-
5 carcinomas that commonly spread to bone
- Breast
- Lung
- Thyroid
- Renal
- Prostate
-
3 most common sites of bone mets
- Spine #1: most common thoracic
- Proximal femur #2
- Humerus #3
-
Most common site of fracture secondary to mets
Proximal femur
-
Mechanism of action of osteolysis in bone mets
Tumor induced activation of osteoclasts: Through RANKL pathway
-
Metastatic hypercalcemia: 4 treatment options
Hydration: volume expansion
Loop Diuretics
Bisphosphonates
calcitonin: inhibit osteoclast and increase renal excretion of calcium
-
Why is the axial skeleton the most common site of mets
There is vascular spread through Batson's vertebral plexus: Valveless venous plexus of the spine that provide a route for mets from organs to axial structures
-
Mechanism of bone sclerosis in bony mets
Activation of WNT pathway: for example prostate cancer secretes endothelin 1
-
5 symptoms of metastatic hypercalcemia
- COnfusion
- Muscle weakness
- Poluuria/polydipsia
- Nausea/vomiting
- dehydration
-
2 bony mets that are cold on bone scan
Thyroid and myeloma: evaluate with skeletal survey
-
If there is metastatic adenocarcinoma not identified by CT, what is the most likely source
Small cell lung cancer
-
Most likely primary: Cortical mets
Lesions distal to elbow and knee
-
2 cancers that require pre op embolization
-
GCT: gender
Age
Location
Where does it metastasize
- Female > male
- 30-50
- Distal femur > proximal tibia > distal radius > sacral ala
- Lung: usually benign
-
GCT lesions that have greatest chance of metastasis
Hand lesions
-
eccentric lytic epiphyseal/metaphyseal lesion that often extends into the distal epiphysis and borders subchondral bone: Diagnosis
GCT
-
Medical management of GCT
Bisphosphonates: osteoclast inhibitors...may decrease size of defect
Denosumab: Rankl inhibitor > shows sclerosis and reconstitution of cortical bone
-
GCT: malignant transformation to
Location in the spine
If suspecting, always rule out
HIgh grade sarcoma: poor prognosis
Anterior elements
Teleangectatic osteosarcoma
-
DDx benign aggressive tumors 5 + how to treat it
- GCT
- CHondroblastoma
- Aneurysmal bone cyst
- Osteoblastoma
- Chondromyxoid fibroma
Treatments is extended intralesional curetage and bone grafting
-
5 causes of secondary chondrosarcoma
- Osteochondroma: <1% risk
- Multiple hereditary exostosis: 1-10% transformation
- Enchondromas: 1%
- Ollier's disease: 25-40 risk malignant transformation
- Maffucci's: 100% risk malignant transformation
-
Chondrosarcoma: what correlates to rate of recurrence
2 subtypes
RT-PCR showing increased telomerase activity
Clear cell chondrosarcoma: can look like low grade chondroblastoma
Mesenchymal chondrosarcoma: needs neoadjuvant chemo followed by wide resection
-
Treatment of: low grade chondrosarcoma
High grade chondrosarcoma
Mesenchymal chondrosarcoma
- Intra-lesional curettage
- WIde excision: no chemo or radiotherapy
- Chemotherapy + wide excision
-
A benign dysplasia that leads to unilateral varus of the tibia: Dx
treatment
Focal fibrocartilagenous dysplasia: classic consists of a lucency in the medial cortical border of the metaphysis
Observation: varus will correct with growth
-
medullary lesion of sheet-like central lucency surrounded by sclerosis with a serpiginous border : diagnosis
Bone infarct: also looks like smoke up chimney
-
Angiosarcoma: Prognosis
Treatment
Tumor marker
- Poor: very aggresive
- Wide excision: does not respond to chemo or radtx
- CD31/CD34
-
Most common solid tumor in children
Neuroblastoma
-
