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{{Infobox Disease
 
| Name          = Adamantinoma
 
| Image          = Adamantinoma1.jpg
| Caption        = [[Micrograph]] of an adamantinoma showing the biphasic histomorphology. [[H&E stain]]..
| DiseasesDB    = 31676
| ICD10          =
| ICD9          =
| ICDO          = 9310/0
| OMIM          =
| MedlinePlus    =
| eMedicineSubj  =
| eMedicineTopic =
| MeshID        = D050398
}}
{{SI}}
{{SI}}
{{CMG}}
 
{{CMG}}; {{AE}}{{MA}} [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu] [mailto:malihash@bidmc.harvard.edu]
 
{{SK}}  


==Overview==
==Overview==
'''Adamantinoma''' is a rare [[bone tumor|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.
* 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 [[Epithelium|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]] [[Tumor|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 node|lymph nodes]]. The diagnosis of adamantinoma is based on the findings of radiologic studies such as [[x-ray]], CT, and [[Magnetic resonance imaging|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==
==Historical Perspective==
The condition was first described by Fischer in 1913.<ref name="urlAdamantinoma: Overview - eMedicine"/><ref>Fischer B. Uber ein primares Adamantinom der Tibia. 12. Frankfurt: Zeitschr. f. Path.; 1913:422-441.</ref>
Adamantinoma was first discovered by Fischer in 1913.<ref name="pmid182795172">{{cite journal |vauthors=Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y |title=Adamantinoma: a clinicopathological review and update |journal=Diagn Pathol |volume=3 |issue= |pages=8 |date=February 2008 |pmid=18279517 |pmc=2276480 |doi=10.1186/1746-1596-3-8 |url=}}</ref>
===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.<ref name="Cusack"/> In 1885, this kind of odontogenic neoplasm was designated as an ''adamantinoma'' by Malassez<ref name="Malassez"/> and was finally renamed to the modern name ''[[ameloblastoma]]'' in 1930 by Ivey and Churchill.<ref name="Ivey"/><ref name="Madhup"/> Some authors still confusingly misuse the term adamantinoma to describe ameloblastomas, however they differ in histology and frequency of malignancy.
==Classification==
 
 
Adamantinoma is classified into 2 distinct types: classic and differentiated (Osteofibrous dysplasia (OFD) - like)
 
{| class="wikitable"
|+
!
{| class="wikitable"
| colspan="1" rowspan="1" |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 predominatly located in tibia ( most in mid-portion of tibia). <ref name="pmid2743266">{{cite journal |vauthors=Keeney GL, Unni KK, Beabout JW, Pritchard DJ |title=Adamantinoma of long bones. A clinicopathologic study of 85 cases |journal=Cancer |volume=64 |issue=3 |pages=730–7 |date=August 1989 |pmid=2743266 |doi= |url=}}</ref> It is a biphasic tumor including [[Epithelium|epithelial]] and osteofibrous components.<ref name="pmid16906392">{{cite journal |vauthors=Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M |title=Adamantinoma in childhood: report of six cases and review of the literature |journal=Pediatr Radiol |volume=36 |issue=10 |pages=1068–74 |date=October 2006 |pmid=16906392 |doi=10.1007/s00247-006-0272-5 |url=}}</ref>.  
 
Most locations of the tumor include:<ref name="pmid27432662">{{cite journal |vauthors=Keeney GL, Unni KK, Beabout JW, Pritchard DJ |title=Adamantinoma of long bones. A clinicopathologic study of 85 cases |journal=Cancer |volume=64 |issue=3 |pages=730–7 |date=August 1989 |pmid=2743266 |doi= |url=}}</ref>
* [[Tibia]] ( 80 to 85 percent of cases)
* Ipsilateral [[fibula]] (10 to15% of cases)
* [[Humerus]]
* [[Ulna]]
* [[Femur]]
* [[Fibula]]
* [[Radius]]
* [[Rib|Ribs]]
* [[Spine]]
* Small bones of the [[hand]] and [[foot]]
 
====== Gross pathology: <ref name="pmid4426036">{{cite journal |vauthors=Unni KK, Dahlin DC, Beabout JW, Ivins JC |title=Adamantinomas of long bones |journal=Cancer |volume=34 |issue=5 |pages=1796–805 |date=November 1974 |pmid=4426036 |doi= |url=}}</ref> ======
* 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: <ref name="pmid27432663">{{cite journal |vauthors=Keeney GL, Unni KK, Beabout JW, Pritchard DJ |title=Adamantinoma of long bones. A clinicopathologic study of 85 cases |journal=Cancer |volume=64 |issue=3 |pages=730–7 |date=August 1989 |pmid=2743266 |doi= |url=}}</ref> ======
* Admixture of both [[Epithelium|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
 
