giant cell lesions Flashcards
cherubism
- Genetic
o Autosomal dominant trait
o Mapped to chromosome 4p16
▪ Gene (SH3BP2) mutation → pathological activation of osteoclasts & disruption in jaw dev. - May be sporadic: 2˚ to spontaneous mutations
- Facial appearance similar to cherub (Angels)
- Male predilection
cherubim - clinical features
Peak incidence: 2-5years
Progress until puberty,
stabilisation, regression
Painless bilateral expansion of
the posterior mandible(angles
and ascending rami)
Rim of sclera visible beneath
the iris
“eyes upturned to heaven”
Maxilla may be affected
Widening of alveolar ridges
Tooth displacement
Delayed eruption
cherubim - radiographic features
Bilateral
multilocular
expansile
radiolucencies in
the posterior
mandible
cherubim - histological features
Multinucleated giant cells, with an osteoclastic origin
Oesinophilic cufflike deposits around blood vessels
Vascular fibrous connective tissue
NB: SPECIFIC DIAGNOSIS REQUIRES CLINICOPATHOLOGICAL CORRELATION.
All giant cell lesions have similar histological features
cherubim - differential diagnosis
- brown tumour of hyperparathyroidism
- peripheral and central giant cell granuloma
- aneurysmal bone cyst
final diagnosis
Based on clinical and radiographic information with support from microscopic findings
Familial history
Bilateral presentation
Defined age predilection
No abnormal biochemical findings: normal parathyroid hormone and calcium levels
treatment
No treatment
Lesions undergo remission and involution
Contouring for aesthetic reasons
giant cell granuloma
intra-osseous: occurs within bone
Non-neoplastic
Age range: 2-80 years
80% in patients 30yrs
Female predilection
70% arise in the mandible
Anterior mandible >
posterior mandible
Asymptomatic
Painless bone expansion,
If symptomatic
pain , paraesthesia, cortical
plate perforation, mucosal
ulceration
central giant cell granuloma - nonaggressive lesions
Few/no symptoms
Slow growth
No cortical
perforation - no soft tissue involvement
No root resorption
No tendency to recur
central giant cell granuloma - aggressive lesions
Pain
Rapid growth
Cortical perforation
Root resorption
Tendency to recur
central giant cell granuloma - radiographic features
Radiolucent
Unilocular/ multilocular
Well defined Noncorticated
Size: 5mm -10cm
single>multiple
Multiple
Exclude: cherubism in childhood, Brown tumour of hyperparathyroidism in adults
central giant cell granuloma histological features
Multinucleated giant cells
Focal/diffusely arranged
Ovoid to spindle shaped
mesenchymal cells
Macrophages
RBC extravasation/
haemosiderin
NB: clinicopathological
correlation
central giant cell granuloma - treatment
- curettage
- radical surgery (aggressive lesions)
- non surgical interventions:
corticosteroids
calcitonin
interferon alpha - 2a
peripheral giant cell granuloma - clinical features
Soft tissue counterpart of the central
giant cell granuloma
Non-neoplastic
Gingiva/edentulous alveolar ridge
Red/ red-blue nodular mass
<2cm in diameter
Age range: 31-41 years
Female predilection
Mn > Mx
peripheral giant cell granuloma - differential diagnosis
pyogenic granuloma
kaposi’s sarcoma
cavernous haemangioma
plasmablastic lymphoma
peripheral giant cell granuloma - radiographic features
cupping of the underlying alveolar bone
peripheral giant cell granuloma - histopathological features
Multinucleated giant cells
Ovoid/spindle shaped mesenchymal cells
Haemorrhage
50%- ulceration
Chronic inflammatory infiltrate
Reactive bone formation
Dystrophic calcifications
peripheral giant cell - treatment
Local surgical excision
Scaling and polishing to minimise source of irritation and minimise recurrence
10% recurrence rate
hyperparathyroidism
- excessive production of parathyroid hormone (PTH)
- PTH produced in parathyroid glands in response to a decrease in serum calcium levels
primary hyperparathyroidism
Uncontrolled production of PTH as a result of
Parathyroid adenoma: 80-90%
Parathyroid hyperplasia: 10-15%
Parathyroid carcinoma: 1%
MEN types 1/2
Hyperparathyroidism jaw tumour syndrome
secondary hyperparathyroidism
PTH continuously produced in response to chronic low serum calcium levels
Chronic renal disease
hyperparathyroidism - clinical features
Primary Hyperparathyroidism
60yrs<
Female predilection
primary hyperparathyroidism - triad of symptoms
Triad of symptoms:
Bones
Generalised loss of lamina dura
Decrease in trabeculae density
Blurring of trabeculae pattern
(ground glass appearance)
Stones
Renal calculi due to elevated
calcium levels
Metastatic calcifications
Abdominal groans
Duodenal ulcers
brown tumour of hyperparathyroidism - radiological features
Well demarcated
Uni/multilocular radilucencies
Solitarymultiple
mandible, ribs, clavicles, pelvis