Neuroblastoma: Location
Age
treatment
- CLose to adrenals or spinal cord: from sympathetic neural tissue
- Children < 2
- Neoadjuvant chemo, stem cell transplant and surgical excision
-
5 small round blue cell tumors
- WIlms
- Rhabdomyosarcoma
- Neuroblastoma
- Non hodgkin lymphoma
- Ewing sarcoma
-
Lederhosen disease: what is it
Plantar fibromatosis similar to dupuytrens
-
Undifferentiated pleomorphic sarcoma: histology
Treatment
Spindle cells in cartwheel pattern
Wide local resection and adjuvant radiation: if tumor >5cm add radiation
-
Teleangectatic osteosarcoma: looks like
Behaves like
Treatment
Good prognostic sign
- ABC
- Regular osteosarcoma
- Neoadjuvant chemo and limb salvage resection: chemo is doxy/cisplatin/methotraxate/fosamide
- 98% necrosis in biopsy: good prognosis
-
Melanoma 5 poor prognostic factord + most important
- Depth: most important..if <0.7 mm 96%..if >4mm 47% (breslow classification)
- Male sex
- lesion on neck or scalp
- Positive lymph nodes or mets
- ulcerations
-
Melanoma:Cell origin
Neural crest cells
-
Melanoma treatment if: <1mm thick
1-2mm thick
Subungual melanoma
- Local resection with 1 cm margin
- Local resection with 1-2 cm margin and sentinel node biopsy
- Amputation
-
DDx of an epiphyseal/subchondral lesion 4
- CHondroblastoma
- Infection
- GCT
- Clear cell chondrosarcoma
-
DDx of sacral lesion on young patient 5
- GCT
- ABC
- Ewings
- CHordoma
- Osteosarcoma
-
4 primary bone tumors treated with wide resection alone
- Chondrosarcoma
- Paraosteal osteosarcoma: low grade only
- Chordoma
- Adamantinoma
-
dripping candle wax” appearance with dense hyperostosis that flows along the cortex of the bone: diagnosis
Melorheostosis
-
Melorheostosis: Typical appearance
Prognosis
Treatment
- Dripping candle wax
- Benign
- Symptomatic: treat if severe contractures,limited mobility, pain
-
Leiomyosarcoma: cell origin
Treatment
Smooth muscle lining blood vessels
Early wide resection +/- chemo
-
Mazabraud syndrome: what is the pathology
Multiple intramuscular myxomas associated with fibrous dysplasia
-
Intramuscular fibromas: are
Differentiate from myxoid liposarcoma
Treatment
Benign soft tissue mass confined to skeletal muscle: intramuscular
Liposarcomas are intermuscular
Marginal excision: if symptomatic
-
Marjolin ulcer: is
SCC resulting from chronic draining wounds or burn scars
Rate of transformation is 1%
-
GLomus tumor: location
symptoms 3
3 physical exam findings
treatment
Subungual region
- Paroxysmal pain
- Exquisite tenderness to touch
- Cold intolerance
- Small bluish nodule: difficult to see if subungual
- Love test: pressure with pinhead leads to exquisite tenderness
- Hildreth test: Tourniquet inflation reduces pain and tenderness caused by love test
Marginal excision is curative
-
Soft tissue mass with phlepboliths: dx
Need to rule out
Treatment options 3
- Soft tissue hemangioma
- Angiosarcoma: use MRI
- Non-op: NSAID's stockings, activity modification
- Sclerotherapy or embolization
- Surgical excision
-
Tumoral calcinosis: pathophysiology
What is a secondary cause of it
Treatment
Metabolic dysfunction of phosphate regulation: leads to periaricular calcinosis in extracapsular soft tissues
Renal failure
Treat like HO: Remove once mature
-
Difference btw neurofibroma and schwanoma
Neurofibroma: from non-myelating schwann cells as well as fibroblast (predominant)
Schwannoma: Schwann cells alone
- ON MRI
- Schwanoma: eccentric to nerve
- Neurofibroma: Central to nerve fibers
-
Plexiform neurofibroma: dx
Neurofibromatosis
-
7 diagnostic criteria for NF