* A few cases from a large series have exhibited [[nuclear]] [[atypia]]
* Foci of [[calcification]], [[giant cells]] <ref name="pmid5909059">{{cite journal |vauthors=Donner R, Dikland R |title=Adamantinoma of the tibia. A long-standing case with unusual histological features |journal=J Bone Joint Surg Br |volume=48 |issue=1 |pages=138–44 |date=February 1966 |pmid=5909059 |doi= |url=}}</ref>
* Foci of [[xanthoma]] and [[spindle cells]]<ref name="pmid852865">{{cite journal |vauthors=Weiss SW, Dorfman HD |title=Adamantinoma of long bone. An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes |journal=Hum. Pathol. |volume=8 |issue=2 |pages=141–53 |date=March 1977 |pmid=852865 |doi= |url=}}</ref>
 
====== Immunohistochemistry: ======
* Positives for [[keratins]] 14 and 19<ref name="pmid11107052">{{cite journal |vauthors=Maki M, Saitoh K, Kaneko Y, Fukayama M, Morohoshi T |title=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 |journal=Pathol. Int. |volume=50 |issue=10 |pages=801–7 |date=October 2000 |pmid=11107052 |doi= |url=}}</ref>
 
==Causes==
 
Adamantinoma may be caused by: <ref name="pmid20777206">{{cite journal |vauthors=Ryrie BJ |title=ADAMANTINOMA OF THE TIBIA: AETIOLOGY AND PATHOGENESIS |journal=Br Med J |volume=2 |issue=3752 |pages=1000–1020.1 |date=December 1932 |pmid=20777206 |pmc=2522231 |doi= |url=}}</ref>
* 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 of bone|giant cell tumor]], chondromyxoid fibroma, [[osteomyelitis]], [[chondrosarcoma]], epithelial [[metastasis]], [[hemangioendothelioma]], nonossifying fibromas, [[angiosarcoma]].  


==Epidemiology and Demographics==
==Epidemiology and Demographics==
Most commonly, patients are in their second or third decade, but adamantinoma can occur over a wide age range.
Adamantinoma is a rare bone cnacer ( 0.1–0.5% of all primary bone tumors ).<ref name="pmid182795177">{{cite journal |vauthors=Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y |title=Adamantinoma: a clinicopathological review and update |journal=Diagn Pathol |volume=3 |issue= |pages=8 |date=February 2008 |pmid=18279517 |pmc=2276480 |doi=10.1186/1746-1596-3-8 |url=}}</ref>
 
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.<ref name="pmid3514033">{{cite journal |vauthors=Moon NF, Mori H |title=Adamantinoma of the appendicular skeleton--updated |journal=Clin. Orthop. Relat. Res. |volume= |issue=204 |pages=215–37 |date=March 1986 |pmid=3514033 |doi= |url=}}</ref>


==Risk Factors==
==Risk Factors==
Benign osteofibrous dysplasia may be a precursor of adamantinoma<ref name="pmid16636523"/><ref>{{cite journal |url=http://www.ncbi.nlm.nih.gov/pubmed/7994967|title=Relationship between osteofibrous dysplasia and adamantinoma.| author=Springfield DS, Rosenberg AE, Mankin HJ, Mindell ER. |pmid=7994967 |journal=Clin Orthop Relat Res. |year=1994 |pages=234–44 |issue=309}}</ref> or a regressive phase of adamantinoma.<ref>{{cite journal |url=http://journals.lww.com/ajsp/Abstract/2008/03000/Osteofibrous_Dysplasia_and_Adamantinoma_in.3.aspx |title=Osteofibrous Dysplasia and Adamantinoma in Children and Adolescents: A Clinicopathologic Reappraisal |author=Gleason, Briana C., Liegl-Atzwanger, Bernadette |journal=American Journal of Surgical Pathology |issue=3 |volume=32 |pages=363–376 |doi= 10.1097/PAS.0b013e318150d53e}}</ref>


==Pathophysiology==
Risk factors in the development of adamantinoma may include benign [[osteofibrous dysplasia]]. It maybe a precursor of adamantinoma.<ref name="pmid166365232">{{cite journal |vauthors=Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S |title=A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature |journal=Tohoku J. Exp. Med. |volume=209 |issue=1 |pages=53–9 |date=May 2006 |pmid=16636523 |doi= |url=}}</ref>
===Gross Pathology===
 
The tumor is typically well-demarcated, [[Osteolysis|osteolytic]] and eccentric, with cystic zones resembling soap bubbles.<ref name="Radiation" />
==Screening==
===Microscopic Pathology===
There is insufficient evidence to recommend routine screening for adamantinoma.
Islands of epithelial cells are found in a fibrous stroma.
 
