bone tumours
TRANSCRIPT
Classification based on: Histological grade (G) Site (T) Metastases (M)
ENNEKING'S SURGICAL STAGES
STAGE GRADE SITE METASTASES
1A 1B
Low(G1)
Low(G1)
Intracompartmental(T1)
Extracompartmental(T2)
None(M0)
None(M0)
2A 2B
High(G2) High(G2)
Intracompartmental(T1)
Extracompartmental(T2)
None(M0)
None(M0)
3Low(G1) or
High(G2)
Intracompartmental(T1) Or
Extracompartmental(T2) Yes(M1)
Clinical examination (age, sex, site and past history) ◦ Thyroid ◦ Breasts ◦ Chest ◦ Liver ◦ Kidney ◦ Rectal (prostate & rectal tumours)
Bloods ◦ FBC (leukaemic cells etc) ◦ ESR (often elevated) ◦ Biochemistry (Ca++, PO4, liver enzymes and Alkaline Phosphatase) -> mets ◦ Acid Phosphatase (prostate and increased with metastatic deposits) ◦ Thyroid function tests ◦ PSA ◦ Serum Protein Electrophoresis (Myeloma)
Urinalysis Urine Bence-Jones (myeloma) CXR Abdominal ultrasound Bone scan -> other sites MRI -> soft tissue extent and association with nerves and vessels CT of lesion and chest (-> staging) Angiography -> tumour blood supply and relationship to major vessels Biopsy
Should know probable diagnosis and stage of tumour before biopsy
Performed by the surgeon who will perform the definitive surgery
Biopsy tract orientation & location is critical - will need to be included in the definitive surgery if lesion is malignant.
Meticulous haemostasis to avoid tracking haematomas
Send samples for microbiological analysis
Intra-lesional ◦ through the tumour ◦ leaves macroscopic tumour ◦ not therapeutic
Marginal ◦ through pseudo-capsule of tumour / reactive zone ◦ controls non-invasive benign tumours ◦ recurrence of malignant tumours = 25-50%
Wide ◦ around reactive zone, leaving a cuff of normal tissue ◦ skip lesions left ◦ recurrence of malignant tumours = < 10%
Radical ◦ removal of entire compartment or compartments ◦ distant metastases left
Amputation ◦ should be thought of as a form of reconstruction where
surgical control of the tumour precludes useful function.
Benign Malignant Other
Birth - 5yr
1. Eosinophilic Granuloma [onion skin periosteal Rxn]
2. (Unicameral bone cyst- rare)
1. laeukaemia 2. Metastatic
Neuroblastoma
1. Osteomyelitis 2. healing/ stress
fracture
6-18yr
1. Unicameral Bone Cyst 2. Aneurysmal Bone Cyst 3. Nonossifying Fibroma 4. Eosinophilic
Granuloma 5. Enchondroma 6. Chondroblastoma 7. Chondromyxoidfibroma 8. Osteoblastoma
1. Ewings Sarcoma 2. Osteosarcoma
1. Osteomyelitis 2. Fibrous Dysplasia 3. Osteofibrous
Dysplasia
19-40yr
1. Giant Cell Tumour 2. Eosinophilic granuloma
1. Ewings Sarcoma
40+yrs
1. Metastases (lung, breast, prostate, renal, thyroid, colon)
2. Multiple Myeloma 3. Lymphoma 4. Osteosarcoma
(Pagets) 5. Chondrosarcoma 6. Fibrosarcoma/
Malignant Fibrous 7. Histiocytoma
1. Hyperparathyroidism 2. Osteomyelitis 3. Paget's
FibroxanthomaFibrous cortical defectNon ossifying fibroma
Fibrosarcoma
Fibrous dysplasia
Round cell lesionsEwingsReticulum cell sarcomaMyeloma
Chondromyxoid fibromaChondrosarcoma
Osteoid osteoma
Cortical fibrous dysplasia Adamantinoma
DIAPHYSISDIAPHYSIS
osteosarcoma Enchondroma
Giant cell tumour
osteochondroma
Bone cyst Osteoblastoma
Chondromyxoid fibromaChondrosarcoma
FibroxanthomaFibrous cortical defectNon ossifying fibroma
METAPHYSISMETAPHYSIS
chondroblastoma
Articular osteochondromaDysplasia epiphysealis
hemimelica
Giant cell tumour
EPIPHYSISEPIPHYSIS
Fibrous DysplasiaOsteoblastomaGiant Cell Tumour Metastasis/ Myeloma Aneurysmal Bone Cyst Chondroblastoma/ Chondromyxoid Fibroma Hyperparathyroidism (brown tumour)/ Haemangioma Infection Non-ossifying Fibroma Eosinophilic Granuloma/ Enchondroma Simple Bone Cyst
Vascular ◦ hemangiomas ◦ infarct
Infection ◦ chronic osteomyelitis
Neoplasm ◦ primary
osteoma osteosarcoma
◦ metastatic prostate breast other
Drugs ◦ Vitamin D ◦ fluoride
Inflammatory/Idiopathic Congenital
◦ bone islands ◦ osteopoikilosis ◦ osteopetrosis ◦ pyknodysostosis
Autoimmune Trauma
◦ fracture (stress) Endocrine/Metabolic
◦ hyperparathyroidism Paget's disease
Pattern of bone destruction Tumour matrix Cortical expansion/penetration Periosteal reaction Adjacent soft tissues Size & shape of lesion Trabeculation Growth Plate
Benign lesion - during growth 20% of benign bone lesions Age 5-15 years Not found in adults Sex m:f 3:1 The most common location is the proximal humerus
