Bone Flashcards
Osteogenesis imperfecta: secondary to mutation in either the _ or _ genes, which guide type 1 collagen formation
COL1A1 or COL1A2
osteopenia, bowing, angulation, or deformity of long bones; multiple fractures; wormian bones of the skull
osteogenesis imperfecta
most common and least severe form of osteogenesis imperfecta, moderately severe bone fragility, blue sclera, hearing loss before age 30, some show opalescent teeth
type 1
most severe form of osteogenesis imperfecta, extreme bone fragility with frequent fractures, blue sclera, some opalescent teeth
type 2
most severe form of osteogenesis imperfecta beyond perinatal period; moderately severe bone fragility, blue sclera, kyphoscoliosis, some opalescent teeth
type 3
type _ of osteogenesis imperfecta; mild to moderate bone fragility, blue sclera fades with age, bone fractures, decrease after puberty, some opalescent teeth
type 4
hereditary disorder characterized by increased bone density; secondary to defect in osteoclastic function; the defect results in thickening of cortical bone and sclerosis of cancellous bone
osteopetrosis
_ form of osteopetrosis: normocytic anemia, symptoms secondary to cranial nerve pressure, pathologic fracture, skull deformities, hypertelorism, delayed tooth eruption, autosomal recessive
infantile (malignant)
_ form of osteopetrosis: milder, typically detected upon routine radiograph, no anemia, no cranial nerve compression, uncommon pathologic fracture, osteomyelitis, autosomal dominant
adult (benign)
numerous osteoclasts; lack of howship’s lacunae; dense bone formation
osteopetrosis
hereditary bone defect of the bone and clavicles; secondary to defect in the CBFA 1 gene, which guides osteoblastic diffentiation and bone formation; autosomal dominant inheritance pattern
cleidocranial dysplasia
frontal and parietal bossing; ocular hypertelorism; mid-face hypoplasia; open fontanels and wormian bones
cleidocranial dysplasia
hypoplasia or absence of clavicles; oral: numerous unerupted and supernumerary teeth, high arched palate
cleidocranial dysplasia
harmless process that may mimic pathology; may be secondary to aberrant bone regeneration following extraction, persistance of fetal marrow, and/or marrow hyperplasia
focal osteoporotic marrow defect
F>M, posterior mandible, asymptomatic, ill-defined radiolucency with fine trabeculations; normal bone, cellular hematopoietic marrow
focal osteoporotic marrow defect
focal area of bone density of unknown etiology; often confused with condensing osteitis
idiopathic osteosclerosis
age 20-40; mandibular first molar area; asymptomatic; well defined area of radiopacity
idiopathic osteosclerosis
disease of bone characterized by abnormal resorption and deposition of bone; unknown etiology; evidence has suggested a possible viral etiology; recent gene mutations have also been identified
paget’s disease of bone (osteitis deformans)
M>F; older individuals; monostotic vs. polyostotic; thickened enlarged, and weakened bones; often painful
paget’s disease
maxilla>mandible, enlarged alveolar ridges, may see spacing of the teeth
paget’s disease
radiograph: early- decreased radiodensity and altered trabeculation, later- patchy, sclerotic bone (cotton wool), hypercementosis
paget’s disease
increased serum alkaline phosphatase; normal calcium and phosphorous levels; elevated urinary hydroxyproline; osteoblastic formation and osteoclastic resorption of bone; basophilic reversal lines (mosaic or jigsaw pattern)
paget’s disease
_ develops in about 1% of Paget’s patients
osteosarcoma
non-neoplastic lesion of the jaws; significance lies in differentiation from brown tumor of hyperparathyroidism and association with cherubism
central giant cell granuloma
F>M, aggressive vs. non-aggressive, typically painless expansile, mandible, anterior to the first molar, may cross the midline, unilocular to multilocular radiolucency
central giant cell granuloma
spindle cell proliferation with variable numbers of multinucleated giant cells, erythrocytic extravasation and hemosiderin deposition, may see osteoid
central giant cell granuloma
rare autosomal dominant condition of the jaws, children, painless bilateral expansion, usually involves the posterior mandible
cherubism
“eyes turned up to heaven” appearance, multilocular, expansile radiolucencies, Noonan-like syndrome
cherubism
giant cell granuloma with more loosely arranged stroma, giant cells are focally aggregated, eosinophilic hyaline angiopathy
cherubism
benign empty or fluid-filled cavity of uncertain pathogenesis; actually not a true cyst (no epithelial lining); trauma-hemorrhage theory is probably the most popular of the potential explanations; others include ischemic necrosis, growth disturbances
traumatic bone cyst (simple bone cyst)
M>F, most involve the mandible; typically asymptomatic; radiolucency with sharply or ill-defined margins; scalloping between roots; thin lining of fibrovascular connective tissue along the bony wall; no epithelial lining
traumatic bone cyst
