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Tumours of MSK system Flashcards

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1
Q

Epidemiology of bone tumours

A

Sarcoma of Bone account for 0.2% of all malignancies
Approx 500 diagnosed in the UK every year
However represent 4% of all malignancies in Children <14 years
Slight higher incidence in males (13:10)
Approx 3275 new cases per year in UK
Almost equal distribution between Males and Females (51:49)

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2
Q

Typical patient history for bone tumour

A

Presenting Complaint:
?Mass
?Pain
?Loss of Function

History of Presenting Complaint:
Duration of symptoms, including full Pain / Mass history
How issue came to patient’s attention

Past Medical History and Medication History
Family History
Social History – what is their current level of function?

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3
Q

Red flag signs in history

A

Rest pain
Night pain
Lump present – tender, enlarging, deep to fascia, above 5cm in diameter
Loss of function
Neurological symptoms
Unwell / Weight loss

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4
Q

Examination of patient

A

Look, Feel, Move
Assess function of a joint/limb:
Range of motion
Neurovascular status
Assess any masses
Scars
Skin lesions
sensations
Reflexes

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5
Q

Initial investigations

A

Blood tests – FBC, U+E, Ca2+, Alk Phos
Plain Xrays
Ultrasound
CT Scan
MRI Scan
Bone scan
Raised CRP and ESR
Urine

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6
Q

CT scan and tumours

A

Better for assessing bone quality, as well as solid tumours
Staging CT of Chest / Abdo / Pelvis essential if suspect metastases

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7
Q

MRI scan

A

Better for assessing reactive changes of soft tissue / bone marrow
Recent studies assessed full-body MRI to look for metastases
T1 imaging: anatomy
T2 imaging: pathology
Soft tissue masses
Skip lesions

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8
Q

Bone scan

A

Better for assessing reactive changes of soft tissue / bone marrow
Recent studies assessed full-body MRI to look for metastases

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9
Q

Plain x ray

A

Look to see what the lesion is doing to the bone and vise-versa
Definition / Bone density / Zone of Transition / Periosteal reaction
Osteoblastoma (looks dark) vs Osteosarcoma (looks bright white)

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10
Q

Plain x ray can show the location of the tumour in the bone… what are the locations in bone?

A

Epiphysis
Physis
Metaphysis
Diaphysis
Central/Cortical

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11
Q

A plain x ray shows the zone of transition what are they

A

Narrow - good
Wide - bad

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12
Q

Plain x rays can show the matrix, what is a matrix

A

Represents the extracellular matrix produced by tumor cells
Depends upon cellular lineage of progenitor cells

Fibrous – ground glass
Osteoid – Cumulus cloud
Chondroid – Popcorn

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13
Q

Plain x ray can show cortical involvement such as

A

Scalloping
Cortical thinning
Expansile

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14
Q

Ultrasound

A

Chepa and quick
Real time assessment
Differential normal vs abnormal, solid vs cystic

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15
Q

Staging: Grading of Malignant Tumours

A

Enneking system:
G0 = Histologically Benign
Well Differentiated i.e. resemble cell of origin
Low mitotic count
G1 = Low Grade Malignant
Moderate differentiation; Few mitoses
Local spread only. Low risk of metastasis
G2 = High Grade Malignant
Poorly differentiated; Frequent mitoses
High risk of metastasis

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16
Q

Enneking classification Grade 1

A

Latent
Well defined margin. Grows slowly then stops
May heal spontaneously and very low risk of recurrence

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17
Q

Enneking classification Grade 2

A

Active
Progressive growth limited by natural barriers
Well defined margin but may expand and thin cortical bone (e.g. ABC)
Negligible recurrence after marginal resection

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18
Q

Enneking classification Grade 3

A

Aggressive
Growth not limited by natural barriers (e.g. GCT)
Metastasis present in 5% patients
High recurrence following marginal resection (extended resection therefore needed)

