Rank2 sem2

Question 1

1. Cherubism

Answer 1

= autosomal dominant condition causing enhanced osteoclast activity resulting in bone lytic lesions.

a. Clinical presentation
i. Posterior of mandible bilaterally ALWAYS +/-maxilla: full cheeks and jaw giving chubby face appearance
ii. Normal at birth Visible swelling ONSET 2yo  rapid enlargement  statis after from 7 to puberty  shrinks from puberty onwards  normal looking jaw by 40s
iii. Abnormalities with dentition:
1. Premature loss of deciduous
2. Failure of permanent teeth development
3. Lack of eruption
b. Histo:
i. Cellular and vascular fibrous tissue
ii. MNGC – focally collected around thin-walled blood vessels
iii. Eosinophilic deposits around vessels
iv. Deposition of fibrous tissue and woven bone
c. Radio
i. Mandibular:
1. Bilater multilocular radiolucent lesion
a. Starts at angle of mandible expands into BODY+ ramus, SPARES CONDYLES
ii. Maxillary
1. If present, affects tuberosities
2. May obliterate sinuses
d. TX: surgery only if major deformity; otherwise normal looking by 40yo

Question 2

2. Aneurysmal bone cyst

Answer 2

= AGGRESSIVE+ HIGH RECURRENT non-malignant cyst (with unknown etiology) comprised of numerous blood filled spaces not lined with endothelia

a. Clinical presentation: firm swelling on angle/body/ramus of mandible + pain and facial deformity
b. Histology
i. Fibrous tissue contains MNGC, erythrocytes and hemosiderin
ii. Fibrous tissue divides cyst into many spaces, which are filled with blood
iii. Blood filled spaces not lined by endothelium
iv. Blood not clotted (presumably anticoags produced by fibrous tissue)
c. Radio:
i. Multilocular radiolucency
ii. “blow-out” appearance due to aggressive expansion
d. Tx: enucleate + curette

Question 3

3. Hyperparathyroidism (brown tumors)

Answer 3

= consistently elevated PTH levels causing bone resorption

a. Etiology = PTH elevates blood Ca+2 levels by 1) increasing bone resorption 2) increasing renal resorption of Ca+2 and 3) activating vit D causing increased GI absorption of Ca+2
i. Primary hyperparathyroidism = tumor of parathyroid gland
ii. Secondary hyperthyroidism = rickets/osteomalcia (child/adult vit D deficiency/resistance)  low blood Ca+2 increased PTH to compensate
b. Clinical presentation:
i. SMGB (stone, bones, moans, groans)
1. S: Kidney stones
2. M: Coma/memory loss/depress
3. G: GI ulcers
4. B: Fibrous replacement of calcified bone
c. Histology: identical to central giant cell granuloma
i. highly vascular stroma rich with MNGC and spindle-shaped cells (i.e. fibroblasts, MNGC precursors and endothelium)
ii. hemosiderin staining
d. Radio:
i. Multiple multilocular radiolucent lesions
ii. Loss of/thinning of lamina dura (general)
e. Tx:
i. 1) if primary – remove parathyroid gland
ii. 2) if secondary – treat cause: Renal failure or ricket/osteomalacia
1. Kidney transplant
2. Vit D supplementation

Question 4

4. Sialadenosis

Answer 4

= non-inflammatory enlargement of major salivary gland due to abnormal neurosecretory control of acini, leading to granule buildup in acinar cells.

a. Etiology: cause of abnormal neurosecretory control can be
i. Hormonal (hypothyroid, pregnant, diabetic)
ii. Anorexia
iii. Alcoholism
iv. Medications
b. Clinical presentation: BILATERAL SWELLING OF MAJOR SAILVARY GLANDS (COMMONLY PAROTID)
c. Histology:
i. Enlarged acini densely packed with secretory granules
d. Tx:
i. Treat underlying cause if treatable
ii. If underlying cause not treatable:
1. Take medications increasing secretory granule release
2. Severe cases parotidectomy (i.e. partial/complete removal)

Question 5

5. Primary antral mucocele

Answer 5

= blockage of ostium (sinus opening), causes sinus to fill with mucin (not mucus) and turn into one large expanding cyst

a. Etiology:
i. Ostium blockmucin building up hydrostatic pressure expansionosteoclastic activity on adjacent bone
b. Clinical presentation
i. Swelling of cheek and mucobuccal fold
ii. Displacement of molars & destruction of adjacent bone
c. Histology:
i. Respiratory epithelial lining (i.e. ciliated columnar)
ii. Mucin filled
iii. Calcification in lumen (results in cloud antrum on radio)
d. Radio
i. Enlargement of sinus cavity w/ extension past canine and thin/perforated walls
ii. Cloudy antrum (b/c of the calcifications)
e. Tx:
i. Surgery NECESSARY (enucleation and curettage) otherwise will keep osmotic expansion and destruction of maxillary molar bone

Question 6

6. Surgical ciliated cyst

Answer 6

= mucin filled cyst in soft tissue lining of sinus

a. Etiology: trauma or surgery that results in respiratory epithelium becoming trapped in sinus soft tissue  proliferation of said epithelium and production of mucin to form cyst
b. Clinical/histo/radio/Tx: all identical to primary antral mucocele