- 2 or more neurofibromas or one plexiform neurofibroma
- Axillary/groin freckling
- 6 or more cafe au lait spots: 5mm if prepubertal or 15mm in postpubertal
- Sphenoid wing displasia or CPT
- 2 or more lisch nodules
- Optic glioma
- 1st degree relative with NF1
-
Neurofibroma: can transform to
Rate malignant transformation
- MPNST
- 5% of patients with NF
-
MPNST: arise from
treatment
Stains positive for
- Neurofibromas
- Wide surgical resection +radiation
- S100
-
Ostoblastoma: location
Size
Associated with
NSAIDs
Management
- Most common in posterior elements spine
- >2cm osteoid osteoma: has a nidus
- Secondary ABC
- Does not respond to NSAID's
- Surgery: does not respond to non op
- Curetage or marginal excision with bone grafting
-
Difference btw pre and post op RT:
Wound complications
Edema/stiffness/fibrosis
Radiation field
Pseudocapsule
Tumor shrinkage
Local recurrence
-
Radiotherapy sideeffects: 3 early
3 late
- Delayed wound healing
- Infection
- Desquamation
- Stiffnes
- Secondary sarcoma
- Fractures
-
6 risk factors for post radiation fracture
- Radiation dose >59 Gy
- Weight bearing bones: femur
- Female
- Anterior femoral compartment excision
- Periosteal stripping
- Osteoporosis
-
Most common primary spinal tumor in adults
Chordoma
-
Chordoma: most common location
Metastasis to
Physical exam
Treatment
- Sacrum and coccyx in midline
- Lung: 30-50%
- Palpable on rectal
- Wide resection including nerve roots: radiation if negative margin not achieved
-
Chordoma: how to preserve bowel/bladder function
- Bilateral S2 nerve roots
- Unilateral S2-4 nerve roots
-
Malignant Fibrous Histiocytoma (MFH): AKA
Presentation
Location
Risk factors
Mutation
Good prognosis
Treatment
- Undifferentiated pleomorphic sarcoma
- Similar to osteosarcoma: but lacks osteoid formation
- Most common metaphysis of long bones
- 25% arise from bone infarcts, pagets, radiation
- P53
- >90% necrosis to neoadjuvant chemo
Neoadjuvant chemo + wide resection + post op chemo +/- radiation(if questionable margins)
-
Chondrommyxoid fibroma: location
Mutation
Negative prognostic factors 2
Treatment
- Long bone metaphyseal eccentric: lytic
- Chromosome 6 (q13)
- Children + lobulated tumor with abundant myxoid material
- Extended intralesional curetage and bone grafting
-
Primary bone lymphoma: most common type
Most common sites
Prognosis
Appearance x ray
Histology
Treatment
- Non hodgkin b cell lymphoma
- Pelvis, spine, ribs
- Better than secondary lymphoma of bone
- Large ill defined diffuse lytic lesion with mottled appearance
- Small round blue cells
- Multiagent chemo +/- radiation (in persistant disease)
-
Jail bar vertebrae: dx
Hemangioma
-
Hemangioma:2 interventions for symptomatic patients
- Curetage and bone grafting
- Low dose radiation: 25-40Gy)>>if lesion is not accesible
-
3 advantages of prophylactic fixation over pathological fracture fixaton
- Shorter operative time
- Decreased morbidity
- Quicker recovery
-
-
4 factors that affect prognosis of bone tumors in general
- Metastasis
- Skip lesions
- Histologic grade
- Tumor size
-
Describe the ennekin classification of benign bone tumors
- Latent: NOF, enchondroma
- Active: ABC, UBC, Chondromyxoid fibroma, chondroblastoma
- Aggresive: GCT
-
Describe the 3 variables in the ennekin classification of malignant tumors
- Grade: low or high
- SIte: Intracompartmental (within bone cortex) or extracompartmental (outside bone cortex)
- Mets: yes or no
-
Schwannoma: common location
Mutation
MRI finding
Histology pathogomonic finding
