Chrondrogenic tumours Flashcards Preview

Pathology FRCS > Chrondrogenic tumours > Flashcards

Flashcards in Chrondrogenic tumours Deck (50):
1

what is an enchondroma?

  • A Benign chrondrogenic tumour- 2nd most common
  • composed of Hyaline cartilage
  • caused by abnormality in chondrocyte function in the physis
  • M=F
  • most common 20-50 yrs
  • usually in medullary canal in diaphysis and metaphysis
  • most common in hand 60%
  • location
    • ​distal femur, proximal humerus,tibia
    • rare in spine/pelvis

A image thumb
2

What is the pathophysioogy of enchondroma?

  • enchondromas represent incomplete endochondral ossification
  • chondroblasts and fragments of epiphyseal cartilage escape from the physis, displace into the metaphysis and proliferate there



     

A image thumb
3

Name associated conditions of enchondromas?

  • Solitary enchondromas
  • Ollier's disease
    • multiple enchondromatis
    • no genetic predisposition

    • skeletal dysplasia with failure of normal endochondral ossification 


    •  

      enchondromas throughout the metaphyses and diaphyses of long bones 


    • involved bones are dysplastic, with shortening and bowing 


    • risk of malignant transformation <30%

 

  • ​Maffucci syndrome
    • ​sporadic inheritance
    • multiple enchondromas & soft tissue angiomas
    • risk of malignancy 100%
    • increased risk of visceral malignancy

 

 

 

 

A image thumb
4

Can you describe the enneking staging of benign lesions?

  • Grade 1- Latent lesion-= enchondroma, non ossfiying fibroma
  • Grade 2- Active lesion =ABC/UBC/Chondroblastoma
  • Grade 3- Aggressive lesion = GCT

5

Describe the symptoms and signs of enchondromas?

Symptoms

  • asymptomatic- found incidentially- tibia
  • pathologcial fx- hand
  • Pain - uncommon
    • most chondrosarcomas have non mechanical pain

Signs

  • Angular deformity and shortening
  • blusih angiomas of maffucci

A image thumb
6

What is seen on xrays of enchondromas?

  • pop corn stippled calcification & rings
  • bone expansion
  • Lytic in hand
  • in Maffucci = angiomas are visible as calcified phleboliths 

A image thumb
7

How are chondrosarcomas different from enchondromas?

  • unlike enchondromas, chondrosarcomas display
     

    • cortical thickening and destruction

    • endosteal erosions and scalloping >50% of the width of the cortex 

      are larger (>5cm)



       

       

8

Describe the histology in enchondromas?

  • hypocellular with bland, mature hyaline cartilage (blue balls of cartilage) separated by normal marrow

A image thumb
9

Describe the tx of enchondromas?

  • non operative
    • observation
      • majority 
      • with serial xrays in 3-6months for 1-2 years
      • long term FU with multiple enchondromas
  • Surgery
    • intralesional curettage & bone graft
      • lesions that show any change in lesion
    • immobilisation, currettage & bone graft
      • path fx
      • immobilisatio until union then currettage & BG

10

What is periosteal chondroma?

  • A rare type of chondroma - benign
  • occurs on surface of long bones
    • under periosteum of distal femur,prox humerus and prox femur
    • 59% prox humerus
  • 10-20 yrs old

A image thumb
11

what are the symtpoms of periosteal enchondromas?

  • Pain
    • 2ary to irriation from tendons

A image thumb
12

What is seen on radiographs with periosteal enchondromas?

  • saucerization of underlying bone
  • punctate mineralisation in 1/3
  • well demarcated, shallow cortical defects

A image thumb
13

What is seen on histology of periosteal enchondromas?

  • Bland hyaline cartilage
  • small chondroid cells in lacunar spaces

A image thumb
14

What is the tx of periosteal enchondromas?

  • Operative
    • marginal excision including underlying cortex
    • if symptoms intereferring with function
    • lesion with reoccurif cartilage is left behind

15

What is an osteochondroma & multiple hereditary exostosis?

  • A Benign chrondrogenic lesion derived from aberrant cartilage from the periochondral ring that may take the form of
    • solitary osteochondroma
    • Multiple hereditary exostosis ( MHE)
  • ​Most common Benign bone tumour
  • Many asymptomatic
  • common adolescents/young adults
  • location on surface of bones, often at sites of tendon insertions
    • knee
    • prox femur
    • prox humerus
    • sugungal exostosis- at hallux
  • Autosomal Dominant
  • Mutation in EXT gene affects prehypertrophic chondrocytes of growth plate

A image thumb
16

What are the associated conditions of osteochondroma & multiple hereditary exostosis?

