- Mutliple bone involvement

- Multiple bone involvement + cafe-au-lait spots of skin (Jaffe-Lichtenstein syndrome)

- Multiple bone involvement plus cafe-au-lait spots of skin + multiple endocrinopathies (sexual precocity, pituitary adenoma, hyperthyroidism) (McCune-Albright syndrome)

- Multiple bone involvement + intramuscular myxomas: Mazabraud syndrome

- Multiple bone involvement

- While jaws may be involved, most prominent involvement is in long bones, causing pain and deformity, limb length discrepancy

- Jaws involved in as many as 1/3rd of patients, most commonly mandible

- Radiographically affected bone generally lucent, often w/ multilocular appearance; cortex thinned

- Cafe-au-lait pigmentation usually well-defined unilateral tan macules w/ irregular borders on trunk and thighs (may be congenital, oral mucosal macules may be present)

- McCune-Albright syndrome (sexual precosity most common endocrine distubrance, esp females)

Bone lesions managed individually and similarly to monostotic FD

Endocrine abnormalities much more serious and can be life-threatening

- 90% in females; predilection for 3rd-6th decades of life

- More common in whites than in blacks

- Most common site: posterior mandible

- Most often asympatomatic, detected on routine radiographic examination

- Female>male 10-14:1

- 70% in blacks

- Most commonly diagnosed b/t 30-50 y/o

- Usually asymptomatic, detected on routine radiographic exam

- Teeth vital unless coincidentally non-vital

- Usually multiple but occassionally solitary

- Well-circumscribed radiolucency w/ varying degrees of radiopacity

- Usually discreet lesions but occasionally fuse together linearly

- Age: most commonly in middle-aged to older adults

- Gender and race: vast majority in women and blacks

- Site: most commonly posterior portion of both jaws, most common bilateral and often quadrilateral

- May be completely asympatomic or produce dull pain, alveolar sinus tract, slight expansion of bone

- multiple foci of discrete although irregular radiolucencies/radiopacities

- Generally leaves intact the PDL space of teeth in area, although occasionally may fuse w/ teeth producing a hypercementosis-like appearance

- None of these lesions "shell out" of the bone

- Surgeon encounters multiple fragments of gritty tissue rather than a lesion which has enough internal integrity to remove in one piece

- Margins of lesions difficult if not imporssible for surgeon to determine

- THese surgical findings suggest a dysplastic rather than neoplastic process and also aid in diagnosis

- Replacement of native bone by proliferation of spindle-shaped fibroblasts and collagen fibers w/ numerous small blood vessels and varying amounts of woven bone, lamellar bone & cementum

- Diagnosis of fibro-osseous lesions depends on correlation of histologic findings w/ clinical radiologic and (if performed) surgical findings

- W/ periapical and florid osseous dysplasias w/ classic presentations, clinical, and radiographic features may be sufficient to make diagnosis making biopsy unnecessary - but only if everything is "typical"

- Suspected focal osseous dysplasia should be biopsied

- If any doubt, biopsy

- While patient may remain w/out signs or symptoms indefinitely, these lesions can occasionally become infected and necrotic

- Surgery should be very conservative and focused on relieving pain & infection w/ minimal surgical intervention

- Otherwise prognosis is good

True neoplasm occuring in the jaw and other craniofacial bones composed of fibrous tissue containing varying amounts of bone and cementum-like tissue

- True neoplasm of bone w/ concommitant growth potential

- Wide age range, most in 3rd/4th decades of life

- Female gender predilection: mandible >>> maxilla

- Mandibular premolar/molar region most common

- Small lesions often asymptomatic, detected on routine radiographic exam; larger ones may cause painless swelling; pain and paresthesia rare

- Well defined and unilocular lesion, may show sclerotic border, varying from completely radiolucent to almost completely radiopaque

- Root divergence or resorption of teeth in area may occur

- Large lesions may cause not only bucco-lingual expansion but also downward bowing of inferior mandibular border

Benign neoplasm composed of mature compact or cancellous bone

Essentially restricted to craniofacial skeletal, rarely if ever found in other bones

Radiographically: well-circumscribed opaque masses

Histologic: mature compact or cancellous bone, w/ or w/out included bone marrow

Small lesions may not be necessary beyond diagnostic biopsy; larger lesions should be removed surgically

Prognosis good: do not tend to recur after removal nor transform to malignancy

100% penetrance, but approx 1/3 of cases occur spontaneously

- Culprit gene identified as adenomatous polyposis coli (APC) tumor suppressor gene mapped to the long arm of chrom 5

- GS part of spectrum of diseass w/ familial colorectal polyposis

- Colonic polyps typically dev in 2nd decade of life and are adenomatous with high incidence of transformation to adenocarcinoma

- By age 30, 50% of pts dev colorectal adenoca; as patient ages, freq of malignant change approaches 100%

- Up to 90% of patients

- Most common of these are osteoms - can occur anywhere in skeleton but most common are skull, paranasal sinuses and mandible (most commonly at mnadibular angles and condyles)

- Osteomas usually noted during pubterty and precede detection of the colonic polyps - thus important early diagnostic sign

- Prophylactic colectomy usually recommended

- Symptomatic management of other features, including osteomas

- Variable, but without prophylactic colectomy, chance of colonic adenoca closely approach 100%