Treatment
- Flexor surface of extremities and head and neck
- NF-2
- String sign
- Verocay bodies
- Excision if symptomatic
-
Synovial chondromatosis: Pathophysiology
most common location
Problem
Treatment
MRI finding
- Metaplasia: synovial transformation to chondroid tissue
- Knee
- Multiple intra articular loose bodies
- Symptomatic
- Cartilage nodules only visible on MRI initially
-
Most common sarcoma in children
Rhabdomyosarcoma
-
Rhabdomyosarcoma: 4 subtypes
Best survival
Fatal
Embryonal: infants
Alveolar: Adolescents
Botryoid: infants..in vagina
Pleomorphic: older patients
- Embryonal has best survival
- Botryoid is fatal
-
Rhabdomyosarcoma: Histology
Treatment (old vs young)
- t2:13 translocation
- Small round blue cells
In young: Wide excision and chemo
In Old:Wide excision and radiotherapy
-
Most common soft tissue sarcoma in the foot
Synovial sarcoma
-
extra abdominal desmoid tumor: 2 associated syndromes
Most common location
Lab test that is high
metastasis
Medical management
Surgical mgmt
- Gardner and familial adenomatous polyposis
- Shoulder>chest wall/back>thigh
- Beta catenin
- No risk
- Low dose chemo or tamoxifen: since it has 100% estrogen receptor
- Wide excision with radiotherapy
-
Principles of tumor biopsy 5
- Longitudinal incision in line with extensile approach
- Avoid neurovascular structures
- Maintain hemostasis
- Through one compartment only
- Drain in line with incision
-
Most common soft tissue sarcoma in the hand and wrist
Epithelioid sarcoma
-
Epithelioid sarcoma: age
Presentation
Treatment
- Adolescent and young adults
- Painless growing mass
Wid excision +adjuvant radiotherapy + sentinel LN biopsu
-
Most common: Site of fracture secondary to mets
Solid tumor in children
Primary spinal tumor in adults
Sarcoma in children
Soft tissue sarcoma of the hand
Soft tissue sarcoma of the foot
Benign bone tumor
- Proximal femur
- Neuroblastoma
- Chordoma
- Rhabdomyosarcoma
- Epithelioid sarcoma
- Synovial sarcoma
- Osteochondroma
-
Adamantinoma: Is
Location
Age
Mets
Classic imaging
Treatment
- A low grade malignant tumor
- Mid tibia: anterior cortex
- Yong adults: 20-40
- Can go to lung: 25%
- Soap bubble appearance: multiple sharply circumscribed lucent lesions with interspersed sclerotic bone in the mid tibia
- Wide surgical excision:often requires intercallart resection
-
3 types of langerhans cel histiocitosis
- EG: single self limiting lesion in younger patients
- Hand Schuller Chirstian: Disseminated LCH with bone and visceral involvement
- Letterer-siwe disease: fatal...occurs in young children
-
Hand schuller christian: what is it
classic triad
Treatment
Type of langerhans cell histiocytosis
- Lytic skull lesions
- Diabetes insipidus
- exophthalmos
Chemotherapy and referal to pediatrics for assessment of systemic disease
-
EG: histology
Mgmt
Coffee bean idented nuclei
COnservative: usually a self limiting entity
-
Paraosteal osteosarcoma: location
Prognosis
x rays
treatment
- Metaphysis of long bones: most common posterior distal femur
- 95% long term survival if local control achieved
- Heavily ossified lobulated mass arising from cortex: appears stuck on cortex
Wide local excision: no need for chemo unless high grade component on histology
-
Osteofibrous dysplasia: location
deformity
x ray
problem with this pathology
- Anterior tibia cortex
- Anterior or anterolateral bowing of tibia
- Anterior eccentric lytic lesion in tibia
- Pseudoarthrosis: 10-30%
-
Myositis ossificans:caused by 2
2 most common locations
Natural history
Non op mgmt
