Giant Cell Lesions of the Bone Flashcards

1
Q

What is a giant cell lesion of the bone

A
  • Lesions that arise in and destroy bone and are rich in osteoclast giant cells
  • Defined by their histological appearance
  • Doesn’t include foreign body granuloma, TB, sarcoidosis… All have giant cells but not in bone
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2
Q

What is the classification of giant cell lesions of the bone

A
  1. Giant cell granuloma
    a. Central giant cell granuloma
    b. Peripheral giant cell granuloma (giant cell epulis)
  2. Brown tumour of hyperparathyroidism
  3. Anerusymal bone cyst
  4. Cherubism
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3
Q

Clinical features of central giant cell granuloma

A
  • Solid lobulated mass of proliferating vascular connective tissue packed with giant cells (osteoclasts)
  • Benign tumour of unknown aetiology
  • More common in young females, but seen over a wide age range
  • Forms in alveolar ridge, anterior to 6s, more frequently in mandible but often in maxilla
  • Very expansible and may be destructive. May penetrate cortical bone and periosteum to produce a purplish soft tissue swelling
  • Frequently a slow-growing painless swelling, but growth is sometimes rapid
  • Lesions are typically several cm across
  • No association with hyperparathyroidism
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4
Q

Histological features of central giant cell granulomas of bone

A
  • Same as brown tumour of hyperparathyroidism and cherubism
  • Many multinucleate giant cells
  • Stroma of fibroblasts and mononuclear cells
  • Arranged in lobules separated by fibrous tissue septa, and sometimes a thin layer of osteoid or bone forms in them, hence honeycomb appearance on rx
  • Vascular and lots of haemorrhage - hence colour change clinically
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5
Q

How are central giant cell granulomas diagnosed

A
  • Expansion ± soft tissue mass
  • Bluish/maroon colour
  • Radiology, biopsy
  • Exclude hyperparathyroidism
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6
Q

What are the radiographic features of central giant cell granulomas

A
  • Radiolucent, well defined and scalloped outline
  • Unilocular or multilocular
  • Surrounded teeth may ‘float in air’ be displaced of occ resorbed (if very large)
  • Can make teeth mobile
  • May contain wispy opaque lines or honeycomb pattern
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7
Q

What is the treatment for central giant cell granulomas

A
  • Many granulomas grow slowly, and some have been shown to resolve spontaneously
  • However, the majority enlarge and require removal by curettage
  • ~15% of lesions recur, but a second curettage is usually curative
  • Fast growing granulomas indicate a higher risk of recurrence - surgery to excise the lesion with surrounding bone, particularly in the maxilla and facial bones where effective curettage in thin bones is difficulty to achieve
  • Maybe medical tx to partially control growth (used for rapidly enlarging Examples, when pt factors prevent immediate surgery or in children where facial growth might be affected by surgery)
    [injection of corticosteroids converts lesions to fibrous tissue, calcitonin, interferon alpha, bisphosphonates]
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8
Q

What are the clinical features of peripheral giant cell granulomas

A
  • ‘Giant cell epulis’
  • Unrelated gingival hyperplastic lesion (arises on gingiva)
  • No lesion in underlying bone
  • Histologically identical to the central lesion but is superficial and outside cortical bone
  • All ages, but commoner in children
  • Bluish maroon colour clinically
  • Local cause, often recent loss primary tooth
  • Tx: complete excision
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9
Q

What does an overproduction of parathyroid hormone (PTH) cause? And what can cause the overproduction?

