Silent but deadly – how to spot a sarcoma

Craig Gerrand

Consultant Orthopaedic SurgeonFreeman Hospital, Newcastle

Introduction

• What is a sarcoma, and why does it matter?

• Who gets sarcomas?• What is the best treatment for a

sarcoma?• How can I tell a sarcoma from a

ganglion?

What is a lipoma?

Benign tumour of mature adipose tissue

What is a ganglion?

A simple fluid filled cyst usually arising from a joint or tendon.

What is a sarcoma?

Bone and soft tissue sarcomas

• Are rare malignant tumours arising from connective tissues

• Heterogenous in type and location

• Most are mesenchymal in origin

How rare is rare?

• Soft tissue masses are common (many hundreds in a GP lifetime)

• Malignant soft tissue sarcomas are uncommon (1 or 2 in a GP lifetime)

• England and Wales- 400 primary bone tumours per annum- 1500 soft tissue sarcomas per annum

How malignant 1?

• Survival of all patients with bone sarcomas

How malignant 3?

• Survival of all patients with soft tissue sarcomas

Primary bone tumoursMorphological classification

• Over 30 variants

Histological type Benign Malignant

Haemopoetic Myeloma Lymphoma

Chondrogenic Osteochondroma Chondroblastoma Chondromyxoid fibroma

Chondrosarcoma

Osteogenic Osteoid osteoma Osteosarcoma

Unknown Giant cell tumour Ewing’s tumour

Fibrogenic Benign fibrous histiocytoma

Malignant fibrous histiocytoma

Notochordal Chordoma

Soft tissue sarcoma Morphological classification

• Over 100 variants

Mesenchymal differentiation

Other differentiation, consistent pattern

No specific differentation, variable

pattern

Liposarcoma Leiomyosarcoma Rhabdomyosarcoma Angiosarcoma MPNST Osteosarcoma Chondrosarcoma Fibrosarcoma

Synovial sarcoma Epithelioid sarcoma Clear cell sarcoma Ewing sarcoma/PNET Alveolar soft parts sarcoma

MFH Sarcoma NOS

Aetiology

• Most are sporadic• Predisposing factors

- inherited predisposition- immunosuppression- irradiation- specific chemicals

• Genetic changes within cells lead to tumours

Age distribution of primary bone tumours

Age distribution of soft tissue sarcomas

Soft Tissue SarcomaDistribution

• 55% extremities• 35% retroperitoneum and

viscera• 10% head and neck

What is the best treatment for sarcomas?

Best treatment

• Early detection and referral• Multidisciplinary team

management

Why refer early?

• Delays in diagnosis are common• Risk of metastasis relates to size

of tumour

Where do delays occur?

• Patient (7 months)• GP (7 months)• Hospital

Biopsy of sarcomas

Sarcomas are implantable

Therefore

Careful planning is mandatory

The Whoops! procedure

• Excision without regard to principles of tumour surgery and usually without imaging

• ie tumour “shelled out” without considering the possibility of malignancy

• Further treatment complicated

Principles of biopsy

• Image first• Careful planning with regard to definitive

surgery• If in doubt, discuss!• Excise the biopsy track during definitive

surgery• Extensile incisions• Avoid neurovascular structures • Good haemostasis• Drain through or close to the wound

Treatment of sarcomas

• Multidisciplinary team • Combined approach with

radiotherapy, surgery and chemotherapy

• Specialist centre

Patient perspective

• Rare tumour• Often incorrectly reassured• May have to travel for treatment

Low grade fatty tumours

• Low risk of local recurrence• Low risk of metastatic disease• Treatment by planned marginal

excision

Indications for amputation

• Adequate margins not possible with limb salvage

• Major complications of radiotherapy would follow

• A below knee amputation may be more serviceable than a salvaged distal extremity

• Some cases of local recurrence may not be treatable by standard surgery and radiotherapy

The future

• Changes in administrative structure (NICE, NSCAG, NCRI)

• Better identification of risk groups

• Better chemotherapy• Better radiotherapy• Tissue engineering• Gene therapy• New imaging modalities

Who to refer 1?

• Soft tissue masses- Size >5cm- Painful- Increasing in size- Deep to fascia- Recurring after previous excision

Who to refer 2?

• Undiagnosed bone pain needs an x-ray!

• Bone abnormalities on X-ray- Bone destruction- New bone formation- Associated soft tissue swelling- Periosteal elevation

North of England Bone and Soft Tissue Tumour Service

• Orthopaedics- Shona Murray- Craig Gerrand- Mike Gibson

• Plastic Surgery- Rick Milner

• General Surgery- Derek Manus- Paul Hainsworth

• Thoracic surgery- Sion Barnard

• Clinical Oncology- Helen Lucraft- Charles Kelly

• Medical Oncology- Mark Verrill- Ruth Plummer

• Paediatric Oncology- Juliet Hale- Quentin Campbell

Hewson- Alan Craft

• Radiology- Geoff Hide- Chris

Baudoin

• Pathology- Petra Dildey

• Macmillan nurse- Joy Dowd

Thank you