- Direct trauma/intramuscular hematoma
- Quads/brachialis
- Mass begins to decrease after one year
- Rest and activity modification: avoid passive stretching
-
4 indications for chemotherapy in orthopaedic oncology
- Osteosarcoma
- Dedifferentiated chondromsarcoma
- Malignant fibrous histiocytoma
- Ewing sarcoma
-
Side effect: Doxyrubicin
bleomycin
CYclophosphamide
- Cardiotoxicity: leads to CHF
- Pulmonary fibrosis
- Myelosupression
-
multiple myeloma: types of proteins
Heavy chains (IgG and light chain (kappa or lambda)
-
MOst common primary bone malignancy
Multiple myeloma
-
5 poor prognostic factures in multiple myeloma
- CHromosome 13 deletion or translocation
- CIrculating plasma cells
- increased beta 2 microglobulin
- Decreased serum albumin
- INcreased marrow microvessels
-
Multiple myeloma mnemonic for end organ damage
CRAB
- Hypercalcemia
- renal insufficiency
- Anemia
- BOne lesions
-
Multiple myeloma: labs to order 4
- SPEP/UPER
- CALCIUM
- HEMOGLOBIN
- CREAtinine
-
Multiple myeloma: medical management 3
- Chemotherapy
- Stem cell transplant
- Bisphophonates: reduce skeletal events
-
Multiple myeloma: cell type involved
type ofimmunoglobulin
If only one lesion
- Plasma cell
- Light chains
- Solitary plasmocytoma
-
Heterotrophic ossification: most common location
Pathology associated with elbow HO
- Between muscle and joint capsule: hip>knee>elbow>shoulder
- Brain trauma
-
Heterotrophic ossification risk factors
In acetabular #
In THA
in TKA
- HIgh ISS
- TBI: in spastic limb
- SCI: compete >incomplete
- Prolonged coma
- Decubitus ulcers
- DISH/ank spond
- Femoral nail: worse if piriformis entry
- Surgical approach: iliofemoral > kocher langenbech > ilioinhuinal
- THA approach: Anterior > lateral > posterior
- TKA if notching or periosteal stripping
-
Best test for early diagnosis of HO
Triphasic bone scan
-
Heterotrophic ossification abnormal labs (4)
- ALP: increased > removes inhibitors of mineraization
- CRP: Elevated >>once normalized may be sign of maturity
- ESR: elevated 12 weeks after THA is predictor for HO
- CK: correlates with muscle involvement
-
Heterotrophic prophylaxis 2 types
INdomethacin 75mg/day for 10 days to 6 weeks: literature seems to show no great benefit
Radiotherapy: single dose of 700cGy within 72 hrs of surgery
-
Most common benign bone tumor
Osteochondroma
-
Osteochondroma: Pathophysiology
ages
Inheritance
Gene
Malignant transformation to
- Derived from aberrant cartilage from the perichondral ring
- 10-20 years old
- Autosomal Dominant
- EXT gene: loss of regulation of indian hedgehog
- Low grade chondrosarcoma
-
3 causes of solitary osteochondromas
- SH fracture
- Radiation
- Surgery
-
Multiple hereditary exostosis: Inheritance
Gene
Which gene is worse
malignant transformation %
- Autosomal dominant
- EXT1-3
- EXT 1
- 5-10%: proximal lesions more likely than distal lesions
-
osteochondroma: xray description
Cortex of lesion in continuity with cortex of native bone...medullary cavity also in continuity with native bone
-
4 associated deformities caused by MHE
Osteochondromas lead to
- Subluxation radiocapitellar joint
- LLD
- Valgus deformity knee
- Ulnar deviation of wrist
-
Most common primary sarcoma of bone
Intramedullary osteosarcoma
-
Intramedullary osteosarcoma: IN elderly associated with
Most common location
Location of mets
Genetic mutation
- PAgets disease
- Distal femur/proximal tibia
- Lung
- Mytation in Rb gene
-
6 poor prognostic factors in intramedulalry osteosarcoma
- Advanced stage
- Response to chemo: tumor necrosis from sample