==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]]. <ref name="pmid35140332">{{cite journal |vauthors=Moon NF, Mori H |title=Adamantinoma of the appendicular skeleton--updated |journal=Clin. Orthop. Relat. Res. |volume= |issue=204 |pages=215–37 |date=March 1986 |pmid=3514033 |doi= |url=}}</ref>. Adamantinoma has mortality rate of 13%to 18%. <ref name="pmid27432665">{{cite journal |vauthors=Keeney GL, Unni KK, Beabout JW, Pritchard DJ |title=Adamantinoma of long bones. A clinicopathologic study of 85 cases |journal=Cancer |volume=64 |issue=3 |pages=730–7 |date=August 1989 |pmid=2743266 |doi= |url=}}</ref>
 
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 epithelium|squamous]] differentiantion of tumor are related with unfavorable clinical outcome. <ref name="pmid182795175">{{cite journal |vauthors=Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y |title=Adamantinoma: a clinicopathological review and update |journal=Diagn Pathol |volume=3 |issue= |pages=8 |date=February 2008 |pmid=18279517 |pmc=2276480 |doi=10.1186/1746-1596-3-8 |url=}}</ref>
 
==Diagnosis==
===Diagnostic Study of Choice===
 
The diagnosis of adamantinoma is based on the findings of radiologic studies such as [[X-rays|x-ray]], [[CT-scans|CT]], and [[Magnetic resonance imaging|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) <ref name="pmid10954102">{{cite journal |vauthors=Qureshi AA, Shott S, Mallin BA, Gitelis S |title=Current trends in the management of adamantinoma of long bones. An international study |journal=J Bone Joint Surg Am |volume=82-A |issue=8 |pages=1122–31 |date=August 2000 |pmid=10954102 |doi= |url=}}</ref> and neurological symptoms in spinal lesions.<ref name="pmid16883154">{{cite journal |vauthors=Dini LI, Mendonça R, Adamy CA, Saraiva GA |title=Adamantinoma of the spine: case report |journal=Neurosurgery |volume=59 |issue=2 |pages=E426; discussion E426 |date=August 2006 |pmid=16883154 |doi=10.1227/01.NEU.0000223497.06588.4A |url=}}</ref> 
 
===Physical Examination===
Adamantinoma may present with bowing deformity of the [[tibia]].<ref name="pmid109541022">{{cite journal |vauthors=Qureshi AA, Shott S, Mallin BA, Gitelis S |title=Current trends in the management of adamantinoma of long bones. An international study |journal=J Bone Joint Surg Am |volume=82-A |issue=8 |pages=1122–31 |date=August 2000 |pmid=10954102 |doi= |url=}}</ref>
 
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]]<ref name="pmid7161313">{{cite journal |vauthors=Altmannsberger M, Poppe H, Schauer A |title=An unusual case of adamantinoma of long bones |journal=J. Cancer Res. Clin. Oncol. |volume=104 |issue=3 |pages=315–20 |date=1982 |pmid=7161313 |doi= |url=}}</ref>
 
===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 Lesion|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.<ref name="pmid169063923">{{cite journal |vauthors=Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M |title=Adamantinoma in childhood: report of six cases and review of the literature |journal=Pediatr Radiol |volume=36 |issue=10 |pages=1068–74 |date=October 2006 |pmid=16906392 |doi=10.1007/s00247-006-0272-5 |url=}}</ref>
 
===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 bone|cortical]] involvement and the soft tissue extension if it exist. 
 
