Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 1361

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

MUSCULOSKELETAL Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 4  |  Page : 543-544
Adamantinoma in the distal end of tibia


Department of Radiodiagnosis and Imaging, JJM Medical College, Davangere 577004 Karnataka, India

Click here for correspondence address and email
 

Keywords: Ameloblastoma/DI, Aneurysm, Bone Cysts/DI, Epitheloid Cells, Muskuloskeletal Diseases, Magnetic Resonance Imaging, Human, Male, Adolescent, Case Report,

How to cite this article:
Setty N H, Desai R S. Adamantinoma in the distal end of tibia. Indian J Radiol Imaging 2002;12:543-4

How to cite this URL:
Setty N H, Desai R S. Adamantinoma in the distal end of tibia. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Dec 4];12:543-4. Available from: https://www.ijri.org/text.asp?2002/12/4/543/28535
Adamantinoma of the long bones is an extremely rare, locally malignant tumor. Our recent experience, with a case of Adamantinoma in the distal end of left Tibia presented with few atypical radiological features, which prompted us to report this rare tumor.


   Case Report Top


A 17 year old boy presented with eight months history of a painful lump in the lower end of the left tibia. To start with, patient gave a history of blunt trauma to the left lower leg followed by painful swelling which progressively increased in size. He had consulted an Orthopaedician four months earlier who diagnosed the lesion to be a bone cyst by plain film radiography. The progression of symptoms prompted the patient to come to our hospital. Clinical examination revealed an irregular bony swelling with no ulceration or sinuses over the skin surface. He was further investigated radiographically with plain films and scan.

Plain radiography - anteroposterior and lateral view of the distal part of the left leg revealed a large expansile, centrally located lytic lesion in the distal diaphyseo-metaphyseal region of left tibia. There were thick irregular unevenly arranged trabeculations within the lesion. Disruption of the overlying tibial cortex was seen. Few small satellite lytic lesions with surrounding sclerosis of the cortex and medulla were seen just proximal to the large lytic lesion [Figure - 1]. The zone of transition was irregular and permeative in nature. Minimal periosteal reaction with Codman's triangle [Figure - 2] was seen proximal to the large lytic lesion. Surrounding the lesion there was soft tissue swelling with displacement of the fat planes. There was no pathological fracture. Rest of the tibia and fibula were normal. A differential diagnosis of aneurysmal bone cyst or Adamantinoma or fibrous dysplasia was made.

Non enhanced axial CT of the left leg was done for further evaluation which showed an inhomogeneous soft tissue mass in the distal diaphysen-metaphyseal region with destruction of the overlying tibial cortex [Figure - 3]. There was no cavity or fluid levels within the lesion. Hence the possibility of Aneurysmal bone cyst was ruled out. As there were no specific radiological features to differentiate between adamantinoma and fibrous dysplasia, the lesion was open biopsied. Histopathology showed nests and bands of epithelial cells. Typical peripheral palisading of the basaloid-appearing cells with a microcystic center containing stellate-shaped tumor cells was less. All these histopathology features were of classical adamantinoma. Screening for secondaries was performed by plain film radiography, which was negative. Other laboratory investigations were normal. The patient subsequently underwent below knee amputation.


   Discussion Top


Adamantinoma or Angioblastoma is an extremely rare, locally aggressive or malignant tumor of the bone. The prevalence of this tumor is 0.1% of all primary bone tumors. Most occur in the second to fifth decade. These are slightly more common in men than women with a ratio of 5:4. Females with these tumors are between 11 and 30 years of age and males are between 30 and 50 years of age [1]. It can occur rarely in children. On histopathology, the classical type of adamantinoma with a low-grade malignant potential is usually seen in adults whereas the differentiated type with a benign course is seen in children [2]. A history of trauma is frequent and many affected persons described local swelling with or without pain as the major clinical symptoms. A striking predilection for the long bones (97 percent of cases) and, specifically, the tibia (80 to 85 percent cases) represents the most characteristic feature of this tumor. Other bones that are involved, in order of decreasing frequency, include the humerus, ulna, femur, fibula, radius and rarely innominate bones, ribs, spine and small bones of hand and foot [3]. Multifocal lesions may rarely develop in a single bone or two or more bones. Within a long tubular bone, diaphyseal localization predominates, although metaphyseal extension of lesions within the diaphysis or isolated involvement of a metaphysic is seen occasionally. In our patient metaphyseal extension was observed.

