Tumour

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

 

 

Primary Spinal Tumours

 

DDx

 

Primary

 

Benign

 

Osteoid Osteoma

Osteoblastoma

Osteochondroma

ABC

Hemangioma

Giant Cell tumour

EG

 

Malignant

 

Chordoma

Ewings

Osteosarcoma

Chondrosarcoma

Lymphoma

Multiple myeloma

 

Secondary

 

Leukaemia 

Prostate / Breast / Lung / Thyroid / Kidney

 

DDx by Site

 

Posterior elements

- OO, OB, ABC

- chordoma / osteosarcoma

 

Vertebral body

- giant cell

- hemangioma

 

Sacrum

- chordoma

- Ewing's

 

Benign Spinal Tumours

 

ABC

 

Patient in teens or twenties

Night pain

Posterior elements

 

Treatment

 

Embolisation

Surgical resection

- complete to prevent recurrence

 

Giant cell tumour

 

30 - 40 years

In sacrum

Present with pain

May have bladder and bowel dysfunction

 

Treatment

 

Wide excision

- may get incontinence

Adjunctive radiotherapy

Now using serial embolisation

 

Hemangiomas

 

Hemangioma Spine

 

Very common

- usually asymptomatic and incidental finding

- vertebral body or posterior element

- thoracic spine

 

Xray

- striations

 

MRI

- High signal on T2

 

Treatment

 

Indicated for collapse or neurology

Surgical excision

Embolisation

Radiotherapy

 

Osteoid Osteoma

 

Male between 20 - 40

 

Posterior elements lumbar spine

- < 2 cm

 

Can cause scoliosis

- in apex of convexity

 

Treatment

 

Surgical Removal / High frequency radioablation

Often scoliosis will then resolve if not too long standing

 

Osteoblastoma

 

Osteoblastoma Spine0001Osteoblastoma Spine0002Osteoblastoma Spine0003

 

Similar histology to OO

- > 2 cm

 

Much more aggressive / less common

 

Found in the posterior elements

- male teens, twenties

 

Treatment

 

Osteoblastoma Exision and Fusion0001Osteoblastoma Exision and Fusion0002

 

Wide excision +/- posterior fusion

 

Can recur

- life long follow up

 

Eosinophilic Granuloma

 

Vertebroplanar in young child < 10

 

Treatment

 

Self limiting

- will reconstitute up to 50% vertebral height

 

Primary Malignant Tumours

 

Chordoma

 

Rare slow growing malignant tumours

- arise from primitive nervous system

- slow to metastasise

 

50% sacrococcygea

 

Elderly patients

 

Treatment

 

Surgical Resection

- very difficult

 

Adjunctive Radiotherapy

 

Life expectancy 10 - 15 years after diagnosis

 

Osteosarcoma

 

Uncommon

Vertebral body

 

Treatment

 

Radical excision

Adjunctive radiotherapy and chemotherapy

 

Chondrosarcoma

 

Middle age or older

Males

Patient with Maffucci / Ollier's


Treatment

 

Wide surgical resection

Overall prognosis is poor as this is difficult

 

Lymphoma

 

Can be solitary lesion

Anterior element

 

Treatment

 

Radiotherapy and Chemotherapy

Surgery if unstable / deformity

 

Ewing's

 

50% in sacrum

Young male in teens

 

Treatment

 

Surgical resection

Radiotherapy and chemotherapy

20% 5 year survival

 

Multiple Myeloma / Plasmocytoma

 

50 - 80 years

 

Treatment

 

Radiotherapy and chemotherapy

Surgery for instability

 

Spinal Cord Tumours

 

DDx by Location

 

Extramedullary

- neuroblastoma

- ganglioneuroma

- Ewings sarcoma

- leukaemia

- lymphoma

- synovial cysts

 

Spine Synovial Cysts MRI

 

Extramedullary Intradural

- dermoid

- epidermoid

- meningioma

- neurofibroma

 

Intradural

- astrocytoma

- ependymoma

- lipoma

 

Meningioma

 

Slow Growing

F:M 9:1

Intra-dural / extramedullary

 

Management

- intradural resection

- RTX for residual tumour which is growing

 

Thoracic Meningioma

 

Ependymoma

 

Primary glial tumour of spinal cord

- intramedullary

- tend to be benign in spinal cord (c.f. intracranial

 

Ependymoma Conus MRI SagittalEpendymoma Conus Axial MRI

 

Management

- debulking

- radiotherapy

Spinal Metastasis

EpidemiologyThoracic Metastasis

 

80% cancer patients have spinal metastasis at autopsy 

 

Spine is number one site for bony metastasis (50%)

 

Cause

 

Hexagon: PBBLTK

 

                 Prostate    Breast

 

Bronchus      MM     Lymphoma        Bowel

   

                 Renal        Thyroid

 

NHx

 

20% of develop cord compression

30% survive >12/12

 

Pathology

 

Site

- thoracic spine  - 70%

- lumbosacral > cervical spine

 

Usually multilevel 

 

