Definition. TumourA swelling or lesion formed by abnormal growth of cells"A tumour can be.BenignMalignant (Primary, secondary)Remember to score points.. What do I need to know for the exam?. RecognitionSymptoms, signs, investigationsStagingTreatment Options. Natural History. Mesenchym
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1. Paediatric Tumours Peter Calder
Royal National Orthopaedic Hospital
The London Intensive Paediatric Course
2. Definition Tumour
“A swelling or lesion formed by abnormal growth of cells”
A tumour can be….
Malignant (Primary, secondary)
Remember to score points….
3. What do I need to know for the exam? Recognition
Symptoms, signs, investigations…
4. Natural History Mesenchymal neoplasms have characteristic patterns of behaviour and growth.
Benign lesions are surrounded by a true capsule composed of compressed normal cells.
5. Natural History Spindle cell sarcomas form solid lesions with circumferential growth. The periphery of the lesion is composed of the least mature cells. (Round cell tumours)
Enclosed by a pseudocapsule consisting of viable tumour cells and a fibrovascular reactive tissue with inflammatory tissue. This varies in thickness dependant on the lesion.
6. Behaviour Benign/latent
Slow growth during normal growth of individual, then stop. Never become malignant. (Non-ossifying fibroma)
Progressive growth (Aneurysmal bone cyst)
Locally aggressive but do not metastasize. There is a pseudocapsule with tumour extension into the reactive zone. Local control can only be achieved by complete removal of the lesion. (Giant-cell tumour)
7. Behaviour Malignant/Low-grade
Low potential to metastasize. There is a pseudocapsule and tumour nodules exist within the reactive zone but rarely beyond. Local control by excision of normal cuff of tissue. (Parosteal osteosarcoma)
Rapid growth and early metastasis. Require local control and systemic therapy to prevent metastasis. (Osteosarcoma)
8. Spread Local anatomical barriers.
Take the path of least resistance.
Compression of normal tissue
Resorption of bone by osteoclasts
Direct destruction of local tissues
Benign usually unicompartmental.
Malignant are mostly bicompartmental.
Dissemination almost exclusively through blood. (Early pulmonary involvement)
Enneking et al
Clin Orthop 1980;153:106-20
Enneking at al
Clin Orthop 1986;204:9-24
10. Staging – Grade (G) Assessment of biological aggressiveness
G0 Histologically benign (well differentiated, low cell to matrix ratio)
G1 Low grade malignant (few mitoses, moderate differentiation, local spread)
G2 High grade malignancy (frequent mitoses, poorly differentiated with cellular atypia, necrosis, significant vascularity high risk of mets)
11. Staging – Site (T) Anatomic setting of the lesion
T2 Extracompartmental (spread beyond ‘fascial’ plane without longitudinal containment)
12. Staging – Metastasis (M) Nodal or blood borne tumour spread
M0 No evidence of regional or distant metastases
M1 Regional or distant metastases evident
13. Clinical versus Viva
14. Age Predilection – Benign Birth – 5yrs
Unicameral Bone Cyst (Rare)
6 – 18 yrs
Unicameral (Simple) Bone Cyst
Aneurysmal Bone Cyst
15. Age Predilection – Malignant Birth – 5yrs
Metastatic Neuroblastoma 6 – 18yrs
Ewings Sarcoma (Round cell tumour)
16. Anatomical Location
17. Soft Tissue versus Bone
18. Lump – Browse’s book Site
19. Diagnostic Clues….Soft Tissue Size
A small mass (<5cm) is unlikely to be malignant.
Accurate measurement with USS, CT or MRI.
A rapid increase in size is more likely to be a sarcoma.
A mass that increases and decreases in size is usually a cystic lesion.
20. Diagnostic Clues….Soft tissue Site
Superficial lesions are more likely to be benign.
Superficial malignant lesions have a better prognosis than deep ones.
Thigh and buttock regions are the commonest sites for soft tissue sarcomas, high index of suspicion.
21. Diagnostic Clues….Soft tissue Consistency
Lipomas are usually non tender and soft to palpation. May be firm if deep and muscles are contracted. Will seem to change when compartment relaxed.
Soft tissue sarcomas tend to be firm and are not painful unless very large resulting in vascular compromise or neural compression.
22. Diagnostic Clues….Soft tissue Cystic versus Solid
Most cystic lesions are inflammatory or benign lesions, eg ganglion.
If solid can be either benign OR malignant.
23. Diagnostic Clues….Bone What is the effect of the lesion on the bone?
High grade lesions spread rapidly destroying bone (describe as aggressive)
24. Diagnostic Clues….Bone What is the response of the bone to the lesion?
High grade lesions spread too rapidly for the bone to react. This results in a wide zone of transition.
Low grade lesions spread slowly resulting in a narrow, sclerotic zone of transition.
25. Diagnostic clues….Bone Features of malignancy
Small is good, large is bad
Lack of sclerotic margin
Presence of soft tissue mass
26. Diagnostic clues….Bone Cartilage tumours have speckled calcification.
Simple unicameral cysts are central
Non ossifying fibromas are eccentric
Simple bone cysts
GCT – nearly always occur near the joint surface in mature bone.
Chondroblastoma = GCT in children. Lytic tumour arising in the epiphysis.
27. Diagnostic clues….Bone As tumour extends through the cortex the periosteum may be elevated
Spicules forming sunburst appearance
28. Diagnostic clues….Bone Ewings sarcoma usually extensive lytic lesion.
Perisoteal reaction – oinion skin
29. Remember Others….
30. What do I do now? ? Transfer to Specialist Centre
“This is what they would do….”
31. What do I do now? Full clinical examination.
FBC, ESR, Biochemistry (Bone profile)
Acid Phosphatase, TFTs, PSA, Protein electrophoresis (Myeloma)
Urinalysis (Bence-Jones proteins)
CT lesion and chest
32. Biopsy Last step of staging.
Performed by unit/surgeon who will perform definitive treatment.
Biopsy tract/orientation is critical.
Haemostasis to avoid tracking haemtoma.
Open versus Needle
33. Surgical Margins Intra-lesional
Marginal – through pseudocapsule
Wide – cuff of normal tissue
Radical – removal of entire compartment
34. Adjuvant Therapy
35. Summary Tumour Characteristics
Age and Site Predilection
Management Decision Making