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62 Cards in this Set
- Front
- Back
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Factor most correlated with mets
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Grade of tumor
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Most common dx for mets in <5yo
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Neuroblastoma/ Wilm's tumor
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Tumor margins
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1. Intralesional. 2. Marginal- through reactive zone. 3. Wide- cuff of normal tissue. 4. Radical- entire compartment removed
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Enneking staging system- malignant
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I- low grade/ II- high grade/ A- intracompartmental/ B- extracompartmental/ III- metastatic
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AJCC staging
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I- low grade/ II- high grade/ A- <8cm/ B- >8cm/ III- skip mets or nodal mets/ IV- mets
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Enneking staging system- benign
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1. Inactive/latent (NOF/Enchondroma). 2. Active. 3. Aggressive (GCT/ABC)
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XRT induced stress fx
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Occur late. At risk if periosteal stripping + XRT. Consider prophylactic intramedullary fixation
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(11:22)
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Ewings
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(12:16)
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myxoid liposarcoma
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(X:18)
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synovial sarcoma
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(2:13)
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rhabdomyosarcoma
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Tumors in <5yo
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Leukemia/ neuroblastoma/rhabdomyosarcoma/ osteomyelitis/ OFD
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Poor prognostic factors soft tissue sarcoma
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mets/ high grade/ >5cm/ below deep fascia
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Preop vs postop irradiation
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postop: higher dose/ higher field/more fibrosis/equal control rates/ lower wound complication rate
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dermatofibrosarcoma protuberans
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nodular cutaneous tumor. Low grade metastatic. Wide resection
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characteristic cell of liposarcoma
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lipoblast- signet ring cell
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Neurilemmoma
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benign nerve sheath tumor. Waxes and wanes in size. Eccentric mass. Can separate from nerve.
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Antoni A
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(neurilemmoma)compact spindle cells/ intranuclear vacuoles/palisading nuclei/ whorling cells. Verocay bodies (2 nuclei together)
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Antoni B
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(neurilemmoma)disorganized/ hypocellular/ vascular
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Neurofibroma
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superficial/ painless. Fusiform nerve enlargement. Elongated cells w/ dark nuclei . Tx- observe most. Otherwise marginal resection +/- nerve grafting.
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Neurofibromatosis
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Multiple neurofibromas (plexiform characteristic)/ café au lait spots/ NOF/Scoliosis/ bowing. Malignant degeneration in 5-30%.
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Rhabdomyosarcoma
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most common sarcoma in <20yo. Histo: spindle cells in parallel bundles/ racquet shaped cells.Cross-striations- rhabdomyoblasts. Tx: CHEMO/ SURG/ XRT
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Angiosarcoma
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tumor of endothelium of blood vessels. Highly malignant/ infiltrative. Tx: amputation often required/ high rate of local recurrence and pulmonary mets
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PVNS
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Reactive condition. Proliferation of synovial villi and nodules. Knee>hip>shoulder. Recurrent hemarthrosis- hallmark. Cystic erosions on both sides of joint. Tx: complete synovectomy (30-50%). XRT reduced recurrence to 10-20%
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Synovial Sarcoma
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High grade malignant tumor. Most common sarcoma in foot. Biphasic histo- epithelial and spindle cell components. Monophasic fibrous common. IH: + keratin/ + epithelial membrane antigen. Tx: wide resection/ adjuvant XRT. Mets common.
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Epithelioid sarcoma
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Most common sarcoma of hand. May ulcerate and mimic granuloma. Mets to nodes. Histo: very eosinophilic. Minimal pleomorphism. Tx; wide excision
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Clear cell sarcoma
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Associated with tendons/ aponeuroses. Usually on foot/ankle. Histo: compact nests of fascicles/fusiform cells w/ clear cytoplasm. Tx; Wide resection and XRT
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Hematoma
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May occur within sarcoma- follow at 6wk intervals until resolves. MRI can't tell between hematoma vs sarcoma w/ hemorrhage
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Osteoid Osteoma
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Self-limiting/ benign. Ages 5-30. Pain relieved by NSAIDS. Joint pain if intracapsular. Painful scoliosis (on concavity- excision may resolve scoli). Thin cut CT best. Histo: osteoid. Tx: NSAIDS- 50% will resolve. RFA-->90% success (cant use near spine). Curettage.
