October 2006). "Adamantinoma in childhood: report of six cases and review of the literature". Pediatr Radiol. 36 (10): 1068–74. PMID 16906392. doi:10.1007/s00247-006-0272-5. 
  23. Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC (December 2004). "MRI of adamantinoma of long bones in correlation with histopathology". AJR Am J Roentgenol. 183 (6): 1737–44. PMID 15547221. doi:10.2214/ajr.183.6.01831737. 
  24. Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. PMC 2276480Freely accessible. PMID 18279517. doi:10.1186/1746-1596-3-8. 
  25. Maki M, Saitoh K, Kaneko Y, Fukayama M, Morohoshi T (October 2000). "Expression of cytokeratin 1, 5, 14, 19 and transforming growth factors-beta1, beta2, beta3 in osteofibrous dysplasia and adamantinoma: A possible association of transforming growth factor-beta with basal cell phenotype promotion". Pathol. Int. 50 (10): 801–7. PMID 11107052. 
  26. Keeney GL, Unni KK, Beabout JW, Pritchard DJ (August 1989). "Adamantinoma of long bones. A clinicopathologic study of 85 cases". Cancer. 64 (3): 730–7. PMID 2743266. 
  27. Khan MH, Darji R, Rao U, McGough R (July 2006). "Leg pain and swelling in a 22-year-old man". Clin. Orthop. Relat. Res. 448: 259–66. PMID 16826129. doi:10.1097/01.blo.0000195924.36103.11. 



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