A 14-year-old female patient was referred to orthopaedics due to long-standing mild pain in the anterior surface of the middle third of the right tibia, with 3 years of evolution. In the past 4 months she noted a swelling in this location and an increase in pain intensity. There were no previous radiology studies.
The plain film (Fig. 1) reveals the presence of small cortical lytic lesions with poorly defined margins on the anterior surface of the tibia. CT (Fig. 1) demonstrates also the associated soft tissue component. There is no periosteal reaction.
On MRI the soft tissue component is better demonstrated. This lesion has a bone origin (anterior cortex of the tibial diaphysis) with cortical rupture and a soft tissue mass, which is hypointense on T1WI and hyperintense and heterogeneous on T2WI (Fig. 2). The lesion shows evident enhancement after contrast administration (Fig. 3).
No other lesions are seen in the remaining tibia.
Background:
Adamantinoma is a rare malignant neoplasm (0.1 to 0.4% of primary bone tumours) that occurs almost exclusively in the anterior cortex of the tibial shaft. There are cases described in other long bones (humerus, femur, fibula and radius). [1, 2]
This is a low-degree but locally aggressive neoplasia that is characterised by epithelial and osteofibrous components consisting of islands of epithelial cells within a fibrous stroma with peripheral palisading in fibrous stroma with haphazard osteoid deposition. Keratin staining is typically positive. [1, 2]
Clinical Perspective:
It typically presents in the 2nd and 3rd decades of life with a male predilection (M:F ratio of 1.3: 1) with gradual pain with or without a palpable mass. 25% have symptoms for 5 or more years.
This is a low-degree but locally aggressive neoplasia with a cortical origin. In about 15% of the cases there is cortical rupture and soft tissue mass. [1, 3]
Imaging Perspective:
Radiography and CT:
It usually presents as an elongated lytic cortical lesions with poorly defined margins interspersed with sclerotic areas. Lesions tend to have an eccentric epicentre and no aggressive periosteal reaction. [1, 3, 4]
MRI:
Single or multi-focal lesions (importance of MRI) hypointense on T1WI and hyperintense on T2WI. The post-contrast enhancement is variable but usually intense. There may be cortical rupture and soft tissue mass and in some cases liquid-liquid levels. [1]
Outcome:
Good prognosis.
Adamantinoma is a low degree of malignancy but locally aggressive. [1]
Metastatic disease in 15% of cases: lung, bone, liver and lymph nodes.
Treatment: block surgery. Does not respond to QT or RT.
Local recurrence: 8 - 32%. [1, 2]
Take Home Message, Teaching Points:
Diaphysial lesion of the tibia in a young patient - think about adamantinoma, even if it is a rare tumour.
Adamantinoma of the anterior tibial diaphysis
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Based on the patient’s provided X-ray and MRI images of the right lower leg, a localized lesion can be observed within the anterior cortical region of the mid-tibia:
No obvious large soft tissue mass is detected, but the tibial cortex shows some degree of destruction or recession. Overall, the findings suggest a focal lesion originating from the tibial cortex.
Based on the patient’s age, location of the lesion, and its radiological features, the following conditions are considered:
Considering the patient’s age (14), the lesion location (anterior cortex of the mid-tibia), imaging findings (lytic lesion with local sclerosis, low signal on T1, high signal on T2, enhancement on MRI, long clinical history, and progressively worsening pain), and the common histological feature of epithelial cell nests with positive cytokeratin staining, the most likely diagnosis is:
Adamantinoma.
To confirm the diagnosis, a biopsy or surgical pathology is required.
Adamantinoma is a low-grade malignant tumor with locally invasive characteristics. The primary treatment is complete surgical resection (en bloc or wide excision) to minimize the risk of recurrence. Chemotherapy or radiotherapy is generally not used unless in special circumstances where individualized treatment may be considered.
Special Note: Since the patient is an adolescent with bones still developing, postoperative monitoring of bone healing and potential recurrence is essential. Any new onset of pain or swelling should prompt immediate medical review.
Disclaimer: This report is intended as a reference for medical imaging and rehabilitation information and cannot replace in-person consultation or the diagnosis and treatment advised by a qualified physician. In case of any concerns or sudden changes, please seek specialist advice or visit a hospital promptly.
Adamantinoma of the anterior tibial diaphysis