- Tumor size
- High serum alk phos
- High LDH
- +ve margins
-
Intramedullary osteosarcoma: classic x ray findings 3
- Sunburst periosteal reaction
- Codman triangle
- Blastic destructive lesion
-
Intramedullary osteosarcoma: treatment
good prognostic factor
Multi agent chemotherapy and wide resection
- Pre op chemo: 8-12 weeks
- Post op chemo: 6-12 months
Tumor necrosis >98%: good prognosis
-
Ewing sarcoma: age
Location
Genetics mutation
Abnormal protein
histology
- 5-25 years
- 50% in diaphysis of long bonesTranslocation T11:22
- EWS-FLI1
- Small round blue cells
-
Ewing sarcoma 8 poor prognostic factors
- Tumor size > 8cm or 100cm3
- Spine and axial worse than proximal extremity worse than distal extremities
- Age >14
- Male
- Elevated LDH
- p53 mutation
- Metastases: MOST IMPOSTANT PROGNOSTIC FACTOR
Chemo responce: <90% necrosis
-
Ewing sarcoma 2 classic x ray findings
- onion skinning
- sunburst priosteal reaction
-
Ewing sarcoma treatment
Neoadjuvant chemo + wide resection + adjuvant chemo +/- adjuvant radiation
Radiation of: positive margins, metastasis, poor chemo response
-
3 complications of medical management of ewing sarcoma
- Bone sarcoma: secondary to radiotherapy ...risk 10-20% at 20 years
- Treatment related acute myeloid leukemia/myelodysplasia
- Metastases
-
myxoid liposarcoma: translocation
t12:16
-
Liposarcoma and risk of mets: 3 categories
- Low grade (well differentiated(: < 1%
- Myxoid (intermediate grade): 10-30%
- High grade: >50%
-
Atypical lipomatous tumor: stain for
MDM2/CDk14
-
Treatment of liposarcoma
Low grade (ALT): wide resection>>>low risk of mets/dedifferentiation
Intermediate or high grade
Wide surgical excision and adjuvant radiotherapy
-
Pre chemotherapy work up
- Fertility consult
- Echocardiogram
- CBC
- 24hr urine
-
SOft tissue sarcoma:site of mets
Lng
-
Soft tissue sarcoma: 3 negative prognostic factors
- High grade
- size >5cm
- Tumor deep to the fascia
-
Soft tissue sarcoma: treatment
Radiation and wide surgical excision
50-60 Gy
-
Soft tissue sarcoma: radiation dose
If given pre
If given post
- 30% wound complication
- Greater radiation induced morbidity and chance of radiation induced sarcoma: need bigger fields
-
Radiation dose for: adjuvant soft tissue sarcoma
Mets
POst op HO prophylaxis
PVNS
- 50-60 Gy
- 30 Gy
- 6 Gy
- 30-35 Gy in 15 fractions or 50Gy in 25 fractions
-
3 cancer lets tthat are purely lytic
Renal, thyroid, lung
-
Spine metastasis: how to determine life expectancy
2 options in management
Tokahashi score: >8 means >6 months to live
palliative: pain control and radiotherapy
SUrgery: decompression stabilization and post op radiation
-
Chondroblastoma: age
Location
Matrix
Mets
x ray finding
HIstology
Treatment
- 80% age <25
- Epiphyseal: distal femur and proximal tibia most common
- CHondroid on histology
- To lung but are benign: similar to GCT
- Well circumscribed epiphyseal lytic lesion with rim of sclerotic done: can have cortical expansion, punctate calcification not always present
- Cobblestone or chickenwire pattern
- Extended intralesional curetage and bone grafting
-
Pagets disease: pathophysiology
Casued by
Inheritance
Medical problem
Malignant transformation to 3 things
- Abnormal bone remodelling: excessive bne resorption and abnormal bone formation
- Paramyxovirus or RSV
- Mostly spontaneus: if inherited its autosomal dominant
- Hiho output cardiac failure
Osteosarcoma, fibrosarcoma and chondrosarcoma
-
Pagets disease 5 ortho manifestations
- Bone pain
- Long bone bowing