  • Secondary Chondrosarcomas
    • a malignant condition that results from malignant transformation of solitary osteochondromas or MHE
  • occurs older pts - 50 yrs
  • most common location is the pelvis

17

What is the prognosis of osteochondroma & multiple hereditary exostosis?

  • Risk of malignant change
    • <1% solitary osteochondromas
    • 5-10% in  multiple hereditary exostosis develop secondary chondrosarcomas

A image thumb
18

Define  multiple hereditary extosis

  • Disorder characterised by multiple osteochondromas
  • mutation affects the prehypertrophic chondrocytes of the physis
  • Autosomal Dominant
  • mutations in EXT1 ,EXT2, EXT 3 genes = tumour suppressor genes
  • ext1 individuals > severe presentation cf EXT 2 mutations
    • higher rate of osteosarcomas
    • more exostoses
    • more limb malignment

19

What is the prognosis of MHE?

  • 5-10% malignant change to chondrosarcomas
  • proximal lesions more likely to undergo malignant transformation than distal lesions

20

What are the symptoms of osteochondromas?

  • most asymptomatic
  • painless mass
  • NV compromise
  • mechanical symptoms

21

What are the symptoms of MHE?

Limb deformities

  • femoral shortening & LLD
  • coxa Valga
  • knee valgus- shortened fibular, patella dislocation
  • ankle valgus- shortened fibular
  • upper limb- ulna shortening, radial head dislocation/radial bowing
  • nay increase in acute apain should raise suspicion of malignancy

A image thumb
22

What is seen on radiographs with osteochondromas & multiple hereditary exostosis?

  • Sessile ( broad base)
  • Pedunculated ( narrow stalk)
  • found on surface of bones
    • >malignancy risk in sessile lesions
    • pedunculated point away from joint
  • cortex of lesion continous w cortex of native bone
  • medullary canal of lesion continous w medullary cavity of native bone

A image thumb
23

What is the histology of osteochondroma & multiple hereditary exostosis?

  • similar to normal physis with
  • cartilage cap consisting of hyaline cartilage
  • normal primary trabeculae
  • linear clusters of active chondrocytes
  • may have thin cartilage cap covering the lesion

A image thumb
24

What is the tx of osteochondroma 

  • Non operative
    • Observation
      • asymptomatic
  • Surgery
    • Marginal resection at base of stalk including cartilage cap
      • symptomatic lesions
      • delay surgery until skeletal maturity

25

What is the tx of multiple hereditary exostosis?

  • non operative
    • observation
    • most don't require intervention prior to reaching skeletal maturity
  • Operative
    • Surgical excision of osteochondromas
      • if dislocation of radial head
      • loss of forearm rotation
      • optimises chances of improved motion

26

tx of secondary chondrosarcomas

  • Operative
  • wide surgical resection
    • tx as typical condrosarcoma

27

what are the complications of osteochondroma & multiple hereditary exostosis?

  • Pseudoaneursym of popliteal artery
  • nerve compression
    • sciatic/common peroneal n, radial n
  • tendon compression
  • Chondrosarcoma
    • in adults cartilage cap >2cm assoc with increased risk of malignancy
    • mean age of dx 31yrs
  • Bursa formation
  • reocurrance
    • 2-5% post resection 

28

What are the differential dx of a surface lesion?

  • Osteochondroma/ MHE
  • Periosteal chondroma
  • Parosteal osteosarcoma
  • Periosteal osteosarcoma

29

Define chondroblastoma?

  • A benign chondrogenic lesion
  • differs to GCT by chondroid matrix
  • 2:1 male/female
  • most pts <25yrs
  • location= Epiphyseal lesion in young pts
  • apophysis/ distal femur/ prox tibia/ prx humerus/prox femur
  • Typically epiphyseal but may cross Physis
  • chromosome abnormalities 5/8

A image thumb
30

What is the prognosis of  chondroblastoma?

  • 1-2% benign chondroblastoma metastasise to lungs
  • similar to GCT
  • reocurrance 10-15% post surgicla resection

31

what are the symptoms of chondroblastoma?

  • Increase pain in involved joint

32

what is seen on xrays with chondroblastoma?

 

  • Well circumscribed epiphyseal lytic lesion- thin rim of sclerotic bone that is sharply demarcated from normal medullary cavity
  • lesions often cross physis into metaphysis
  • cortical expansion present
  • soft tissue expansion rare

A image thumb
33

What is the DDX of the radiograph?

Q image thumb

  • Giant cell tumour
  • chondroblastoma
  • clear cell chondrosarcoma
  • Osteomyelitis

34

What is seen on histology in chondroblastoma?