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19
Q

Enneking classification: T1 AND T2

A

T1 - intracompartmental
T2 - extracompartmental

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20
Q

Guide to naming tumours prefix

A

Osteo… = Bone
Chondro… = Cartilage
Rhabdomyo… = Skeletal Muscle
Lipo… = Fat

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21
Q

Guide to naming tumours suffix

A

oma = Benign tumour
…sarcoma = Malignant connective tissue tumour
…carcinoma = Malignant epithelial/endothelial tumour
…blastoma = (Malignant) Tumour of embryonic cells

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22
Q

Outliers to naming tumours

A

Tumours may have an eponymous name (e.g. Ewing’s)
If the Tissue of Origin is unclear, the tumour may be described by its cellular appearance
Or, especially in soft tissue sarcoma, may come under the term

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23
Q

Where do bone tumours present

A

Osteosarcoma - knee (distal femur, proximal tibia)
Parosteal osteosarcoma - distal femur (posterior cortex)
Chondrosarcoma – pelvis
Giant cell tumors – knee
Chordoma – sacrum
Adamantinoma - tibia

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24
Q

Bone cysts usually found in children

A

Unicameral Bone Cyst (UBC)
Aneurysmal Bone Cyst (ABC)
Fibrous dysplasia
Eosinophlic granuloma (EG)
Intra-osseous lipoma (rare