(67%) followed by the proximal femur (15%) unusual sites (calcaneum, pelvis) in patients >17
yrs
Cysts may be Active or Latent: Active cysts are located near the growth plate, but they move further away as the child grows and become inactive (latent)
Well defined, central osteolytic area with a thin sclerotic margin
Metaphyseal in young - moves towards diaphysis with growth
It fills and slightly expands metaphysis
Pathology Thin walled cavities - blood tinged fluid. The lining cells are cuboidal,
Treatment goal is to minimise fracture risk until the cyst heals (but this can take years)
Steroid injection ◦1-3 percutaneous injections repeated at 2
monthly intervals ◦60-80% success rate
Curettage and bone graft - 50% recurrence rate and possibility of damage to the growth plate
Bone marrow aspirate has recently been used
Benign solitary, expansile and erosive lesion of bone 1% of benign bone lesions Age (85% cases <20 years old) Sex f:m is 2:1 ABC's can be found in any bone in the body The most common location is the metaphysis of the lower extremity long bones, more so than the upper extremity The vertebral bodies or arches of the spine may be involved Approximately one-half of lesions in flat bones occur in the pelvis
Presentation Swelling, tenderness and pain Limited range of motion due to joint obstruction Spinal lesions - neurological symptoms Pathological fractures are rare - eccentric location of the lesion
Placed eccentrically in the metaphysis and appears osteolytic
The periosteum is elevated; cortex is eroded to a thin margin
The expansile lesion - "blow-out” CT scan -for pelvis or spine lesions CT scan can demonstrating multiple fluid-fluid levels MRI can also confirm the multiple fluid-fluid levels
A slow growing, indolent ABC has been observed to regress spontaneously
Most lesions can be treated with curettage and application of a high-speed burr
Recurrence was statistically related to young age and open growth plates, and may be less likely following wide excision than following curettage
Benign, usually solitary and locally aggressive
10% of benign bone lesions malignant transformation (5-10%) Not seen until after the growth plate
closes Rarely metastasises (<1% to lungs) Age 20 - 40 years More common females Most commonly seen in the distal
femur, proximal tibia and the distal radius
Nearly always located at the very end of a long bone (metaphyseal / epiphyseal)
Pathological fracture occurs in 10 - 15% Neighbouring joint often irritated
(effusion)
stagestage clinicalclinical radiologyradiology histologyhistology
II asymptomaticasymptomatic benignbenign benignbenign
IIII symptomaticsymptomatic activeactive benignbenign
IIIIII symptomaticsymptomatic AggressiveAggressive
Mets +Mets +
benignbenign
Usually well defined lesion in the epiphysis extending up to the joint surface without marginal sclerosis, cortex thinned and sometimes ballooned
soap bubble appearance Junction with normal bone
poorly defined
Soft, friable tumour Cut surface tan in colour,
with areas of necrosis and haemorrhage
Numerous multinucleated giant cells. The stromal cells are homogenous mononuclear round/ovoid with large nuclei
Up to 50% have soft tissue extension
Intralesional excision by "extended" curettage Curettage alone has a high local recurrence rate (50%) and
the curettage is "extended" into the bone by a few millimetres by either using a burr, liquid nitrogen or phenol
The resulting cavity can be filled with bone graft or cement
En-bloc resection is possible if the bone is expendable e.g. proximal fibula, proximal radius
Amputation reserved for massive local recurrence, malignant change or infection
Radiotherapy reserved rare cases of unresectable tumours because of increased risk of secondary malignancy
10% of benign bone tumoursMale : Female 2:1Peak age 5 - 25 years (85% in this range)Rare over 40 years
Location: Any bone, rarely multifocal tibia & femur in 50% spine - posterior elements Only occurs in bones formed by endochondral ossification
Clinically Pain - commonest presentation
Pain - worse at night and relieved by aspirin10% occur in the spine Runs a self limiting course > surgery for pain reliefPain usually decreases as the lesion maturesLesion healed by 3 - 7 years
Lytic nidus surrounded by sclerotic bone (which may mask the nidus)Centre of nidus may be calcifiedCT or tomograms -> diagnosis Hot spot on bone scan
