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84 Cards in this Set

  • Front
  • Back

Diaphyseal lesion

HALFEE


- Histiocytoma


- Adamantinoma


- leukemia


- FD


- EG


- Ewings

Epiphyseal lesion in adults

- chondroblastoma

- GCT

- clear cell chondrosarcoma

- infection

- telangiectatic osteosarcoma

UBC V ABC on XR

ABC


- eccentric (v central)


- width > physis

Osteochondroma Transformation risk

hereditary multiple osteochondromatosis is unknown, but may be 25-30% compared to approximately 1% for a solitary osteochondromas.


The risk of malignant degeneration increases as the number and size of the osteochondromas increases.

Osteochondroma Transformation risk

1. Growth after maturity


2. Hot bone scan (can be from overlying bursa)


3. Increased thickness of cartilage cap on CT- > 1-3 cm (Enneking)


4. Pain


5. Pathological fracture


6. Disappearance of calcification


- More common with central lesions


- In general, a sessile lesion is more likely to degenerate into sarcoma than an exostosis.

Osteosarcoma - Rosen in vivo response

Dictates outcome


some OS have P-Glycoproteins pump- remove chemo from the cell


Grade 1 = no cell death, Grade 2 = Partial <90%, Grade 3 = Necrosis >90%, Grade 4 = Complete necrosis


Grade 1 & 2 - < 50% survival


Grade 3 & 4 - > 75% long term survival in OS and MFH

Bloods test for Sarcoma / Mets / Unknown lesion

FBC

LFT (Alk phos)


Ca, PO4, Alk Phos


ESR / CRP


TFT


PSA


Serum and urine electrophoresis


BJ urine


LDH - lymphoma


blood film (leukaemia)


UE (MM and pre chemo)


Urate (gout common with malignancy)

ABC

reactive heamorrhagic tissue arising in bone.


Blood filled spaces seperated by fibrous tissue.


Metaphysis long bone. (Prox hum, femur, tib)


Posterior elements in vert.


Teenagers.


NHx # is rare, Usually resolve with skeletal maturity.


XR eccentric, expansile, cortical thinning.


Fluid fluid of MRI.


Histo: blood, haemosiderin laden macro. GC.


Rx observe, Op mx (Curretage BG, plate) prox femur, spine, recurrent #. Highest reccurent rate in those with open physis.


If spine / pelvis considered embolisation.

Adamantinoma

Low grade Malignant


20yo. 90% in diaphysis tibia.


Soap bubble appearance, eccentric, expansile, little periosteal reaction.


Closely resembles FD and is varient of Ossifying fibroma.


NHx metastasis 20%.


WLE.

Benign Fibrous Histiocytoma

Older patient.


Lytic lesion with sclerotic rim.




Benign foam cells, lipid filled.




Curettage and BG.

Chondroblastoma

Adolescents


NHx benign, locally aggressive. Can have pul mets. Need CT chest.


Epiphysis knee / hip.




Painful, can cause LLD or angular deformity.


Eccentric, well defined lucency, thin rim.


Stippled calcification. may have periosteal reaction.


Difficult to Rx due to position of joint.


Curettage and BG/PMMA. Radiofreq ablation. WLE with joint replacement.

Chondromyxoid fibroma

Benign neoplasm cartilage origin. Myxoid cartilage.


Very rare 20yo.


Upper tibia, metaphysis. usually LL.


Tender and buldge.


Eccentric meta, sclerotic margin, local expansion but cortex intact. characteristic buttress of periosteal new bone.


Cant tell difference from ABC.


Curettage, BG 40% recurrence.

Eosinophilic Granuloma

AKA langerhans cell histiocytosis.


Non neoplastic. infiltration by histocytic cells (monocytes / macro lineage). cell of origin is lanerhans. Unsure origin.


4-7yo. Variable presentation.


Back pain from spine lesion.


Affects any bone.


Diaphyseal - thin endo scalloping.


Vertebra plana, skull.


Rule out HSC BS.


NHx self limiting, tissue is replaced by bone. HSC chemotherapy.

