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Flashcards in Tumour like processes Deck (3)
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1

Paget's Disease 

Pathology is characterised by osteolysis followed by extensive poor-quality repair

  • 5 Stages
    • Pre-radiographic
    • Lytic or hot phase
    • Mixed or combined
    • Sclerotic phase
    • Malignant phase (rearly ocurs under 40yrs)

Radiographic Features 

  • Bone Density Changes
  • Coarsened Trabeculae
  • Cortical Thickening
  • Bone Expansion
  • Long Lesion, Subarticular Extension
  • Pseudo fractures (Looser’s Lines)
  • Bowing Deformities (shepards crook)
  • Pathological Fracture
  • Expansion of vertebrae
  • Thickening of endplates “picture frame” vertebrae
  • Coarsened trabeculae
  • Vertical striations of on vertebrae with thickening of the endplates

When the cortex of the bone can no longer contain the tumour (growing at a rapid rate) then it is a sign the disease has become malignant

Protrusio acetabuli can be similar to other diseases such as RA but with features such as loss of kohler’s line can help differentiate

2

Fibrous Dysplasia 

Non-cancerous group of disorders where normal bone is replaced with fibrous tissue. Lesions are often large and prone to expansion which leads to bone weakening.

  • 3 forms
    • Monostotic (70%) 14yrs M=F
    • Polyostotic (27%) 11yrs M=F
    • Polyostotic with endocrine abnormalities (3%) 8yrs
      • McCune-Albright syndrome
      • Females

Clinical Features 

  • Clinical features dependent on location and size
  • Monostotic
    • More likely to be asymptomatic
    • Upper femur: limb and intermittent pain
    • Persistent pain in long bone: pathological fracture
  • Polyostotic
    • More likely to be symptomatic than monostotic
    • More pronounced deformity/deformity bowing bones
    • Café au lait spots (irregular margins and size)

Radiographic Features 

Monostotic

  • Proximal femur, ribs
  • Diametaphyseal
  • Geographic lucent smoky (ground glass) lesion
  • Thick sclerotic margin (rind)
  • Occasionally septated (soap bubbles)
  • Expansion
  • Cortical thinning and scalloping
  • Elongated lesion
  • Pathological fracture Fibrous Dysplasia
  • Monostotic
  • Proximal femur, ribs
  • Diametaphyseal
  • Geographic lucent smoky (ground glass) lesion
  • Thick sclerotic margin (rind)
  • Occasionally septated (soap bubbles)
  • Expansion
  • Cortical thinning and scalloping
  • Elongated lesion
  • Pathological fracture

Polyostotic

  • Multiple lesions, may be unilateral
  • Pseudofractures
  • Deformities
  • Spine rarely involved

 McCune Albright Syndrome

  • Bone lesions often unilateral in distribution
  • Large area of café au lait spots on trunk
  • Precocious sexual development
  • Irregular menstruation at 5 or 6 yrs followed by development of secondary sexual characteristics

Cherubism

  • Familial fibrous dysplasia of jaws
  • 18mths-2yrs, regresses in puberty
  • Multilocular cystic lesions in mandible/maxilla

3

Neurofibromatosis

Non-cancerous (usually) group of disorders involving nerve sheath growths in the peripheral and/or central nervous system

Clinical + Radiographic Features 

  • Café au lait spots (in 50%)
    • Smooth margins, 6 or more >1.5cm
  • Fibroma molluscum
    • Skin tabs: multiple, soft, elevated
  • Neurofibromas
    • Mainly spinal and peripheral nerves

Bone changes

  • Spine
    • Scoliosis: short, angular, kyphosis
    • Vertebral scalloping:
      • Posterocentral: dural ectasia
      • Eccentric, unilateral: localised dumbell neurofibroma in IVF
  • Skull

    • Orbital defects, Lambdoidal defect, Macrocranium

  • Ribs

    • Scalloped, irregular

  • Long bones
    • Focal gigantism
    •  Bilateral symmetrical non-ossifying fibromas