Differential Diagnosis Bone island (enostosis) Brodie's abscess Osteoblastoma fatigue fracture
NSAIDs ◦ relieves symptoms
Surgical: ◦Nidus excision -> no recurrence◦ Intraoperative localisation with:
Bone scan Tetracycline under UV light) CT X-Ray excised tissue -> contains nidus
Percutaneous radiofrequency coagulation
Cartilage capped bony projection / exostosisCommonest benign tumourDevelopmental abnormality of the metaphysis
Accounts for 45% of benign bone tumours
12% of all bone tumours most become evident under 20 years May be solitary or multiple (diaphyseal
aclasis) Any bone developing by endochondral
ossification may be involved
Autosomal dominant Disordered endochondral growth Multiple osteochondromas Short stature and bowing of limbs Treat individual lesions as
necessary and observe for malignant change
Malignancy Risk = ~ 20% overall or 0.2% per lesion
Trevor's Disease: Osteochondroma on epiphyseal side of the growth plate
x-ray hallmark is blending of tumour into underlying metaphysis
flat, sessile lesion or a peduculated (stalk like) process
pedunculated osteochondromas are oriented in proximal direction
Cartilaginous cap displays irregular areas of calcification
Nil required unless symptomatic (persistent irritation (from bursitis or tendon) or neurovascular compromise)
Extra capsular marginal excision ◦ Including the cartilaginous cap & overlying
perichondrium ◦ Deep bony base has minimal activity & may be
removed piecemeal ◦ The cartilaginous cap should not be traumatised
during removal ◦ Recurrence = < 5%
Decreased risk of recurrence if excised after maturity
Risk of malignant change ~ 0.2% in a solitary lesion
Risk of malignant change in diaphyseal aclasis 20%
Sarcomatous change usually ->low grade Evidence of transformation to
Chondrosarcoma: ◦ Cartilaginous cap thicker than 1 cm in an adult
(in child may be 2-3 cm thick) ◦ Cartilage cap > 8cm diameter ◦ Fluffy outline ◦ Bone scan - Marked increase in uptake in an
adult ◦ CT/MRI - soft tissue mass or displacement of a
major neurovascular bundle
10% of benign bone tumours 50% occur in small bones of the hands and
feet 15% femur and 12% humerus Peak incidence 10 - 50 years May be solitary or multiple (Olliers, Mafuccis)
Clinically Usually metaphyseal 75% Solitary 60% present as fractures pathological fracture, lump incidental finding
X-Rays Scalloped erosions on endosteal surface
flecks of calcification - sometimes called 'ground glass'
enchondroma (typical appearance & site)
Macroscopically - bluish white well demarcated
hypocellular; nests of mature cartilage cells,
Ollier's disease - more cellular; 50% ->malignant transformation
Mafucci's disease - associated with multiple haemangiomata and associated with nearly 100% malignant change somewhere
Observe - x-ray 6 months & 1 year after presentation
Curettage and grafting if latent Recurrence - en block excision Prognosis Risk of malignant change in Olliers is 50% malignant change in Mafuccis is nearly
100%
5 - 20% benign bone lesions usually monostotic Affects children and adolescents Median age at onset 8 years Male > Female (Albrights - Female > Male)
McCune - Albrights Syndrome Polyostotic disease (unilateral usually) Skin pigmentation
◦ cafe au lait spots with serrated borders (called "coast of Maine") that tend to stop abruptly at the midline of the body
Precocious puberty (endocrinopathy) usually presents earlier, may be unilateral or widespread,
affecting long bones, hands, feet & pelvis Malignant transformation (chondrosarcoma or osteosarcoma) is
about 4 %;
Lucent lesion in medullary space Sclerotic margin. Ground glass appearance No periosteal reaction Shepherds crook - proximal femur expansion of cortex
Pathology Bone replaced by firm, whitish
tissue of gritty consistency bone trabeculae separated by
fibrous tissue. Bone is woven rather than lamellar
Pagets disease FCD Hyperparathyroidism osteoblastoma osteosarcoma
Treatment Monostotic -> curettage and grafting if symptomatic Polyostotic -> symptomatic treatment May require osteotomy for deformity or lengthening / shortening
procedures
Prognosis Monostotic lesions cease activity at puberty but may be reactivated
by pregnancy Polyostotic - 85% -> pathological fracture malignant change occurs after radiotherapy