Eosinophilic Granuloma sub groups

E-H-L



  • EG 70% 5-20yo only osseous.
  • Hand-Schuller-Christian (20%) Multiple EG, widespread with diabetes insipidus, exopthalmos, skull defect, large spleen, liver, skin lesions, pulm disease. 10% die.
  • Letterer-Siwe disease (10%) < 1yo. Fulminant course all die.

Fibrous cortical defect

AKA NOF.


Hamartomatous defect in metaphyseal cortex, skeletal immature adolescents.


<2cm FCD >2cm NOF


Defect in cortex long bone. 35% kids. Most common cause of pathological #.


NHx self limiting usually ossify by adult.


Femur / tibia.


Eccentric, metaphysis, slight expansion sclerotic margin.


Fibroblast whorling, multinucleated GC.


Serial observation.


Op if > 1/2 bone, Curettage and BG.


If fracture - heals in normal time.

Fibrous dysplasia

Developmental Hamartoma areas of bone fail to mature normally, will remain indefinitely, poorly mineralised bone.


Can be associated with endocrinopathy.


Femur, tibia, skull.


NHx remains relative unchanged through out life. Malignant transformation monostotic 0.4%, polyostotic 4% (OS/CS/FS).


Polystotic - usually presents as kid, pain (#), limp and deformity.


XR ground glass appearance, angular deformity.


Coxa vara, Shepherds crook deformity, Sabre tibia.


BS looking for other sites.


Pathology Chinese letter.


Dont operate unless deformity. Morcellised BG is resorbed. Bisphos relieves pain.


Prox femur. Stabilise prevent worsening. When mature valgising osteotomy.


Activation mutation GS alpha, chromo 20. leads to a sustained activation of adenylate cyclase cAMP.

McCune Albrights syndrome

Triad


Fibrous dysplasia


Cafe-au-lait spots (Coast of maine)


Precocious puberty.

GCT

Benign wide spectum behaviour (can behave in malignant fashion 2% metastasizes to lung).


20yos, after maturity.


Epiphysis - Knee, DR, sacrum, vert bodies.


Dull ache, effusions.


Well defined lesion, extending into subchondral bone. No sclerotic rim. can destroy cortex and extend into ST.


Mutlinucleated GC, background fibrous tissue.


Curettage and PMMA (recurrence 15%) or WLE and allograft/arthroplasty. medical Bisphos disomaz.



Haemangioma

Hamartomatous proliferation of vascular tissue.


Capillary or Cavernous.


Can be in any tissue. Ache, limb heaviness, tender mass.


XR may have small calcified phleboliths.


MRI bright.


Management ST embolization, resection.


Intraosseous.


commonest sites spine and skull.


Spine honeycomb appearance.

Haemangioma Associations


  • Klippel Trenaunay Weber Syndrome - Hemihypertrophy secondary to angiogensis. usually unilateral.
  • Maffucci syndrome
  • Sturge - weber - Congenital, often facial malformations. Hemiatrophy, mental retardation.

Metaphyseal enchondroma

metaphsis of cortex long bones.


Prox humerus.


Difficult to DDx from chondrosarcoma.


NHx doesnt ossify, remains as immature cartilage. No malignant potential.

Unicameral bone cyst

Solitary cavity contain clear fluid.


Metaphysis in kids, adjacent to physis in kids.


? due to secondary venous obstruction / hypertension.


Prox metaphysis Humerus 67%, femur 15%. Can occur in calcanous.


Present with pathological #.


XR near physis, well defined, central, thinned cortex, thin internal septa.


Fallen leaf sign.


NHx become latent resolve in adulthood, may resolve post #. 15% heal after #.


Will heal but on average # 4 times.


Observe, inject HLCA, curettage BG, cyst drain, ORIF.


Humeral # Rx in sling.


Prox femur Traction, paediatric hip screw and bone graft < 15yo. IMN>15yo.

Bone infarct

Divers, Sickle cell, long term steroid




MFH may arise from long term bone infarcts.

Enchondroma

Benign intramedullary cartilage.


Remnants of cartilage from epiphyseal growth mechanism which failed to under go enchondral ossification.