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Osteoblastoma
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Similar to osteoid osteoma- not self limiting- gets very large. Likes posterior spine. Histo: loosely arranged tissue/ very vascular. Tx: curettage/marginal excision
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Osteosarcoma- poor prognostic factors
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P-glycoprotein/ high ALK Phos/ high LDH/ vascular invasion/ no change after chemo/ no anti-shock protein 90 antibodies after chemo
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Parosteal osteosarcoma
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low grade malignant. Classically posterior distal femur. Tx: wide margin.- no chemo. 1/6- dedifferentiated. Invasion of medullary canal doesn't change prognosis
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Periosteal osteosarcoma
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intermediate grade malignant. Diaphysis of long bones. RAD: suburst appearance on cortical depression. Histo: chondroblastic. Tx: like classic osteosarc
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Telangiectatic osteosarcoma
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bag of blood with few cells. RAD: lytic/expansile. Look like ABC
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Ollier's disease
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Occur late. At risk if periosteal stripping + XRT. Consider prophylactic intramedullary fixation
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Maffucci syndrome
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Multiple enchondromatosis w/ hemangiomas- 100% risk of malignancy (astrocytomas/visceral/ GI)
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Malignant trans of osteochondroma
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<1% (10% in multiple exostoses). Usually low grade- tx with excision alone- rare mets.
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EXT mutations
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EXT1/EXT2- multiple exostoses. EXT2--> more exostoses and high risk of malignancy
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Chondroblastoma
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Benign. Epiphyseal. Pt w/ open physis. XR: central lytic lesino- sharply demarcated w/ sclerosis. Tx: intralesional curettage. 2% mets to lungs
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Chondromyxoid fibroma
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Tibia most common. Lytic eccentric lesion. Sharply demarcated. Lobulated. Tx: curettage/grafting
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NOF
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Asymptomatic incidental finding. Metaphyseal/eccentric lucent lesion w/ sclerotic rim. May have thinned/expanded cortex. Histo: cellular/fibroblastic tissue. Cells with whorled bundle. Tx; observe. If 50-75% of cortex involved curettage/bone graft
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Chordoma
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Sacrum/spheno-occipital region. 10-15% anterior mobile spine. May present w/ GI symptoms. High rate of recurrence. Metastasize late. Histo: physaliferous cells in strands w/ mucous background. Tx: wide excision +/- XRT.
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Vertebral hemangioma
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Pain/pathologic fractures. XR: lytic destruction/ vertical striations/honeycomb appearance.
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Lymphoma of bone
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Can be isolated or associated with systemic/ metastatic disease. Affects any age. XR: long lesion. Mottled appearance w/ bone destruction. Tx: CHEMO/RADS. OR only for stabilization.
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Multiple myeloma
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50-80yo. Sx: fatigue/Anemia/ARI/hypercalc/amyloidosis. XR: punched out lytic lesion. Dx: skeletal survey. MRI best to detect vertebral involvement. Histo: plasma cells/perinuclear clear zone=golgi. Monoconal immunostaining. Clock face nucleus. RAD:palliation of pain/ tx neuro symptoms.
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POEMS syndrome
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polyneuropathy/organomegaly/endocrinopathy/M-proteins/skin changes.
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Giant cell tumor
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benign/aggressive. Epiphyseal-metaphyseal. 10% in spine. Sacrum is most common axial location. XR: lytic destructive lesion, abuts subchondral bone. Tx: extensive exteriorization w/ adjuncts. Recon w/ bone graft or cement- control rate 85-90%. May have coexisting malignant sarcoma. usually after irradiating benign GCT.