  • Chondroblastoma arranged in Cobblestones or dhicken wire- see pic
  • occasional multinucleated giant cells
  • scattered multinucleated giant cells with focal areas of chondroid matrix

A image thumb
35

Describe the tx of chondroblastoma?

  • operative
    • Extended intralesional curretage and bone grafting
      • ​in symptomatic pts
    • resection of rare benign pulmonary mets
      • ​if pulmonary mets exist

36

Describe chondromyxoid fibroma?

  • A rare
  • Benign chondrogenic lesion
  • characterised by variable amounts of chondroid, fibromatoid and myxoid elements
  • > in males
  • most common in 2nd/3rd decades of life
  • location
    • long bones- tibia, distal femur, pelvis ot hands
  • Mutations of chromosome 6 position q13
  • may reocur in 20-30% cases

A image thumb
37

What are the symptoms of chondromyxoid fibroma?

  • Long standing pain- months- years
  • Pain and swelling

38

What is seen on xrays with chondromyxoid fibroma?

  • Lytic, eccentric metaphyseal lesion
  • scalloped and sclerotic rim
  • calcifications RARE

A image thumb
39

What is seen on mri with chondromyxoid fibroma?

  • T1 weighted low signal
  • T2 weighted high signal

A image thumb
40

What is seen with histology with chondromyxoid fibroma?

  • Lobules of fibromyxoid tissue
    • hyperchromatic nuclei
    • lobules contain spindle shaped cells/stellate shaped
    • high power stellate cells seen

A image thumb
41

What is the tx of chondromyxoid fibroma?

  • Operative
  • intralesional curretage & bone graft ( or PMMA)
    • mainstay of tx

42

what are the complications of chondromyxoid fibroma?

  • Reocurrance 25% cases

43

What is Chondrosarcoma?

  • A Malignant chondrogenic lesion that can occur in 2 forms
    • primary chondrosarcoma
      • low grade,high grade
      • clear cell chondrosarcoma
      • mesenchymal chondrosarcoma
    • Secondary chondrosarcoma
      • arises from benign cartilage lesions
        • osteochondromas
        • Mutliple hereditary exostosis
        • Enchondromas (1% transformation)
        • Ollier's disease (20-45% transformation)
        • Maffucci ( 100% transformation)

A image thumb
44

Describe the epidemiology of chondrosarcomas?

  • Age
    • older pts 40-75 yrs
    • slight male predominance
    • locations= pelvis, prox femur, scapula
    • 85% of  chondrosarcomas are grade 1/2
    • axial and proximal extremity lesions have a more aggressive course
    • survical
      • grade 1 = 90%
      • Grade 3= 30-50%
      • de differentiated 10%

45

Describe clear cell chondrosarcomas?

  • malignant immature cartilagenous toumour
  • accounts for
  • common 30-040 yrs
  • insidious onset of pain
  • epiphyseal in location ( ddx from GCT ( giant cells) /chondroblastoma ( young pts)
  • locally destructive with potential to metastatsize

A image thumb
46

describe mesenchymal  chondrosarcomas?

  •  chondrosarcomas variant which present with a biphasic pattern of neoplastic cartilage with associated neoplastic small round blie cell component
  • younger pts
  • tx includes neoadjuvant chemo then wide surgical resection

47

What does a pt with  chondrosarcomas present with?

  • Pain - most common
  • slow growing mass/ bladder or bowel obstruction ( mass effect in pelvis)
  • 50% of de-differentiated  chondrosarcomas pc pathological fracture

A image thumb
48

What is seen on xrays in a pt with  chondrosarcomas?

  • Lytic or blastic reactive thicknening of cortex
  • low grade
    • similar appearance to enchondroma w additional cortical thickneing/expansion
  • high grade
    • cortical destruction & a soft tissue mass
  • intra-lesional 'popcorn mineralisation'
  • MRI- useful ti determine cortical destruction
  • bone scan- v hot

A image thumb
49

Describe the histology of  chondrosarcomas?

  • No needle biopsy as difficulties in diagnosis
  • low grade
    • few mitotic figures with bland histologic appearance
    • enlarged chondrocytes w plump nucleated lacunae - see pic
  • high grade
    • hypercellular stroma w blue balls of cartilage lesion which permeate the bone traecular
  • De differentitated- high level of spindle cells

A image thumb
50

What is the tx of  chondrosarcomas?

  • Operative
    • Intra-lesional curettage
      • grade 1 lesions
    • Wide local excision
      • ​grade 2/3 lesions
      • no sig role for radiotherapy/chemo as cartilage lesion is slow growing in typically intramedullary  chondrosarcomas
    • Wide local excision w multi-agent chemo
      • mesenchymal  chondrosarcomas
      • the role of chemo in de-differentiated very controversial