Young adults. Stop growing in adults.


Malignant transformation. Never in children, never in hands and feet. BS and histology not helpful.




Start near physis become diaphyseal with growth. Can have periosteal chondroma.


Stippled calcificaition, no periosteal reaction, no scalloping.




> 6cm rarely benign.


Difficult to distinguish low grade chondrosar and enchondroma.


Histo hyaline cart.


Management observe. Clinical, and XR.


Hand # - let heal then Curettage and BG.

Olliers

Enchondromas symmetrically distributed through an extremity.


Usually unilateral and monostotic.


Not heritable.


Often associated with growth deformity


LLD, Bowing wrist and elbow.


Malignant transformation 25% @ 40yos.

Maffuccis syndrome

Multiple enchondroma, Random distributed.


Associated with hemangiomas.


100% risk malignant transformation. long latent phase 30yrs.


Associated with other tumours / visceral carcinoma.


Skeletal deformity. Short tubular bones, hands and feet. LLD, Bowing forarms, Varus/valgus legs.


Mx osteotomy to correct angulation, guided growth. Lifetime surveillance.

Enostosis

Bone island.


Hamartomatous lesion.


Mature cortical bone in midst of normal cancellous bone.




Sharp, cortical density, round/oval, smooth, narrow zone. usually < 1cm.




Osteopoikilosis multiple, AD, periarticular. Extremelt rare malignant transformation.

Melorheostosis

1:1000000, from child hood.


50% dvp symptoms by 20.


Abnormality in sensory nerve of sclerotome.


Pain, stiffness, progressive deformity. LLD, contractures, Spinal stenosis.


XR Sclerotic lesion cortical hyperostosis (dripping wax).




NHx slowly progressive, symptoms may require treatment.

Osteochondroma

Outgrowth of benign cartilage from bone.


? defect in perichondral ring la croix.


NHx Stops growing when physis close.


Malignant transformation. Low grade CS < 1%.


Suspicious features. Grow after maturity, hot BS, C Cap > 2cm, pain, pathological #, disappearance of calcification.


Knee, prox humerus.


Pain, tenderness, decreased ROM, Compression Nerve.


XR adjacent physis, away from jt, coritcal and medullary continuous with normal bone, may have calcified cap.


MRI C cap and where NV bundle lies.


DDx juxtacortical OS/CS, periosteal chondroma, osteoma.


Management. Excise if, painful compressing, restore ROM, suspicious lesion, central (high risk).

Multiple hereditary exostosis

AD variable penetrance.


Mildy short, nerve compressions (CPN).


XR Coxa Valga, brevis. Genu valgum, ankle valgus (Fib short, wedge shape tibial epip).


Forearm (Ulna short R dowing, ulna dev wrist, RH dislocation)


Excision symptomatic or suspicious lesions.


Guided growth or deformity correction if severe.




Malignant transformation. 10% overall


CS>OS. Better prognosis than primary CS.


Danger areas more central.

Nora's

Bizarre parosteal osteochondromatous proliferation.




Hand/feet 20-30's.


Grows rapidly aggressive on imaging.


Mildly painful, incr size over mths.


XR on surface bone, well defined, wide base.




Not contigous with medulla or cortex no cartilage.




NHx lesion is benign.




WLE limits local recurrence (50%).

Osteoblastoma

young boys.


30% spine posterior elements. (scoliosis)


Long bones 25%. (limp)


Pain less severe than OO, not relieved by aspirin.


XR spine- sclerotic or loss pedicle, enlagement.


L bone- meta-diaphyseal, faintly radiolucent, thin reactive rim, maybe expansile.


CT stippled ossification.


BS hot


OO < 2cm.


NHx can be locally aggressive (distruction, ST extension). Not self limiting therefore need surgery.


Mx extended curettage, WLE, radiofreq.

Osteoid osteoma

Benign, bone forming, small nidus surround by wide zone mature bone.


5-25yrs old. Though to be glomus tumour of bone.


Pain secondary to prostaglandin.


intracoritcal position. femur, tibia at end diaphysis, posterior spine elements.


