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28 Cards in this Set

  • Front
  • Back
BFOL definition and types
Benign Fibro-osseous lesions
- Normal bone architecture is replaced by fibroblasts and collagen fibers with variable mineralization
- A process, and not a specific diagnosis

Fibrous dysplasia
Ossifying fibroma
- Juvenile ossifying fibroma
Cemento-osseous dysplasia
- Florid
- Focal
- Periapical
Fibrous dysplasia cause, types, radiographic appearance and cause, histology, treatment
Mutation of the GNAS 1 gene
Monostotic: 80-85%. Can become Craniofacial fibrous dysplasia of MX is involved and extend to other bones
Polyostotic: up to 75% of skeleton. Pathologic fractures and hockeystick leg deformity. Females more common. Jaffe syndrome & McCune-Albright syndrome

Radiographic: Radiolucent Early Cystic, mid Sclerotic, Late phase Mixed "pagetoid"
- Characteristic poorly circumscribed radiopacity like "ground glass" or "orange peel".
- Opacity cased by superimposition of disorganized bone trabeculae
- Shows superior displacement of inferior alveolar canal with narrowing or loss of PDL***

Histology shows immature woven bone with no osteoblasts.
- Bone matrix arises from metaplasia of fibrous tissue.
-Shows curvilinear shapes like "Chinese characters."

- Small lesions can be excised
- Large polyostotic lesions are monitored and shaved down. 20% have regrowth
- Radiation is contraindicated due to risk of post-radiation sarcoma
Jaffe vs McCune Albright syndrome
Jaffe syndrome:
Polyostotic fibrous dysplasia with cafe au lait pigmentation

McCune Albright syndrome
- Polyostotic fibrous dysplasia with cafe au lait pigmentation and multiple endocrinopathies.
Central Ossifying fibroma
- True neoplasm with 90% mandible and 5x Female predicament
- Presents with a painless downward bowing of the inferior cortex
- histology shows well demarcation from surrounding bone. May have fibrous capsule so more easily removed than fibrous dysplasia.
Juvenile Ossifying fibroma characteristics, clinical signs, and histology
More aggressive than central ossifying fibroma
- Maxilla more often
- Can cause exophthalmos and proptosis
- Histology shows small strands or "seams" of osteoid matrix as clusters of multinucleated giant cells
Periapical Cemento-osseous dysplasia characteristics, radiographic appearance, histology
- Most common in the apices of mandibular incisors
- Up to 14x female predicament
- Teeth are invariably vital

Radiographic
- Early lesions are well demarcated radiolucencies
- Then becomes mix of osteolytic and osteoblastic lesions

Histology
- Mature lesions show more calcification
- No evidence of inflammation
Focal Cemento-Osseous dysplasia
- Usually seen in posterior mandible and found more often in females
- May be uni-quadrant florid cemento osseous dysplasia.
- Doesn't separate cleanly from bone
Florid Cemento-Osseous dysplasia characteristics, etiology
Limited to Jaws and may be multiquadrant. Can become infected to cause confusion with chronic sclerosing osteomyelitis
- Simple bone cysts may develop within these lesions
- Calcified material is dense and avascular prone to poor healing and osteomyelitis
- Etiology is an abnormal reaction in bone.
Cherubism characteristics, radio, histology
Painless bilateral expansion of jaws
- Autosomal dominant and early onset at about 7yrs then stabilize after puberty

Radiographic
- Most commonly see multiquadrant multilocular radiolucencies

Histology
- Fibrous connective tissue stroma with multinucleated giant cells and RBCs with eosinophilic deposites
- In differential with CGCG, brown tumor, and ABC but cherubism shows Eosinophilic cuff like deposits around small blood vessels
Multilocular radiolucencies involving multiple quadrants
Cherubism

Nevoid basal cell carcinoma syndrome
Proliferative Periostitis
Periosteal reaction to the presence of inflammation
- Presents as several layers of reactive vital bone that parallel each other and expand the surface cortex
- Occlusal radiograph shows onion skinning but with an intact cortex. If unintact, suspect neoplastic rather than inflammatory process
Hyperparathyroidism osseous changes, triad, Histology
Osseous
- Generalized loss of lamina dura
- Brown tumor showing up as a uni or multilocular radiolucency
- Osteitis fibrosa cystica develops in long standing brown tumors

Traid
- Stones: Kidney
- Groans: Duodenal ulcers
- Moans: Mental disturbances

Brown tumors consist of scattered multinucleated giant cells with extravasated RBCs. So must determine if patient has hyeprparathyroid in central giant cell lesions.
Paget's disease characteristics, radiographic, d/d, histology, treatment & prognosis
Presents as aching bone pain
- Shows leontiasis ossea due to enlargement of maxilla more than mandible
- Elevated serum alkaline phosphatase

Radiographic
- Shows cotton wool appearance of bone
- Hypercementosis and Loss of lamina dura

- May look like fibrous dysplasia when maxilla is expanded. But pagets in 40's while FD in less than 20yr

Histology
- Shows basophilic reversal lines resulting in jigsaw puzzle or mosaic pattern
- See resorptive and osteoblastic phase

Treatment for pain is asprin
- May see spontaneous development of malignancy and form a sarcoma 1-13% of time
Osteopetrosis characteristics, types
Defect in osteoclast function
- Thickening of cortical bone and sclerosis of cancellous bone

