2. • It is basically a rare,slowly growing locally aggressive malignant tumor that
arises from remenants of the notochord.
• Chordomas usually occur in sacrum(Sacrococcygeal region) and
cranium(Base of skull)
• They account for 1-4 percent of all malignancies.
• Sacral Chordoma is the most common primat malignancy of the sacrum.
First described by Virchow in 1857.
• The notochordal hypothesis is supported by the fact that the site of
notochord vestiges correspond closely to the distribution of chordomas
with a morphological similarity between these remnants and the
histopathology exhibited by chordomas.
3. Etiopathogenesis
• It is a remnant oF the notochord.
• Usually males are affected in the 5th-7th decade.
• Pathology
• Gross: Tumour is large,round,smooth soft bluish white with fibrous septate.Bone destroyed
replaced by Tumour.
• Microscopy:Lobular Architecture is the hallmark with abundant cytoplasm and nuclear atypia
• 3 types are there
• Conventional Chordoma (Most common)
• Chondroid Chordoma(Best prognosis)
• Dedifferentiated chordoma(Poor prognosis)
4.
5. Clinical features
• Males in 5th-7th decade are affected.
• Patients may have a palpable mass.
• The patient will complain of lower back pain which worsens on sitting
and is not relieved with NSAIDS.
• There may be assoct urinary tract infection and constipation.
6. Investigations
• 1)X-ray-Vertebral destruction
• 2)CT-Centrally located,well circumscribed, destructive lyric lesion with
sclerosis,expansile soft tissue mass
• 3)MRI-Low intensity T1 and high intensity T2
7.
8. Differential diagnosis
• 1) Chondrosarcoma-Matrix has a typical ring and arcs
appearance.Chondrosarcoma usually affects thoracic spine.Usually
neuralarch is more commonly affected than vertebral body.
• 2)GCT-Females are affected
• Mc site sacrum>Thoracic spine>Cervical spine>Lumbar spine
• 3)Spinal Mets
9. Treatment
• Complete en bloc surgical resection is the treatment of choice with colostomy bone reconstruction and
stabilization of spine with myocutaneous flaps.
• Drugs-Imatinib,cisplatin, Doxorubicin.
• Radiotherapy is reserved for irresectable cases and palliative therapy as tumour is relatively radioresistant.
• The main complication is Reccurence (5-20 percent)
• 7-14 percent also have metastatic spread which could be to the lungs,brain or abdomen.
• As the tumour is locally aggressive the prognosis is poor With 10 year survival rate of approximately 40
percent.