nonspecific term used to describe a number of processes that are characterized by fibrous connective tissue and mineralized tissue; due to similarity in histopathologic features, radiographic and/or clinical correlation is necessary to establish a definitive diagnosis
benign fibro-osseous lesions of the jaws
All of theses processes are _: fibrous dysplasia, cemento-osseous dysplasia, ossifying (cemento-ossifying) fibroma
benign fibro-osseous lesions of the jaws
developmental process secondary to a postzygotic mutation in the GNAS 1 gene; three froms; typically affects children during the first or second decade; M=F
fibrous dysplasia
_ fibrous dysplasia: slowly growing enlargement of a single bone; frequently involves the jaws; “ground glass” radiograph
monostotic
_ of fibrous dysplasia: typically involves the long bones; pathologic fracture, pain, deformity; cafe au lait pigmentation
jaffe-lichtenstein
_ of fibrous dysplasia: polyostotic features; cafe au lait pigmentation; multiple endocrinopathies (sexual precocity, gigantism, etc)
McCune-Albright syndrome
cellular fibrous stroma; irregular shaped trabeculae of woven bone (chinese script writing); skull and jaw lesions mature differently
fibrous dysplasia
african-american females; mandibular anterior area, asymptomatic; radiolucent to mixed appearance
periapical cemental dysplasia
F>M; whites>blacks; posterior mandible; solitary; asymptomatic
focal cemento-osseous dysplasia
adult black females; often symmetric involvement of all four quadrants
florid cemento-osseous dysplasia
Neoplasm of fibro-osseous differentiation; amount of cementum-like and osseous-like is variable from lesion to lesion and can be used to determine the terminology
ossifying fibroma
F>M; adults; mandibular molar-premolar area; typically an asymptomatic swelling
ossifying fibroma
unilocular; varying degrees of opacity; bowing of inferior cortex
ossifying fibroma
benign tumor of compact or cancellous bone; predominantly restricted to the bones of the craniofacial skeleton; may occur on the surface (periosteal) or within medullary bone (endosteal)
osteoma
young adults; asymptomatic; most commonly affect the mandible or condyle; circumscribed, sclerotic mass; sinus lesions- sinusitis, headache, exopthalmos
osteoma
autosomal dominant condition characterized by colorectal polyps (adenomatous), osteomas, epidermoid cysts of the skin, and desmoid tumors; chief significance lies in the potential for adenocarcinoma of the colon
gardner syndrome
osteomas, particularly of the jaws and bones of the skull; epidermoid cysts; multiple odontomas and impacted, supernumerary teeth
gardner syndrome
Without appropriate treatment of gardner syndrome, the incidence of colorectal carcinoma approaches _% in the elderly
100
Benign lesion of bone arising from osteoblasts; significant radiographic and histopathologic similarities with cementoblastoma
osteoblastoma
M
osteoblastoma
odontogenic neoplasm; probably the only true neoplasm of cementum; many similarities to osteoblastoma, warranting discussion with bone pathology
cementoblastoma
mandibular molar region; variable pain; expansile; calcified mass with radiolucent rim associated with the root of a tooth
cementoblastoma
similar to osteoblastoma, but fused to the tooth root; sheets or trabeculae of mineralized material; uncalcified matrix arranged in radiating columns at the periphery
cementoblastoma
malignancy of mesenchymal cells that are characterized by the ability to produce osteoid or bone; most common malignant tumor of bone; increased prevalence associated with Paget’s disease or previous radiation
osteosarcoma
M>F; avg. age 33 for jaw lesions; most occur in the area of the knee; swelling, pain, parasthesia, loosening of teeth; variable radiographic appearance; spiking root resorption; “sunburst or sunray” appearance; early widening of PDL
osteosarcoma
Osteosarcoma: Direct production of _ by mesenchymal cells
osteoid
variant of osteosarcoma that arises from the surface of bone; much better prognosis than intramedullary osteosarcoma; parosteal and periosteal trypes
peripheral (juxtacortical) osteosarcoma
malignant tumor of cartilage formation; about half as common as osteosarcoma; rarely affects the jaws
chondrosarcoma
M=F; avg. age ?; painless mass or swelling; separation or loosening of teeth; poorly defined radiolucency; scattered radiopaque flecks; may see the sunburst pattern
chondrosarcoma
controversial lesion of bone, probably neuroectodermal in origin; distinct genetic translocation - t(11;22); uncommon tumor of the jaws
ewing’s sarcoma
M>F; children and adolescents; swelling with intermittent pain; parasthesia and loosening of teeth; lytic bone destruction with ill-defined margins; “onion-skin” periosteal reaction; fever, leukocytosis; “blue cell tumor”
ewings sarcoma
most common malignancy involving bone; hematogenous spread; uncommon in the jaws?; breast, lung, thyroid, prostate, and kidney
metastatic tumors to the jaws
older patients; mandible>maxilla; pain, swelling, loosening of teeth, parasthesia; may mimic periodontal disease; moth-eaten radiographic appearance; breast and prostate may show mixed radiolucent/opaque pattern
metastatic tumors to the jaws