- Fractures: tend to be transverse
- Large joint arthritis
- Secondary sarcoma
-
COtton wool exudates in skull: dx
Pagets disease
-
Pagets disease lab values: ALP
calcium
-
Pagets symptomatic medical management
Aim for osteoclast inhibition
- Bisphosphonates
- Calcitonin: decrease size and resorptive capacity of osteoclasts
Teriparatide is contraindicated bc of risk of secondary sarcoma
-
Periosteal osteosarcoma: location
Mutation
Good prognosis
Poor prognosis
x ray
Histology
Treatment
- diaphysis of long bones
- p53
- 98% tumor necrosus
- Expression of MDR gene: pumps chemo out of cell
- Periostal sunburst or hair on end reaction
- Osteoid and chondrobalstic matrix
Neoadjuvant chemo >restage > wide resection > post op chemo
-
PVNS:histology
location
If in hand
Genes affected
x ray findings
MRI
Arthroscopic appearance
- Proliferation of synovial villi and nodules: hemosiderin stained multinucleated giant cells
- Intra articular: Knee > hip > ankle
- Giant cell tumor of nerve sheeth
- 5q33: increased CSF1 expression
- Csystic erosion with sclerotic margins on both sides of joint
- Blooming artifact: becasue of iron in hemosiderin deposits
- Brownish/red inflammaed synovium
-
PVNS treatment
COmbined open/arthroscopic synovectomy: marginal excison
In giant cell tumor of nerve sheeth: marginal excison is ok but recurrence is high
External beam radiotherapy: reduces rate of recurrence to 10-20%
-
UBC: caused by
Age
Location
What happens at skeletal maturity
- Temporary failure of medullary bone formation near physis<20
- Metaphysis adjacent to physis: proximal humerus most common
- UBC decrease in size and may heal
-
UBC classification
Latent: if normal bone separates UBC from physis
Active: Cyst adjacent to physis
-
UBC xray findings 3
- Central, lytic, well demarcated metaphyseal lesion
- Cystic expansion with symmetric thinning or cortices: not wider than physis
- Fallen leaf sign: path fracture with fragment at the base of empty cyst
-
UBC treatment
Immobilization after path fracture: 15% heal after farcture
Aspiration and methylprednisone injection: for active cysts...may require multiple injections
Curetagge, bone grafting and internal fixation: avoid in active UBC bc communication with physis may lead to growth arrest
-
ABC: age
Location in spine
x ray description
MRI finding
- <20
- Posterior elements
- Bubbly appearance: expansile, eccentric lytic lesion with bony septae
- FLuid/fluid levels
-
ABC associated with 5 other tumors
- GCT
- CHondroblastoma
- Fibrous dysplasia
- Chondromyxoid fibroma
- NOF
-
ABC always think to rule out
Teleangectatic osteosarcoma
-
ABC treatment: if fracture
If no fracture and symptomatic
Non op until fracture heals
Agressive curetage and bone grafting +/- adjuvants
- Phenol
- Argon beam
- Liquid nutrogen
-
Synovial sarcoma: location
Gene mutation
Metastasis
Most important positive prognostic factor
Treatment
- Arises near joints but rarely within joint: most common sarcoma of the foot
- Translocation t(x:18): forms SYT-SSX1/2
- Common (30%-60%): goes to lung
- SYT:SSX2: better prognosis
- Wide resection and adjuvant radiotherapy: chemo may be beneficial
-
Sarcomas that can metastatize to LN 5
- Synovial
- Epithelioid
- Angiosarcoma
- Rhabdomyosarcoma
- Clear cell
-
Osteoid osteoma location: lower extremity
Spine
Hand
Foot
- Proximal femur > tibial diaphysis
- Posterior elements: Thoracic and lumbar > cervical in concavity of scoliosis
- Scaphoid and proximal phalanx
- Talar neck
-
Osteoid osteoma: histology
Symptoms
ETOH
Size
Resolution of scoliosis if