Pain, intense, unrelenting (tooth ache) night. relieved by aspirin. worse by alcohol.


NHx resolution with time.


XR sharp round, < 1cm, homogenously dense centre. Cortical fusiform, central nidus, mimics stress #.


DDx infection, stress #, Parosteal OS, chondroblastoma.


Non op -NSAIDs


Op En bloc resection, Burr down tech, Radio freq ablation 6mins 90 deg.

Osteoma

Benign harmartomatous bone forming lesion arising from surface of bone.




Dense bone (like cortical) which arises from the surface of bones formed by intra membranous ossification.




Skull mandible. Shafts of long bone (TIbia)

Chondrosarcoma

Malignant cartilage tumour.


Primary (de novo), secondary (osteochondroma/ enchondroma).


Diagnostic dilemma diff low grade from enchondroma (sapmling errors)


Varients



  • Dedifferentiated - very aggressive, most die <2yrs.
  • Clear cell - epip young males (varient chondroblastoma), low grade, slow mets rare. WLE.
  • Mesenchymal - rare , young, extra skeletal, high metastatic potential. Surgery and Chemo.

XR endosteal scalloping hallmark CS. May have cortical thickening and expansion.


Pain in benign cartilage tumour must assume malignant.


Grade 1 - 3.


Mx WLE. Highly resistant to chemo and radio.


Exceptions in Dediff (chemo), mesen (C & R).


Low grade 90% 5y, high grade <10% 5yr.


Mets grade 2 15-40%, 3 75%.

Osteosarcoma

Malignant spindle cell sarc of bone. Malignant cells produce osteoid. Most aggressive and high grade.


Bimodel, teenagers (75%) elderly (paget/ radiotherapy).


If under 13yo prob ewings on OS.


Classic central (high grade), parosteal, periosteal


Metaphysis Distal femur, prox tibia, pro hum.


Pain, often activity related, night.


10% mets on presentation.


XR meta, cortical destruction, osteoid, wide zone, codman, ST involvement.


MRI local extent and planing and entire bone (skip lesion). BS other lesions. CT chest (mets)


ALP/LDH worse prognosis if incr.


70% survial overall, 90% limb sparing surgery.


12% local recurrence. (use Radn if + margin)


If you survive 2 yr you are likely going to make it.



Secondary causes OS

Paget's


previous radiotherapy


OM


Fibrous Dysplasia


Chondrosarcomatous dedifferentiation

Lichenstein pathological classification OS

Osteoblastic -50% prominent osteoid


Chrondoblastic - prominent cartilage


Fibroblastic - Prominent fibrous tissue look like fibro sarc.


Telangiectatic - worst prog, pools RBC/ giant cells. Can be mistaken for GCT.


Giant cell rich OS - older pts, simialr MFH


Small cell OS - Similar appearance to Ewings, respond to chemo like PNET.

OS algorithm

1. Accurate clinical staging, local (cross sectional imaging - CT / MRI), systemic (bone scan & CT chest / abdomen, biopsy


2. Neoadjuvant chemotherapy


3. Restage- locally and systemic (MRI / CT Chest)


4. Wide resection


5. Post operative chemotherapy +/- radiotherapy if positive margins

OS chemo

Treats micro mets, allow limb salvage.


Rosen in vivo response dictates outcome.


Some OS have P-Glycoproteins pump to remove chemo from cell.


Partial < 90% kill, Necrosis > 90% kill, complete necrosis. Predict survival.




2 cycles pre op MACI (MTX, Adriamcin, Cisplatin, Ifosphamide).


surgery 2 wks after finished (3mth from start).


Goal recestion with 5 cm margin.

Parosteal OS

20-40yo females.


Low grade. Slow growth.


Most common in posteromedial distal femur.


Painless block to back of knee.


May look like osteochondroma.


Broad base from cortex, less dense than bone. String sign thin radiolucent line bw lesion cortex


CT scan. Attached to cortex growing into ST.


WLE 80% cure with surgery alone.

Periosteal OS

V rare, 15-25.


Diaphysis of long bone. typically anterior prox tibia.


NHx Higher grade (Peri is a very bad boy).