A
  • Mobilises Ca from the skeleton and raises the plasma Ca level. Ca then lost in urine and can lead to renal stone formation and renal damage
  • Primary hyperparathyroidism: Causes is usually an adenoma of the parathyroid glands, uncommonly hyperplasia of the gland and rarely a parathyroid carcinoma
  • Secondary hyperparathyroidism: PTH hyper secretion in reaction to low Ca levels causes by vitamin D deficiency, gut malabsorption of Ca or renal failure (as kidney enzymatically activate Vit D)
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10
Q

What are the features of browns tumours of hyperparathyroidism

A
  • Common: malaise, hypertension, peptic ulcer and kidney stones
  • Significant bone disease now uncommon due to early tx
  • Generalised rarefaction of bone, loss of density and lamina dura
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11
Q

What causes brown tumours

A
  • Foci of osteoclasts in highly vascular stroma
  • Extensive internal haemorrhage
  • Breakdown of erythrocytes producing haemosiderin pigment and colours the lesion brown
  • Histologically indistinguishable from giant cell granuloma of the jaw
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12
Q

How do browns tumours of hyperparathyroidism present radiographically

A
  • Reduced bone density, loss of trabecular pattern and definition of lamina dura
  • In severe disease, these changes are more marked and pt may also develop one or more brown tumours (cyst like unilocular/multilocular radiolucencies)
  • Bony changes can cause pathological fractures but reverse with tx
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13
Q

What is the treatment for browns tumours of hyperparathyroidsm

A
  • Treat underlying condition
  • Primary
    surgical removal of causative adenoma
  • Secondary
    Ca supplementation to counter losses and correct any remediable underlying renal disease if possible
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14
Q

What are the two types of anerusymal bone cysts

A
  • Primary: benign neoplasms of bone; genetic (USP6 gene activated)
  • Secondary: develop in association with Fibro-osseous and other lesions, and are not neoplasms
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15
Q

What are the clinical features of aneurysmal bone cysts

A
  • Replace and expand bone with a vascular soft tissue containing numerous giant cells
  • Rapidly growing painless swelling => ballooning expansion
  • Rare in jaws; jaw lesions usually in mandibular ramus and angle
  • Affects pts usually between 10-20y
  • Form very expansile soap-bubble radiolucencies
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16
Q

What are the histological features of anerusymal bone cysts

A
  • Mass of blood-filled spaces with scattered giant cells

- No endothelial lining

17
Q

How are anerusymal bone cysts diagnosed

A
  • Biopsy necessary

- May be useful to detect genetic changes

18
Q

What are the radiographic features of aneurysmal bone cysts

A
  • Resemble ameloblastoma or giant cell granuloma
  • Radiolucent cavity appearing Multilocular or divided by faint septa
  • Adjacent teeth displaced, occasionally resorbed but vital
  • Extensive ‘blow out’ periosteal expansion with a thin cortex (rather than cortical perforation)
19
Q

What is the tx for anerusymal bone cysts

A
  • Thorough curettage
  • Sometimes recur
  • Surgery if extensive
  • For secondary cysts, associated lesion needs to be treated
20
Q

What are the clinical features of Cherubism

A
  • Inherited by autosomal dominant trait
  • Caused by one of several mutations in SH3BP2 gene that encodes a signalling protein
  • Causes multiple Multilocular bone lesions in the mandible and maxilla that develop in early childhood which enlarge then regress with skeletal maturation

Onset 2-4y with :

  • Symmetrical giant cell lesions in angles of mandible giving Chubby face
  • Alveolar ridges expanded
  • Gross lingual mandibular swellings may interfere with speech, swallowing and even breathing
  • Often missing teeth, as destroys tooth germs
  • Symmetrical involvement of maxilla in more severe cases
  • No pain but rapid growth until puberty
21
Q

What are the histological features of cherubism lesions

A
  • Lesions consist of loose fibrous tissue containing clusters of multinucleate giant cells, overall resembling giant cell granulomas or hyperparathyroidism
  • With time, giant cells become fewer and there is bony repair of the defect
22
Q

What are the radiographic features of cherubism

A
  • Bilateral large radiolucent lesions with fine bony septa producing a Multilocular appearance
23
Q

What is the treatment for cherubism

A
  • None if mild, regression is almost complete
  • In more severe cases, there is residual disfigurement => lesions respond to curettage or to paring down of excessive tissue
  • Surgery delayed until growth complete and regression assessed