Transcript Tumours of the musculoskeletal system

Silent but deadly –
how to spot a sarcoma
Craig Gerrand
Consultant Orthopaedic Surgeon
Freeman Hospital, Newcastle
Introduction
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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
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Are rare malignant tumours arising
from connective tissues
Heterogenous in type and location
Most are mesenchymal in origin
How rare is rare?
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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
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400 primary bone tumours per annum
1500 soft tissue sarcomas per annum
How malignant 1?
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Survival of all patients with bone sarcomas
How malignant 3?
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Survival of all patients with soft tissue sarcomas
Primary bone tumours
Morphological classification
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Over 30 variants
Histological type
Benign
Haemopoetic
Chondrogenic
Malignant
Myeloma
Lymphoma
Chondrosarcoma
Osteogenic
Osteochondroma
Chondroblastoma
Chondromyxoid
fibroma
Osteoid osteoma
Unknown
Giant cell tumour
Ewing’s tumour
Fibrogenic
Benign fibrous
histiocytoma
Malignant fibrous
histiocytoma
Chordoma
Notochordal
Osteosarcoma
Soft tissue sarcoma
Morphological classification
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Over 100 variants
Mesenchymal
differentiation
Liposarcoma
Leiomyosarcoma
Rhabdomyosarcoma
Angiosarcoma
MPNST
Osteosarcoma
Chondrosarcoma
Fibrosarcoma
Other differentiation,
No specific
consistent pattern
differentation, variable
pattern
Synovial sarcoma
MFH
Epithelioid sarcoma
Sarcoma NOS
Clear cell sarcoma
Ewing sarcoma/PNET
Alveolar soft parts
sarcoma
Aetiology
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Most are sporadic
Predisposing factors
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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 Sarcoma
Distribution
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55% extremities
35% retroperitoneum and viscera
10% head and neck
What is the best
treatment for
sarcomas?
Best treatment
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Early detection and referral
Multidisciplinary team management
Why refer early?
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Delays in diagnosis are common
Risk of metastasis relates to size of
tumour
Where do delays occur?
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Patient (7 months)
GP (7 months)
Hospital
Biopsy of sarcomas
Sarcomas are implantable
Therefore
Careful planning is mandatory
The Whoops! procedure
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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
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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
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Multidisciplinary team
Combined approach with radiotherapy,
surgery and chemotherapy
Specialist centre
Patient perspective
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Rare tumour
Often incorrectly reassured
May have to travel for treatment
Low grade fatty tumours
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Low risk of local recurrence
Low risk of metastatic disease
Treatment by planned marginal
excision
Indications for amputation
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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
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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?
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Soft tissue masses
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Size >5cm
Painful
Increasing in size
Deep to fascia
Recurring after previous excision
Who to refer 2?
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Undiagnosed bone pain needs an x-ray!
Bone abnormalities on X-ray
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Bone destruction
New bone formation
Associated soft tissue swelling
Periosteal elevation
North of England Bone and Soft Tissue
Tumour Service
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Orthopaedics
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Plastic Surgery
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Derek Manus
Paul Hainsworth
Clinical Oncology
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Sion Barnard
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Juliet Hale
Quentin Campbell
Hewson
Alan Craft
Radiology
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Mark Verrill
Ruth Plummer
Paediatric
Oncology
Thoracic surgery
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Helen Lucraft
Charles Kelly
Medical Oncology
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Rick Milner
General Surgery
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Shona Murray
Craig Gerrand
Mike Gibson
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Pathology
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Geoff Hide
Chris Baudoin
Petra Dildey
Macmillan
nurse
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Joy Dowd
Thank you