Classically shows cartilage.


XR punched out lesion, in saucer shaped defect in cortex.


Mx WLE, with neoadjuvant and adjuvant chemo.

Ewings

70% long term survival.


Small round cells of uncertain histogenesis.


? neuroectoderm. translocation 11,22.


usually second decade 5-30yo.


Pain then swelling. Fever, LOW, malaise.


XR Diffuse permeative destruction, ext in ST. Codmans, onion skinning, sunburst.


Elevated ESR LDH poor prognosis.


CT chest, MRI, Bone scan (10% have multiple bone at presentation)


Treatment Algorithm similar to OS.


Chemo VAAC (Vincristine, actinomycin, adriamycin, Cisplatin) loooking for >95% kill.


Survival Mets at diagnosis 40% 5yr, no mets 60%. 5% local recurrence with surgery.

Myloma

Proliferation of single clone of plasma cells.


50-60yo. Heavy chain (IgG most common, light chains kappa and lamda = Bence Jones proteins)


Highly differentiated B cells. Usually bone marrow of entire skeleton involved.


Bone pain, fatigue, fever, night sweats.


Anaemia, ESR > 100, Bence jones in urine. Serum and urine electrophoresis.


XR punched out lytic lesion. widely disseminated = soap bubble appearance. No sclerotic rim. Diffuse osteopenia, Vertebrae plana.


Bone scan 25% neg. Skeletal survey. Bone marrow biopsy. sheets plasma cell no stroma.


Plasmocytoma - 70% go onto MM. 30% cured by en bloc resection and DXRT.


MM - Chemo and Radiotherapy. Surgical stabilisation of pathologic #.


MM is very aggressive with early death.

Metastatic disease

Avoid pathologic #, improve survival, easier to fix


80% malignancies to bone PBL (prostate, breast lung). Kidney, thyroid, GIT 20%.


Lytic- lung ,kid, breast, thyroid, GI, neuroblast


Mixed - breast, prostate, lung, bladder


Blastic - Prostate, breast, bladder, GI.




Bone scan is good screen.


Dont forget to XR whole bone.




Mortality post #


lung 100% at 6 mths


breast 50% at 6 mths.


Mean survival metastatic disease. 50% 6mth. 30% 1yr.

Metastatic prophylaxis

Principles


Need to live longer than recovery.


Reconstruct so can FWB


Address all areas of bone


DXRT post op.




Other:


Healing is slower than normal bone.


DXRT > 14days post op doesnt cause non union.


if large lesion >75% augment with PMMA.


renal cell = embolise.


Chemo can incr infection and blood loss.


Healing of pathological fracture without fixation very poor 70% non union.

Metastatic Paeds

Leukemia


Neuroblastoma (Tumour of sympathetic nervous system)


Wilms


Bone scan

Enneking questions

Where is it?


What is it doing to the bone? expansion, zone


What is bone doing to it? periosteal, reactive rim.


Any other clues? ST, matrix, bone forming.

Stage local and Systemically

To accurately define the extent of disease, prior to proceeding with biopsy and definitive treatment.

Biopsy

To determine Benign or Malignant


To determine specific cell type


To determine grade


Provide representative sample without compromising definitive Rx.


To be done at treating hosp (5x compication)


Have pathologist on stand by.


Traverse single muscle/compartment, which will be removed for WLE.


Stay away from NV bundle.


Direct approach without going through muscle if possible. Do not go thru NV interval.

Staging Malignant

1A Low Grade Intra-compartmental


1B Low Grade Extra-compartmental


2A High Grade Intra-compartmental


2B High Grade Extra-compartmental


3 Metastasis

Compartment

anatomically confining space will resist tumour spread beyond its boundaries.




Intra compartment. Intra-osseous, intra fascial compartments, superficial to deep fascia, par-osseous




Extra compartment, pelvis, popliteal fossa, axilla, cubital fossa

Staging Benign

Stage 1 Benign inactive (NOF)


Stage 2 Benign active (ABC)


Stage 3 Benign aggressive (GCT)

Margins

Intralesional - within lesion, tumour remains


Marginal - within reactive zone


Wide - intra compartment and outside reactive zone (Cuff of normal tissue)


Radical - Extra compartment. removal of all compartment.