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25
Destructive lesions in young adults
Osteosarcoma Ewings Infection Giant Cell Tumour (GCT) ABC
26
Destructive lesions in those >50
Osteosarcoma Chondrosarcoma Metastasis Myeloma/Lymphoma Giant cell tumour Infection
27
Bone Tumours
Benign Osteoid Osteoma Osteoblastoma Osteochondroma Malignant Osteosarcoma
28
Fibrous tumours
Benign Fibrous Dysplasia Non-ossifying Fibroma Malignant Fibrosarcoma
29
Cartilage tumours
Benign Enchondroma Chondroblastoma Malignant Chondrosarcoma
30
Cystic tumours
Unicameral bone cyst Aneurysmal bone cyst
31
Unknown aetiology tumours
Giant cell tumour Ewing Tumour Adamatinoma
32
Osteoid osteoma
Benign, bone-forming lesion in young pts Localised pain and self-limiting (<7yrs). Produces high levels Prostaglandin E2 Proximal femur, Tibial diaphysis, Spine Xray – Active reactive bone + nidus (5-10mm) Best appreciated on CT
33
Management for osteoid osteoma
Medical Mx: NSAID’s (50% successful) Surgical Mx: Radiofrequency Ablation/ En-bloc Resection
34
Osteoblastoma
A rare bone producing tumour The “big brother” of osteoid osteoma > 2cm M:F= 3:1 Presentation = pain, not self-limiting Locations: Spine, proximal humerus, sacrum Spine lesions can present with neurology X-rays: Bone destruction surrounded by reactive new bone.
35
Histology of osteobastoma
Interlacing trabeculae + loose fibrovascular stroma
36
Treatment for osteoblastoma
Excision with at least a marginal line of excision
37
Osteochondroma
Metaphyseal lesion, covered by cartilage cap, grows away from growth plate and stops growing after puberty PC: painless lump Males > females May have a narrow stalk (pedunculated), or broad base (sessile)
38
Treatment for osteochondroma
Surgical excision if symptomatic. Chance of malignant change: less than 1%.
39
What is osteosarcoma
Spindle cell neoplasms that produce osteoid
40
Different types of osteosarcoma
Intramedullary osteosarcoma (high grade) Parosteal osteosarcoma (low grade) Periosteal sarcoma (high grade) Telangiectatic osteosarcoma
41
Secondary osteosarcomas
Paget’s, Post-Radiation, Fibrous Dysplasia
42
What are osteosarcomas assosciated with?
Also associated with Chondrosarcoma and STS
43
High-grade intramedullary osteosarcoma
“Classic” osteosarcoma Most common type Location: knee, proximal humerus, proximal femur Age: children, young adults 90% are high grade and penetrate the cortex early forming a soft tissue mass (IIB lesion) 10-20% have pulmonary mets X-rays: bone formation with bone destruction MRI useful for staging
44
Pagets disease
Older population only. Pagets present in 0.7-0.9% population, hence this is rare Median age 64yrs for onset. High risk for pulmonary metastasis
45
Irradiation
60-70% survival for extremity lesions, 27% axial lesions Children treated with high dose radiotherapy at greatest risk Tend to be high-grade tumours. High risk of pulmonary metastasis Treat as per primary bone tumour guidelines
46
Fibrous dysplasia
Associated with McCune-Albright syndrome and Polyostic Fibrous Dysplasia McCune-Albright = fibrous dysplasia + café-au-lai spots + endocrine abnormalities
47
Management of osteosarcoma
If left untreated will be fatal Poor prognosis: Aggressive local growth Rapid haematological spread (15-20% present with pulmonary mets) Poor response to chemotherapy agents Multi agent chemo pre op 8-12 weeks Repeat procedures to Stage the Tumour Limb Salvage surgery (where possible) + adjuvant chemotherapy
48
Enchondromas
Benign lesions Tend to occur in small bones (Hand, Foot) Can occur anywhere in skeleton Do not destroy bone Xray – metaphyseal “popcorn” Hot on a bone scan Histology = “island of cartilage” 1% chance of malignant change
49
Chondroblastoma
Rare Epiphyseal tumour arising from chondral precursor cells. Location: distal femur (most common), proximal humerus Oval lytic lesion, sclerotic rim and soft tissue extension Patient: Male - 10-20yrs Similar to GCT (Giant Cell Tumour)
50
Chondrosarcoma
Histology important: Grade I rarely metastasise (low grade) Grades: II, III, and dedifferentiated (worst prognosis) Dedifferentiated have 75% risk of mets and 10% survival Chondrosarcomas are most commonly found in the pelvis Mainstay of treatment = Surgery + adjuvant chemotherapy
51
Fibrous dysplasia
developmental abnormality of bone - Failure to produce normal lamellar bone, mutation of GS alpha protein Can occur in any bone; Proximal Femur is the most common Progressive in Children, Non-progressive in Adults Can worsen during Pregancy (hormone receptors) Can also be painful due to increased bone turnover or fracture Increased risk of malignancy if Polyostotic
52
What do X rays show in fibrous dysplasia
Ground glass lesion with scleritic margin Always include fibrous dysplasia on a benign looking bone lesion Histology: “chinese alphabet”
53
Treatment for fibrous dysplasia
Medical = Biphosphonates Surgical = Curettage and cortical autograft.
54
Non-ossifying fibroma
Variants of normal growth. Benign only. Become more diaphyseal as child grows Approx 55% around the knee; 80% LL Radiographic features: Eccentric[to one side of the bone] Metaphyseal Sclerotic rim
55
Unicameral bone cysts
Metaphyseal Purely lytic Expand the bone in a symmetric fashion Often border the growth plate May have trabeculations in them once they have fractured