Chest CT scan can detect pulmonary [[metastasis]]. <ref name="pmid169063922">{{cite journal |vauthors=Van Rijn R, Bras J, Schaap G, van den Berg H, Maas M |title=Adamantinoma in childhood: report of six cases and review of the literature |journal=Pediatr Radiol |volume=36 |issue=10 |pages=1068–74 |date=October 2006 |pmid=16906392 |doi=10.1007/s00247-006-0272-5 |url=}}</ref>


==Natural History,Complications,Prognosis==
===MRI===
===Complications===
[[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.<ref name="pmid15547221">{{cite journal |vauthors=Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC |title=MRI of adamantinoma of long bones in correlation with histopathology |journal=AJR Am J Roentgenol |volume=183 |issue=6 |pages=1737–44 |date=December 2004 |pmid=15547221 |doi=10.2214/ajr.183.6.01831737 |url=}}</ref>
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==
===Other Imaging Findings===
Patients typically present with swelling in long bones with or without pain.
[[Bone scan]] may be helpful in the diagnosis of adamantinoma. Findings on a [[nuclear medicine]] suggestive of  adamantioma include:<ref name="pmid182795174">{{cite journal |vauthors=Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y |title=Adamantinoma: a clinicopathological review and update |journal=Diagn Pathol |volume=3 |issue= |pages=8 |date=February 2008 |pmid=18279517 |pmc=2276480 |doi=10.1186/1746-1596-3-8 |url=}}</ref>
* Increased blood flow in the region of the [[tumor]]
* Increased accumulation of [[technetium-99m]] methylene diphosphate in the area of the [[tumor]]


==Physical Examination==
===Other Diagnostic Studies===
===Extremities===
There are no other diagnostic studies associated with adamantinoma.
The slow-growing tumor predominantly arises in long bones in a subcortical location (95% in the [[tibia]] or [[fibula]]).<ref name="pmid18279517"/>


==Treatment==
==Treatment==
Treatment consists of wide resection or [[amputation]].This tumor is insensitive to radiation so [[chemotherapy]] is not typically used unless the cancer has [[Metastasis|metastized]] to the lungs or other organs.<ref name="Radiation" />  
===Medical Therapy===
There is no medical therapy for adamantinoma. Radition therapy and [[chemotherapy]] are not effective. <ref name="pmid111070522">{{cite journal |vauthors=Maki M, Saitoh K, Kaneko Y, Fukayama M, Morohoshi T |title=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 |journal=Pathol. Int. |volume=50 |issue=10 |pages=801–7 |date=October 2000 |pmid=11107052 |doi= |url=}}</ref>


==See also==
===Surgery===
*[[ameloblastoma]]
Surgery is the mainstay of treatment for adamantinoma.
*osteofibrous dysplasia
* Tumor [[resection]] with wide operative margins and then limb reconstruction <ref name="pmid27432664">{{cite journal |vauthors=Keeney GL, Unni KK, Beabout JW, Pritchard DJ |title=Adamantinoma of long bones. A clinicopathologic study of 85 cases |journal=Cancer |volume=64 |issue=3 |pages=730–7 |date=August 1989 |pmid=2743266 |doi= |url=}}</ref>
* [[Amputation]]<ref name="pmid16826129">{{cite journal |vauthors=Khan MH, Darji R, Rao U, McGough R |title=Leg pain and swelling in a 22-year-old man |journal=Clin. Orthop. Relat. Res. |volume=448 |issue= |pages=259–66 |date=July 2006 |pmid=16826129 |doi=10.1097/01.blo.0000195924.36103.11 |url=}}</ref>
 
===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==
==References==
{{reflist|2}}
{{reflist|2}}
*{{cite book|title = Campbell's Operative Orthopedics|edition=10th edition|year=2003}}
[[Category:Types of cancer]]
[[Category:Orthopedics]]
[[Category:Oncology]]
{{Epithelial neoplasms}}
[[Category:Types of cancer]]


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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] [6]

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 predominatly 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. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
  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. doi:10.1007/s00247-006-0272-5. PMID 16906392.
  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 2522231. 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. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
  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. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
  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. doi:10.1227/01.NEU.0000223497.06588.4A. PMID 16883154.
  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. doi:10.1007/s00247-006-0272-5. PMID 16906392.
  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. doi:10.1007/s00247-006-0272-5. PMID 16906392.
  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. doi:10.2214/ajr.183.6.01831737. PMID 15547221.
  24. ↑ Jain D, Jain VK, Vasishta RK, Ranjan P, Kumar Y (February 2008). "Adamantinoma: a clinicopathological review and update". Diagn Pathol. 3: 8. doi:10.1186/1746-1596-3-8. PMC 2276480. PMID 18279517.
  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. doi:10.1097/01.blo.0000195924.36103.11. PMID 16826129.


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