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Ewings sarcoma
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small round blue cells. Ages 5-30. Tumor grows quickly--> necrosis--> elevated ESR/CRP/WBC. Anemia RAD: lytic lesion with variable reactive bone. Tx: chemo/RADS/resection. Wide resection--> less recurrence and avoidance of XRT. Poor prognostic indicators: spine/pelvis/<90% necrosis after chemo/high LDH/p53 mutation/gene products other than EWS-FL1
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Adamantinoma
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Epithelium-like islands of cells. Tibia. XR: multiple sharply demarcated lucent defects. Cortical bone destruction. Low grade malignant lesion. Tx: wide resection
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ABC
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Reactive aggressive condition. XR: eccentric, lytic expansile metaphyseal lesion. Fluid-fluid levels on T2 MRI. Histo: cavernous blood-filled spaces w/out endothelial lining. Tx: curettage and bone grafting. Recurrence common if open physes.
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UBC
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XR: symmetric cystic expansion with thinning of cortices. Bone expanded no wider than physis. Proximal humerus most common. Pathologic fx common. If abuts physis- active. Tx: aspiration/methylprednisolone. Curettage and bone grafting if recalcitrant. If proximal femur- add internal fixation to prevent fx and AVN.
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Hand-Schuller Christian disease
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multiple eosinophilic granulomas w/ visceral involvement. Classic triad- exopthalmos/DI/lytic skull lesions
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Letterer-Siwe disease
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fulminating form of histiocytosis--> death in infancy
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EG
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highly destructive well defined lesion. +/- soft tissue mass. May expand bone. Histo: Langerhans cell: indented or grooved nucleus. Bilobed eosinophils common. Self limiting. Tx: XRT/curettage w grafting- if fracutre or joint compromise imminent.
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Fibrous dysplasia
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Failure of bone production. Activating mutation of GS alpha protein-->inc cAMP. XR: long lesion in long bone. Ground glass appearance. Histo: disorganized bone fragments- chinese letters. Tx: observe most. Tx curettage w allograft, internal fixation if high stress area. Bisphosphonates help decrease pain if polyostotic
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McCune Albright syndrome
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fibrous dysplasia/ precocious puberty/café au lait spots/
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Osteofibrous dysplasia
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anterior tibial cortex. Bowing/ pathologic fractures common. Radiographs diagnostic. Histo: fibrous stroma w/ bone trabeculae WITH osteoblastic rimming. Tx: observe. Most resolve by maturity.
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Paget disease
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Abnormal bone remodeling. XR: coarsened trabeculae/remodeled cortices. Histo: irregular/broad trabeculae. Pain. Tx: disphosphonates/calcitonin to decrease pain. If arthroplasty planned- pretreat bisphosphonates to decrease bleeding. Etidronate may-->osteomalacia. Pamidronate preferred.
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Paget sarcoma
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Focus of malignant sarcoma within paget's bone. Sx: abrupt onset of pain and swelling. RAD: cortical disruption and soft tissue mass. Very poor prognosis
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Breast CA osteolysis
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tumor cells release PTHrP-->osteoblasts release RANKL-->binds RANK on osteoclast precursors-->differentiate into osteocalsts-->resorption--> release Ca/TGF-beta/IGF-1-->tumor cells multiply
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Risk of fx w/ bone mets
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1. over 50% cortex involved. 2. permeative destruction of subtroch. 3. over 50-75% metaphysis involved. 4. pain after XRT. 5. weight bearing pain
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Dx tests for met of unknown primary
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1. XR affected limb. 2. Technetium bone scan. 3. Chest CT and XR. 4. CT abdomen. 5. Skeletal survey. 6. CBC/ESR/Chem/SPEP/UPEP. Dx primary 80-85%
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