56
Aneurysmal Bone Cyst (ABC)
Always biopsy if large or any doubt Usually < 20yrs Metaphyseal and eccentric Xray: Eccentric, lytic, expansile lesion Look for fluid levels on MRI Treatment: simple curettage= 86% success
57
Giant cell tumours (GCT)
Locally destructive neoplasm with poorly defined cells 20-40yrs; F > M; Rare in Children Benign but <2% metastasise to Lungs Lesions: 50% in the knee; 30% Vertebrae and Sacrum Always get biopsy - histology multinucleated cells Xrays – destructive lesion, no sclerotic rim Treatment: Curettage, washout, dental burr, +/- bone cement. 90% success rate New treatment= Biphosphonates
58
Ewings Sarcoma
Small round cell tumour Arises from neural crest cells Children and young adults (rare if >30yrs) Caucasians > Afro-carribean (9:1) Clasically a diaphyseal lesion: femur, pelvis, tibia, humerus Destructive lytic lesion Balanced translocation
59
Investigation and treatment for Ewings
Bone Marrow Biopsy monotonous blue cell tumour indistinct cell borders no matrix production by the tumour cells. Typically chemo/radio-sensitive Treatment: chemotherapy, radiotherapy and surgical excision/reconstruction Prognosis: Poor if large tumour / pelvic / mets at Dx 70% survival overall; 30% if mets present
60
Adamantinoma
Rare tumour of long bones [tibia] Young adults PC: pain X-ray: rediolucent zones interlaced with sclerosis and some cortical destruction. Histology: epithelial cells in fibrous stroma (biphasic) A low grade malignant lesion But can metastasize to the lungs Treatment: wide surgical excision plus reconstruction
61
Multiple myeloma
Malignant plasma cell disorder Age: 50-80 years PC: bone pain, pathological fracture, fatigue (anaemia) Hypercalacemia Renal failure secondary to amyloid X-rays: punched out lytic lesions (pepper pot skull) Bloods: serum electropheresis-monoclonal band Urine: Bence-Jones proteins
62
Investigation and treatment for multiple myeloma
Histology: plasma cells, perinuclear halo Treatment: surgical stabilisation and radiotherapy Poor prognosis with aggressive variants
63
Lymphoma
Lymphoma of bone is uncommon Can be an isolated lesion or a metastatic focus All age groups PC: pain +/- soft tissue masses Common locations: distal femur, proximal tibia, pelvis, shoulder girdle X-rays: bone destruction +/- reactive bone formation, thickened cortex Histology: Marrow replacement with lymphoid cells Treatment: chemo and radiotherapy. Surgery only used to stabilise fractures
64
Most common tumours of bony metastasis
Breast (Sclerotic/Mixed) Lung (Lytic / Mixed) Prostate (Sclerotic/Mixed) Kidney (Lytic) Thyroid (Lytic)
65
How does bony metastasis occur
Bone resorption via activation of Osteoclasts by tumour cells PTHrP, RANK-L, IL-1, IL-6, TNF RANK-L binds to RANK receptor on osteoclast
66
Mirel's scoring system
scoring system for diagnosing impending pathological fractures. 4 parameters (3 points for each parameter- max score 12) Site of lesion Pain Nature of lesion Width of Cortical destruction score of 9 has a 33% chance of fracture Thus think prophylactic stabilisation if score of 8 or above.
67
Soft Tissue Sarcoma (STS)
Malignant tumour of connective tissue Mutiple types: Fibrosarcoma = Fibrous Tissue Leiomyosarcoma = Smooth muscle Rhabdomyosarcoma = Skeletal muscle Liposarcoma = Fatty tissue Synovial Sarcoma
68
What masses are considered malignant in Soft tissue sarcomas
Size >5cm Deep to the Fascia Enlarging in size Painful
69
Management of soft tissue sarcomas
Chemo/Radiotherapy + Surgery Higher % necrosis after neo-adjuvant radiotherapy = better prognosis
70
4 different surgical margins in oncological surgery
Intralesional: The plane of surgery goes through the tumour. Marginal: The plane of surgery goes through the reactive zone of the lesion Wide: The plane of dissection goes through normal tissue. Radical: The entire anatomic compartment of the lesion is removed.
71
Local complications of surgery
Haematoma Loss of function (NV injury) Infection – Superficial / Deep (Silver coating of implants reduces risk) Local Recurrence (especially if intra-lesional margin)
72
Systemic complications of surgery
Risks to general health Distant Metastasis Death
73
Multiple Hereditory Exotosis (Diaphyseal Achalasia)
Characteristic widening of the metaphysis Large sessile osteochondromas arise from the metaphysis Autosomal dominant. The putative tumour suppressive gene mutation is EXT1, EXT2. Low-grade chondrosarcoma occurs in this condition in approximately 10%.
74
Olliers Disease
Multiple enchondromatosis Unilateral lesions Dysplastic appearance of bone 30% risk of sarcomatous change
75
Maffuci Syndrome
Multiple enchondromas in association with angiomas 100% chance of malignant change Association with other tumours: GI tumour, astrocytoma
76
Chordoma
A low-grade malignancy that has a high rate of local recurrence, but a low risk of metastasis. Clasically seen in sacrococcgeal area. Grows anteriorly Early symptoms = pelvic pain Late symptoms = constipation, sacral nerve dysfunction Although the 10-year survival rate is only approximately 25%, the majority of patients die from local spread rather than from metastases. Treatment: Wide surgical resection. Resistant to chemoradiotherapy

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