- Nidus containing osteobalsts surrounded by sclerotic zone (reactive zone)
- pain worse at night and relieved by NSAID's
- Makes symptoms worse
- <2cm
- Early resection within 18 months in kids <11
-
Osteoid osteoma vs OSteoblastoma:
Incidence
Size
Site
Location
Natural historrt
Symptoms
Management of spine lesions
-
Osteoid osteoma: non op mgmt
Operative
NSAID's until it resolvea
RFA: 90% succesfully treated with 1-2 sessions of rfa
Surgical resection and curetage: digital lesions (RFA would burn skin) or lesions close to nerves/vessels (in spine)
-
MOst common benign tumor in childhood
NOF
-
NOF: incidence
Location
associated syndrome
x ray finding
Treatment
- 30% of children with open physis have NOF
- Metaphysis of long bones
- Jaffe-campanacci syndrome: multiple NOF, cafe au lait, mental retardation, heart eyes and gonads involved
Metaphyseal eccenttic buccly lesion surrounded by sclerotic rim: as child grows lesion migrates towards diaphysis
Observation and follow up: curetage and bone grafting in the case of pathologic lesion
-
Enchondroma: matrix composition
Most common location
Pathophysiology
X ray appearance
Treatment
- Hyaline cartilage
- Hand (60%) > feet
- Chondroblasts escape from the physis into medullary canal and begin proliferating there
- Well defined central lucent medullary lesions that calcify over time (pop corn)
Serial x rays for n patients and long term follow up in mafucci or olliers disease
-
Most common bone tumor in the hand
ENchondroma
-
Enchondroma 2 associated conditions
Ollier's disease: Multiple enchondromatosis >>risk malignant tranformation < 30%
Maffucci syndrome: Multiple enchondromas and sost tissue angiomas.....enchondromas expand cortices and angiomas seen as phleboliths >>>malignant tranformation 50-100%..can also have visceral malignancies
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Genetic inheritance: olliers disease
maffuccii syndrome
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DIfference btw enchondroma and chondrosarcoma
- ENchondroma erosion cortex <50% thickness
- Chondrosarc erosion cortex >50% thickness (scalloping)
- Chondrosarc leads to cortical thickenning and expansion...enchondroma lead to cortical thinning and expansion
- Chondrosarc are larger: >5cm
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Fibrous dysplasia: pathophysiology
Most common site
Gene mutation
- Failure of normal production of lamellar bone
- Proximal femur
- GS alpha protein:increased cAMP production
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Fibrous dysplasia 3 associated syndromes
- McCune albright: cafe au lait, endocrinopathy, unilateral polyostotic FD
- Mazabraud syndrome: polyostotic FD and soft tissue intramuscular fibromas
Osteofribrous dysplasia: Location anterior tibial diaphysis > bowing
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Fibrous dysplasia: malignant transfomation to 3
MFH, fbrosarcoma, osteosarcoma: 1%
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Fibrous dysplasia x ray findings
- Central lytic lesions in medullary canal: may have cortical thinning and be expansile
- Ground glass appearance
- Sheppard crook deformity
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Fibrous dysplasia: non op mgmt
Op magmt
Observation if asymptomatic
Bisphosphonates: in symptomatic polyostotic FD >>decreases pain and bone turnover
ORIF with bone grafting, never use autograft, best choice is cortical allograft...Use nail if possible
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