Skin < 5cm Wide, >5cm radical.

Limb Salvage

must have same survival rates


must not delay adjuvant treatment


reconstruction should be enduring with minimal complications


function should approach that achieved by amputation




CI: PIN LEG patho #, infection, NV bundle, LLD >8cm, Extensive msucle loss, Good biopsy.

Liposarcoma

Rarely arise from Lipoma


S-100 +


almost always subfascial.


Painless enlarging mass.


1/2 met to lungs, 1/2 met to non lung sites.


Difficult to diff liposarcoma V atypical lipoma.


Get sampling errors.


Well diif, myxoid, round cell, pleomorphic, de diff


WLE. DXRT

Malignant fibrous Histiocytoma

Pleomorphic spindle cells, storiform pattern.


primary malignant bone tumor similar to OS, but has no osteoid.


25% secondary to either bone infarct, pagets, radiation (worse prognosis).


Metaphysis, lytic destruciton no bone production.


NHx Agressive usually high grade.


Fast growing lump, minimal symptoms.


Chemo, WLE, Chemo.


50% recurrence, 50% survival 5 yrs.

Clear cell Sarcoma

Small, arise in conjuction with tendons and aponeuroses.


F&A 50% 20-40yrs.


Translocation 12:22


Young male lump in foot Clear cell sarcoma or synovial sarcoma.


Met lymph and blood.

Others

Leimyosarcoma - from smooth muscle


Fibrosarcoma - Malignant spindle cell. Difficult to diff low grade from fibromatosis.


Epithelioid Sarcoma - 50% forarm/wrist often misdiagnosed as ganglion (if not in typical place for gan then be suspicious).

Neurofibrosarcoma

Malignant schwannoma.


Arise from peripheral nerves.


50% have NF.


10% life risk if have NF.


Painful enlarging mass. Often presents with neurological symptoms.


MRI cannot tell diff between Neurofibroma and one that has transformed to sarcoma.


NHx most high grade 5yr 50% survival.


Mets to lung and bone.


Solitary neruosarcomas have better porg than those associated with NF1.

Rhabdomyosarcoma

Malignant tumour of striated muscle.


Most common malignant ST tumour in children < 15yo.


Embryonal, Alveolar, Botryoid, Pleomorphic.


Mx


Neoadjuvant chemo (**unusal for STS) WLE DXRT.

Synovial sarcoma


From synovial cells but not found in joints.


20-30yo.


Can met to lymph.


Classical mineralisation seen on XR.


Most common sarcoma of foot.


All considered high grade.


Translocation X:18


Pre op DXRT the WLE.


70% 5yr.

Origins of sarcoma

Adipose / LS


Fibrous / FS


Fibrohistiocytic / MFH


Muscle / Leiomyosarcoma / Rhabdosarcoma


Synovial / Synovial Sarcoma


Nerve / neurofibrosarcoma


Bone / Cartilage - Extraskeletal OS & CS


Vascular / haemangioendothelioma / angiosarc


Other.

Benign soft tissue lesions

vascular (hemangioma, AVM, lymphangioma, glomus)


neurogenic (NF, schwannoma)


muscular (leiomyoma)


fibroblastic (desmoid tumour, fibromatosis)


fat (lipoma)


simple cyst


PVNS


MO

Lipoma Arborescens

Proliferative synovial condition centre is a lipoma


Knee.


MRI = synovial proliferation


Arthroscopic debridement



PVNS

Pigmented Villo-Nodular Synovitis


Benign inflam process arising from synovial tissue, contains significant amounts hemosiderin


Diffuse or localised.


Major joints, knee, hip, shoulder.


NHx process is progressive, cause destruction cartilage and SC bone resulting in degenrative Arthritis. can cause cystic erosions.


MRI characteristic T1, T2 (dark) except if had resent bleed into it)


Rx synovectomy open / arthroscopic.

Synovial chondromatosis

Chondroid metaplasia of synovium.