Infantile(Malignant) - Diffuse sclerosis with marrow failure. Delayed tooth eruption and osteomyelitis of jaws are common complications.
Adult(Benign) - Axial skeleton with significant sclerosis with little or no effect on long bones
Langerhans Cell disease characteristics, types, histology
aka Histiocytosis X, Langerhans cell granuloma
- Associated pain or tenderness beginning in posterior jaws as loss of alveolar bone

Types
- Acute disseminated form: multisystem, infants, and high mortality
- Chronic disseminated form: Unisystem, children. Presents radiographically with punched out radiolucencies and teeth floating in air. Triad of exopthalmos, Diabetes insipidus, and lytic defects of bone.
- Eosinophilic granuloma: Solitary lesions without visceral involvement. Adults and low mortality


Histology
- Diffused infiltrate of mononuclear cells with kidney shaped nuclei and variable eosinophils
- Also show rod shaped Birbeck granules in cytoplasms of Langerhans cells
Alveolar bone loss in children
Juvenile periodontitis
Langerhans cell histiocytosis
Papillon-Lefevre syndrome
Burkitt's lymphoma
Cyclic neutropenia/agranulocytosis
Palmoplantar Keratodermans
Autosomal recessive Germline mutation in capthepsin C gene
- Includes Papillion Lefevre syndrome and Haim Munk syndrome
- Associated with premature periodontal disease and appears as teeth floating in air
Multiple Myeloma characteristics, radiology, clinical
Monoclonal expansion of malignant plasma cells in older 63yr olds
- Presents with Bone pain and pathologic fractures
- Kappa light chain Bence-Jones proteins in urine

- Punched out radiolucencies with no sclerotic margin

- Hyperglobulinema mostly IgG
- Macroglossia due to deposition of amyloid in tissues
Osteoma types
Endosteal - Have to become large before causing cortical expansion
- Clinical D/D includes odontoma, condensing osteitis or any calcifying odontogenic lesion

Periosteal - Asymptomatic hard swelling on bone surface that presents radiographically like a mushroom

**- Multiple osteomas syspect Gardner syndrome.
Gardner Syndrome
Have APC gene associated with Familial adenomatous polyposis syndromes
- Multiple osteomas of face and jaw and supernumerary teeth
Osteoid Osteoma/Osteoblastoma characteristic differences, radio, histology
- Benign tumors of osteoblasts
Osteoid - Less than 2cm and alleviated by asprin. Most often in leg bones
Osteoblastoma - Greater than 2cm and not relieved by asprin. Most common in vertebrae.

*- Can grow at an alarming rate and presents as a sunburst type RO/RL lesion. Often confused with osteosarcoma

* - Same histology attached to a tooth would be a cementoblastoma
Central Giant Cell Granuloma clinical, histology
Presents as displaced teeth* Different from Myxoma which grows around roots and ameloblastoma which resorbs roots
- 15%-20% recurrence

- Same histology as brown tumor of hyperparathyroidism, and wall of aneurysmal bone cyst. Must look at PTH levels to rule out hyperparathyroidism
Central Hemangioma characteristics, radio, histology, types, treatment
Slowly expanding swellings that show mobility and bleeding.

- Shows multilocular radiolucencies

- Unencapsulated lesion composed of multiple endothelial lined vascular channels

Cavernous - Large dilated vessels. Majority
Capillary - Small vessels


Can use angiogram to identify feeder vessels and tie them off or sclerose them. Commonly facial artery and internal maxillary artery
- All central lesions that look like hemangiomas must be aspirated prior to biopsy or surgical resection
Desmoplastic fibroma characteristics, histology, treatment
Osseous counterpart of soft tissue fibromatosis
- 50% in metaphysis of humerus and tibia. Mandibule is 4th place
- Presents as a painless swelling

- Classified as benign and acts locally aggressive. Radical surgery may be required
Chondroma & Chondrosarcoma characteristics, radio, histology, treatment,
Cartilaginous tumor that can recur many times and eventually metastasize

Chondrosarcoma - Symmetric widening of PDL* Present in both chondrosarcoma and Osteosarcoma. Also poorly defined radiolucency

- Cartilage shows varying degrees of cellularities with two or more cells in a lacuna.

- Don't respond to radiation or chemotherapy or radiation. Radical surgical excision is required for all cartilagenous tumors arising in the jaws. Can metastasize to lung or bone.
Osteosarcoma characteristics, clinical signs, radio, histology, treatment,
Most common primary malignant bone tumors under 40
- 2nd most common after multiple myeloma

- Presents with swelling and pain.
- MD>MX, Male>Female

- Classic sunburst pattern and symmetric widening of PDL

- Osteosarcoma must demonstrate osteoid arising directly from a sarcomatous stroma

- Unlike chondrosarcoma, Osteosarcoma is treated with chemo and surgery. Radiation is not enough.
Peripheral Osteosarcoma characteristics, type,
Arises on surface of bone, usually long bones
- Parosteal: Well differentiated but will recurr with less than en bloc surgery. Mushroom like growth on bone surface with no elevation of periosteum
- Periosteal: Higher grade with prominent cartilaginous component. Prognosis is better than medullary osteosarcoma but worse than parosteal.
Ewing's Sarcoma characteristics, clinical, histology, treatment
Disease of children and adolescents. More common in mandible and caucasians

Very malignant tumor that presents with pain, swelling, fever, and may mimic an infection or osteomyelitis
- Paresthesia and loosening of teeth are common findings in jaw lesions

- Cells contain glycogen granules in the cytoplasm so immunohistochemical stains are needed to confirm the diagnosis.

- Combined therapy with radiotherapy, chemo and surgery. Gnathic Ewing's sarcoma has a lower mortality rate