Fact-sheet: Chordoma


Updated on 12/08/2015 at 10:51 PM

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Definition

Primary malignant tumor arising from notochordal remnants, lobulated, contained within a pseudocapsule.
Age: 30–70 years.
Location: Sacrum (50%), Clivus (35%), Vertebrae (15%), Other sites (5%: mandible, maxilla, scapula)

Radiography

Osteolytic pattern.
Osteosclerotic pattern possible.
Punctate calcifications in 30 to 70% of cases

CT

Relatively hypodense mass, with a hyperdense fibrous pseudocapsule.
Calcifications.
Pure osteolytic pattern, with or without mass effect.
Osteosclerotic pattern possible

MRI

Hypointense to intermediate T1 signal, occasionally hyperintense due to protein content.
Signal heterogeneity related to calcifications, necrosis, or mucoid areas.
Marked T2 hyperintensity.
Anterior, lateral, or posterior extension: spinal cord compression

Management

Metastases (5–40%): liver, lung, lymph nodes, peritoneum, skin, heart.
Near-constant late local recurrence despite surgery

Differential diagnosis

Metastasis.
Lymphoma.
Multiple myeloma.
Eosinophilic granuloma.
Benign notochordal cell tumor.
Giant cell tumor.
Aneurysmal bone cyst.
Hemangioma.
Osteochondroma, Osteoblastoma.
Infection: Pyogenic vertebral osteomyelitis, Tuberculous spondylitis, Brucellosis, Fungal infection, Echinococcosis, Sarcoidosis