Chordoma

Definition

 

Primary malignancy arising from remnants of the notochord

 

Epidemiology

 

Rare malignant tumour

 

Middle-aged adults (50-70)

 

M>F

 

Location

 

In midline in axial skeleton

 

Base of skull 35%

 

Vertebrae 15%

- especially cervical

 

Sacrum 50%

 

Clinical

 

Sacral Tumour

 

Usually presents late (long History LBP)

- slow-growing

- large potential space to expand into

- often very large on presentation

 

Perineal pain

Bladder & bowel dysfunction

Mass effect

Neurological compression

 

Usually can feel rectal mass ~ 50%

 

X-ray

 

Bony destruction is hallmark + soft tissue mass

- 50% Calcification within mass

 

Sacrum

- irregular areas of bone destruction

- sacral expansion

- soft tissue mass

 

CT Scan / MRI 

 

Useful to delineate tumour

 

Sacral Chordoma MRI0001Sacral Chordoma MRI0002

 

Pathology

 

Gross 

- lobulated bluish / gray

- extensive gelatinous translucent areas which are focally cystic & haemorrhagic

 

Histology

- lobular framework of Physaliphorous cells

- cells have bubbly pink cytoplasm & Bulls-Eye nucleus

- vascular fibrous septa

- mucinous matrix

- 1/3 significant Chondroid production (can be mistaken for chondrosarcoma)

 

DDx

 

GCT / ABC

Chondrosarcoma / OS / Ewing's / Myeloma

Metastasis

 

Management

 

Operative

 

Most important predictor of survival is clear surgical margin

- usually difficult due to location

 

Sacral Surgery

- leave at least 1 S3 - 100% continent

- leave at least 1 S2 - 50% continent

- above S2 incontinent because pelvic splanchnics removed

 

Radiotherapy

 

Indications

- resection not possible

- positive margins

 

Rarely effective

 

Prognosis

 

Metastasis 30-50%

- pulmonary mets may occur (late)

 

Death usually 2° local infiltration