Adamantinoma

A rare bone tumor

Classification
External resources

Adamantinoma is a rare, low-grade malignant bone tumor that primarily affects the tibia. It is characterized by its slow growth and potential to metastasize, particularly to the lungs.

Epidemiology

Adamantinoma accounts for less than 1% of all primary bone tumors. It most commonly occurs in young adults, with a slight male predominance. The typical age of onset is between 20 and 40 years.

Pathophysiology

The exact cause of adamantinoma is unknown. Histologically, it is characterized by epithelial-like cells within a fibrous stroma. The tumor is thought to arise from remnants of the epithelial cells in the periosteum or from mesenchymal cells that have undergone epithelial differentiation.

Clinical Presentation

Patients with adamantinoma typically present with a long history of pain and swelling in the affected limb. The tibia is the most common site, but it can also occur in the fibula and other bones. The pain is often insidious and may be associated with a palpable mass.

Diagnosis

Diagnosis of adamantinoma is based on a combination of clinical, radiological, and histological findings.

Imaging

X-ray imaging typically shows a well-defined, lytic lesion in the diaphysis of the tibia with cortical involvement. Advanced imaging techniques such as MRI and CT scan can further delineate the extent of the tumor and its relationship to surrounding structures.

Histology

Histological examination reveals nests and cords of epithelial cells within a fibrous stroma. Immunohistochemical staining is often used to confirm the diagnosis.

Treatment

The primary treatment for adamantinoma is surgical resection. Wide local excision with clear margins is the treatment of choice to minimize the risk of recurrence. In some cases, limb-sparing surgery or amputation may be necessary.

Prognosis

The prognosis for patients with adamantinoma is generally favorable if the tumor is completely resected. However, local recurrence and metastasis can occur, necessitating long-term follow-up.

Related pages

• Bone tumor
• Tibia
• Metastasis
• Histology

Gallery

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  Histological image of adamantinoma

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  X-ray showing adamantinoma of the tibia

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  Adamantinoma

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  X-ray of adamantinoma of the tibia