An adamantinoma of the long bones (not to be confused with an adamantinoma of the jaw) is a rare primary malignant bony tumour.
Typically presents in the 2nd to 3rd decades as a locally aggressive mass 3-15 cm in diameter. There is be a slight male predilection (1.3:1). Patients usually present with dull pain of gradual onset.
Occurrence almost exclusively confined to the tibial diaphysis (especially anterior cortex) 2,5. Since it is a low-grade malignancy, it has the propensity to metastasise to distant locations including : lung, bone, lymph nodes, pericardium and liver.
Plain film and CT
Typically, it appears as a multi-locular or slightly expansile osteolytic lesion. This may be visualised as areas of lysis interspersed with areas of sclerosis 3. Lesions tend to have an eccentric epicenter 4 and a lack of periosteal reaction . There may be locally aggressive disease at presentation.
Some authors have distinguished two morphologic patterns 2
- a solitary lobulated focus
- multiple small nodules in one or more foci.
Separated tumour foci, defined as foci of high signal intensity on either T2 or T1-weighted contrast-enhanced images, interspersed with normal-appearing cortical or spongious bone many be seen in some patients 2.
- C+(Gd) : tends to show intense and homogeneous static enhancement, although there is no uniform dynamic enhancement pattern 2
Treatment and prognosis
Surgical resection with en-bloc resection is often performed. It is a locally aggressive tumour with distant metastases reported in around 15% of cases 1.
It was first observed in the shaft of an ulna in 1900 by C Maier 8, who believed it was a carcinoma although the actual name adamantinoma was given by B Fisher in 1913 9.
Imaging differential considerations include 6
- 1. Kanakaraddi SV, Nagaraj G, Ravinath TM. Adamantinoma of the tibia with late skeletal metastasis: an unusual presentation. J Bone Joint Surg Br. 2007;89 (3): 388-9. doi:10.1302/0301-620X.89B3.18484 - Pubmed citation
- 2. Van der woude HJ, Hazelbag HM, Bloem JL et-al. MRI of adamantinoma of long bones in correlation with histopathology. AJR Am J Roentgenol. 2004;183 (6): 1737-44. AJR Am J Roentgenol (full text) - Pubmed citation
- 3. Day DL, Letourneau JG, Crass JR et-al. Musculoskeletal case of the day. AJR Am J Roentgenol. 1987;148 (5): 1048-52. AJR Am J Roentgenol (citation) - Pubmed citation
- 4. Levine SM, Lambiase RE, Petchprapa CN. Cortical lesions of the tibia: characteristic appearances at conventional radiography. Radiographics. 23 (1): 157-77. doi:10.1148/rg.231015088 - Pubmed citation
- 5. Miller TT. Bone tumors and tumorlike conditions: analysis with conventional radiography. Radiology. 2008;246 (3): 662-74. doi:10.1148/radiol.2463061038 - Pubmed citation
- 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 Am J Roentgenol. 1991;156 (5): 1017-23. AJR Am J Roentgenol (abstract) - Pubmed citation
- 7. Camp MD, Tompkins RK, Spanier SS et-al. Best cases from the AFIP: Adamantinoma of the tibia and fibula with cytogenetic analysis. Radiographics. 28 (4): 1215-20. doi:10.1148/rg.284075173 - Pubmed citation
- 8. Maier C. Ein primers myelogenes platten-epithelkarzinom der ulna. Beitraege zur klinischen Chirurgie 1900;26 : 553-566.
- 9. Fischer B. Uber ein primares adamantinoma der tibia. Virchows Arch [B] 1913;12:422–441