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Adamantinoma
Adamantinoma is a rare, low‑grade malignant bone tumor that most commonly arises in the tibia of young adults and presents with localized pain and a slowly enlarging mass; it is locally aggressive with potential for late recurrence and distant metastasis
Incidence
~0.1–0.3 cases per million
per year
US Estimated and Europe Estimated
Age of Onset
Children - Adult
ICD-10
C40.2
C41.9
Inheritance Pattern
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
Rare View
A rare, low-grade malignant bone tumor that primarily affects the tibia, presenting with pain and swelling. It has a slow-growing nature but can metastasize, typically requiring surgical resection for treatment.
5 Facts you should know
FACT
The name comes from the Greek word adamantinos, meaning "very hard"
FACT
Adamantinoma makes up less than 1% of all bone cancers
FACT
It almost always occurs in the bones of the lower leg
FACT
Involves both epithelial and osteofibrous tissue
FACT
The condition was first described by Fischer in 1913
Interest over time
Google searches
Common signs & symptoms
Clinical presentation is often insidious, contributing to delayed diagnosis.
Local symptoms
• Localized pain
• Most common presenting symptom
• Typically dull, chronic, and progressive
• May be present for months to years before diagnosis
• Swelling or palpable mass
• Usually over the anterior tibia
• Firm, slowly enlarging
• Bone deformity
• Cortical expansion or bowing in longstanding disease
• Pathologic fracture
• Occurs due to cortical destruction and weakening
• May be the initial presentation in some patients
Functional symptoms
• Limp or gait disturbance
• Particularly in lower extremity involvement
• Reduced weight-bearing tolerance
Systemic symptoms
• Generally absent
• Fever, weight loss, and malaise are uncommon
Typical Anatomic Distribution
• Tibia (≈80–90% of cases), often mid-shaft
• Fibula (less common)
• Rare involvement of:
• Femur
• Ulna
• Humerus
• Pelvis
Disease Behavior and Progression
• Slow-growing but locally aggressive
• Late metastases may occur, most commonly to:
• Lungs
• Regional lymph nodes
• Metastatic risk increases with:
• Incomplete resection
• Local recurrence
• Long disease duration
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| MEKTOVI® for the Treatment of Pediatric Adamantinomatous Craniopharyngioma | MEKTOVI (binimetinib) is an oral, highly selective reversible inhibitor of mitogen-activated extracellular signal regulated kinase 1 (MEK1) and MEK2. The biological activity of binimetinib that has been evaluated bith in vitro and in vivo in a wide variety of tumor types In this Phase II, the drug w... | Phase 2 | Recruiting | Drug: Binimetinib Oral Tablet [Mektovi] | More Info |
| FOG-001 in Locally Advanced or Metastatic Solid Tumors | The goal of this clinical trial is to determine if FOG-001 is safe and effective in participants with locally advanced or metastatic solid tumors. | Phase 1, Phase 2 | Recruiting | Drug: FOG-001 Drug: mFOLFOX-6 Drug: Nivolumab Drug: Trifluridine/tipiracil Drug: Bevacizumab | More Info |
References:
Czerniak B, Dorfman HD. Adamantinoma of bone: a clinicopathologic study of 116 cases. Cancer. 1989;64(3):730–739. Hazelbag HM, Taminiau AH, Fleuren GJ. Adamantinoma of the long bones: clinicopathological features and immunohistochemical analysis of 40 cases. J Pathol. 1994;173(4):315–323. Qureshi AA, Shott S, Mallin BA, Gitelis S. Current trends in the management of adamantinoma of long bones. Clin Orthop Relat Res. 2000;(373):255–263. WHO Classification of Tumours Editorial Board. WHO Classification of Tumours of Soft Tissue and Bone. 5th ed. IARC; 2020. National Cancer Institute (NCI). Adamantinoma of Bone. Orthobullets. Adamantinoma – Bone Tumor Overview.