In the tibia, it is usually located in the middle third of the bone and appears as a central or eccentric, multilocular, slightly expansile, sharply or poorly delineated osteolytic lesion. Reactive bone sclerosis and small satellite radiolucent foci in direct continuity with the major lesion may be seen. Lesion can extend into the adjacent fibula [4]. Periostitis usually is not apparent in the absence of pathological fracture. Occasionally, cortical destruction, exuberant periostitis and a soft tissue mass are noted. Unusual abnormalities, including cortical violation with a large soft tissue component, extensive bone sclerosis and marked osseous expansion are possible. It may metastasize to lymph nodes and distant sites in a small percent of patients [4]. CT and MR imaging have been used to study the lesions of adamantinoma and usually the findings are not specific [5]. Although adamantinomas are rare tumors, an accurate radiographic diagnosis is possible when a typical tibial lesion is seen. Fibrous dysplasia, ossifying fibroma and aneurysmal bone cyst or simple bone cyst are the possible differential diagnosis.

The initial treatment should consist of wide excision or amputation and patients should undergo long-term follow-up for the development of recurrent or metastatic disease [6].

In our patient, there were no clinical or radiological features that allowed us to differentiate between adamantinoma from fibrous dysplasia or ossifying fibroma. The radiological signs suggestive of fibrous dysplasia such as ground glass appearance, anterior bowing or pseudoarthrosis of tibia were absent. They were excluded only by histopathology. However aneurysmal bone cyst was excluded by CT scan, as there were no cavities or fluid levels within the tumor

The unusual radiological features in this rare tumor are large expansile multilocular lesion with thick irregular unevenly arranged trabeculations, cortical bone destruction, minimal periosteal reaction and tumor extension into metaphysic. The usual features such as reactive sclerosis and satellite lytic lesion, in continuity with the major lesion were also present. Extensive review of literature did not reveal all these unique features of adamantinoma in one patient.



 
   References Top

1.Moon NF, Mori H: Adamantinoma of the appendicular skeleton - updated. Clin Orthop 1986, 204:215.  Back to cited text no. 1    
2.Kumar D, Mulligan ME, Levine AM, Dorfman HD: Classic adamantinoma in a 3-year-old. Skeletal Radiol 1998, July;27 (7): 406-9.  Back to cited text no. 2    
3.Keeney GL, Unni KK, Beabout JW, et al : Adamantinoma of long bones. A clinicopathologic study of 85 cases. Cancer 1989, 64:730.  Back to cited text no. 3    
4.Letourneau JG, Day DL, Crass JR, et al : Adamantinoma involving the distal tibia and fibula AJR 1987, 148:1049.  Back to cited text no. 4    
5.Young JWR, Aisner SC, Resnik CS, et al : Case report 660. Skeletal Radio 1991, 120:152.  Back to cited text no. 5    
6.Bloem JL, van der Heul RO, Schuttevaer HM, et al : Fibrous dysplasia vs. adamantinoma of the tibia: Differentiation based on discriminant analysis of clinical and plain film findings. AJR 1991, 156:1017.  Back to cited text no. 6    

Top
Correspondence Address:
N HA Setty
Department of Radiodiagnosis and Imaging, JJM Medical College, Davangere 577004 Karnataka, India

Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions


    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

This article has been cited by
1 Adamantinoma in childhood: Report of six cases and review of the literature
Van Rijn, R., Bras, J., Schaap, G., van den Berg, H., Maas, M.
Pediatric Radiology. 2006; 36(10): 1068-1074
[Pubmed]
2 Radiological quiz - Musculoskeletal
Shah, D.M., Patel, S.B., Goswami, K.G., Shah, N., Gohli, Y.
Indian J Radiol Imaging. 2004; 14(3): 329-330
[Pubmed]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Case Report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed4632    
    Printed133    
    Emailed1    
    PDF Downloaded0    
    Comments [Add]    
    Cited by others 2    

Recommend this journal