Vertebral body 

- 85%

- usually posterior body near pedicles

- posterior elements uncommon

 

Symptoms

- neural compression - demyelination / ischaemia

- pathological fracture

- spinal instability

 

Method of Spread

 

1.  Arterial metastasis

- haematogenous via nutrient arteries

- lung / breast

 

2.  Direct invasion

- through intervertebral foramen 

- lymphoma

 

3. Venous 

- via Batson's Plexus

- valveless veins from the pelvis to the internal venous plexus of the spine / prostate

- GIT tumours commonly spread to liver first via the portal system 

- then later to bone

 

4.  Lymphatic

 

Clinical

 

Pain

- 95% neoplastic pain (night and rest pain)

 

Weakness

- 75% at diagnosis

- bilateral & symmetrical

 

Sensory loss

- 50% at diagnosis

- ssually affects the feet first 

 

Loss of sphincter control

- 50% at diagnosis

 

X-ray

 

75% have abnormality

 

Winking Owl Sign

- pedicle loss on AP

 

Lytic / Sclerotic lesion

- need 30% bone loss to see lytic area

 

Vertebral body collapse 

 

Lumbar Metastasis XrayCervical Metastasis MRI

 

Bone Scan

 

Very sensitive 

- detect metastasis > 2mm

- screening tool

 

False positive

- crush fracture

 

False negative

- myeloma

 

"Superscan"

- symmetric increased uptake

- metastatic disease

- renal or endocrine abnormality

 

CT Scan 

 

Define

- bony abnormality

- deformity

- potential instability

 

Cervical Lesions

 

Cervical Spine Metastasis CTDens Metastasis

 

Lumbar

 

Lumbar Metastasis CTLumbar Spinal Met CT

 

MRI

 

Define

- soft tissue masses 

- nerve and cord impingement

 

Lumbar Metastasis MRI

 

Metastatic Tumour L1 MRI T1Metastatic Tumour L1 MRI Axial

 

Cervical Metastasis MRI

 

Classification Harrington

 

Class I

- Minimal bone involvement

 

Class II

- Bone destruction < 1/2 body / no instability / no cord compression 

 

Class III

- Spinal canal compromise due to epidural disease / no significant bone involvement

 

Class IV

- Pathological fracture ± deformity / no significant neurological compromise

 

Class V

- pathological fracture with collapse / instability & neurological compromise

 

Management

 

Prognosis

 

Outcome after treatment = Neurological impairment before treatment

- most ambulatory patients remain ambulatory after treatment

- few paraplegic patients are able to walk after treatment

 

Radiotherapy v Surgery

 

Patchell et al Lancet et al

- randomised multicentred trial

- patients with spinal cord compression from metastasis

- trial had to be stopped

- superior results for surgery c.f. radiotherapy and steroids

- improved patient walking ability / retained walking ability

- better maintenance of continence and Frankel grades

 

Goals

 

1. Preserve neurological function

- ambulation

- bladder and bowel 

 

2. Pain relief

 

3. Spinal stability

 

Decision making

 

Team approach

- oncologists

- radiation oncologists

- palliative medicine

 

Issues

- life expectancy

- fitness for surgery

- tumour type

- spinal stability

 

Harrington Classification

 

Group 1 & 2 +/- 3

- radiotherapy +/- chemotherapy

 

Group 4 & 5

- collapse / instability / impending deformity / deformity / neurology

- surgery

 

Radiotherapy

 

Sensitivity

 

Very - myeloma, lymphoma

Moderate - breast, lung, bowel, prostate

Resistant - thyroid, kidney, melanoma

 

Indications

- Harrington 1 & 2 +/- 3 radiosensitive

- no neurology

- neurology with poor prognosis or unfit for surgery

 

Operative Management

 

Indications

- neurology / cord compression

- failure of radiotherapy

- deformity

- instability

- > 3/12 to live

- fit for surgery

 

Instability

- > 50% height loss

- anterior and posterior columns at same level

- bone loss > 2 vertebrae

 

Options

 

1.  Decompressive laminectomy

- historical operative associated with poor outcomes

 

2.  Percutaneous PMMA / Vertebroplasty

 

Indications

- stable lesion

 

Spinal Met Percutaneous PMMA

 

3.  Posterior stabilisation

 

A.  Long segment stabilisation

 

Thoracic Spine Pathological Fracture StabilisationCervical Tumour Posterior Stabilisation

 

B.  Short segment stabilisation + PMMA

 

Lumbar Metastasis Posterior Stabilisation with PMMAVertebral Met Posterior Stabilisation + PMMA

 

3.  Corpectomy / PMMA / Anterior stabilisation

 

Technique

- remove body and disc to dura

- PMMA sufficient if LE < 6 months

- titanium cage and BG / structural graft if LE > 6/12

- stabilised with anterior plates

 

Cervical Spine Metastasis Anterior StabilisationCervical Spine Anterior Stabilisation 2

 

Post op Radiotherapy

 

Week 2 if no bone graft

Week 6 if bone graft used