Predilection for large joints.


Knee, hip, elbow, shoulder.


Mesenchymal cells in joint capsule become chondroblasts instead of fibroblasts form nest of cartilage. Grow and break off float into jt.


Arthroscopy early to prevent secondary degeneration.

0- 5 yr

Malignant


Leukemia, metastatic neuroblastoma, metastatic rhabdomyosarcoma




Benign


OM, osteofibrous dysplasia

10-25 yr

Malignant


OS, Ewings, leukemia




Benign


EG, OM, Enchondroma, FD

40-80 yr

Malignant


Metastatic disease, myeloma, lymphoma, CS,MFH, pagets sarcoma, radn sarcoma




Benign


Hyperparathyroidism, pagets, enchondroma, bone infart.

Spinal tumors

Anterior


GCT, Hemangioma, EG, Mets, chordoma, MM




Posterior


ABC, OO, OB.

Tumours of Neural tissue

  • Neurilemoma (benign schwannoma) nerve sheath tumor, no sx except mass, tumour grows. MRI eccentric mass from peripheral nerve. Composed of Antoni A and B.
  • Neurofibroma - single or multiple. superifical slow growing painless. 1/2 associated with NF. in major nerve can cause expansion in fusiform manner (tumour in nerve). histo = wavey. marginal excision often causes further loss of function. MRI target sign.
  • Neurofibromatosis - AD, skeletal abnormalities. Malginant change occurs in 10%. Pain and enlarging = transformation.
  • Neurofibrosarcoma - either de novo or in NF. High gradesarcoma.

Tumour questions on Hx (for lump)

Pain



  • Deep like tooth ache
  • intermittent progressing to all time?
  • Increasing in intensity
  • Night / unrelenting?
  • NSAIDs opioids?
  • 1-3mths high grade/6-24mths low grade.

Systemic features



  • Fever
  • LOW
  • Malaise

Zone of transition

Geographic


Permative


Moth eaten




Periosteal reaction


Onion skinning


Start burst


Codman

Tumor suppressor gene Rb

Retinoblatoma, Osteosarcoma.


P16INK4a - familial melanoma, Chondrosarcoma, osteosarcoma melanoma.




EXT1/2 MHE - Osteochondromas, CS


NF1 - sarcoma.

Chromosomal alterations (cytogentics)

Ewings, PNET - 11;22 EWS, FLI1


Synovial sarcoma X:18 SYT, SSX


Clear cell 12;22 EWS,ATF1



Histology words

Pleomorphic


Polychromatic


Mitotic figures.


Osteoid pink. look for thin seams of it = malignant osteoid.


Cartilage is purple/ blue. cells wide apart.

Parosteal osteoma

30/40yo.


uniform radio dense with broad base, no cortical or medullary invasion.


Histo has mature lamellar bone with intact haversian system.

Periosteal chondroma

Hyaline cartilage on surface of bone.


Slow growing no potential of malignancy


classic well defined surface lesion create saucerised defect in underlying cortex.


edges of lesion have mature buttress (rim) of bone.


calcification is variable.

Subungual exotosis

arise from beneath nail in distal phalanx.


Thought to be post traumatic lesions.

CMF

Chondromyxoid fibroma.


benign contain chondroid, fibrous and myxoid tissue.


10-20yo can be older.


Thought to arise from remnant of growth plate.


Proximal tibia.


Meta, eccentric lucent, thinning and expansion of surrounding bone. Sharpe scalloped rim. Shark bite.


Look like cartilage on Histo.


Curettage BG. Local recurrence rate 10-20%.

Hand and feet soft tissue sarcomas

Hand - Epithelioid sarcoma


Feet - Synovial sarcoma

Biomechanics of lesions

  1. When length of longitudinal defect in bone exceeds 75% of its diameter there is a 90% reduction in torsion strength.
  2. centred cortical defect 50% = 60% bending strength reduction.
  3. eccentric cortical defect 50% = 90% bending strength reduction.

Sensativitiy to radation

Very - myeloma, lymphoma


Moderate - breast, lung, bowel, prostateResistant - thyroid, kidney, melanoma