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119 Cards in this Set
- Front
- Back
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Type of bone that is always pathologic in adult
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woven
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Which cell controls most of bone remodeling?
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Osteoblasts are the Boss of osteoclasts
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Genes responsible for patterning and architectural skeletal arrangement?
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homeobox genes
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craniorachischisis?
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failure of skull/spinal column closure that may lead to meningomyelocele or meningoencephalocele
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achondroplasia mutation
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point mutation in fibroblast growth factor receptor. Constant activation leads to decreased chondrocyte proliferation
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most common disease of the growth plate?
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achondroplasia, a major cause of dwarfism
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cinical fature of achondroplasia
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dwarf with normal head
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histology of achondroplasia?
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narrow/disorganized proliferation and hypertrophy zones; normal cortical appositional growth
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most common lethal form of dwarfism?
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Thanatophoric dwarphism; death at birth
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Mutation in thanatophoric dwarfism?
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fibroblast growth factor receptor; death at birth
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Type 3 OI?
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3 tree (progressively deforming and reatrded)
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Type 4 OI?
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fourt is short
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AR form of OI?
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usually Type II and lethal.
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OI mutation?
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in genes for a1 or a2 collagen chains
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Marble stone disease
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osteopetrosis!
where osteoclasts can't acidify the lacunae because of a carbonic anhydrase II deficiency. |
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Erlenmyer flask deformity?
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seen in osteopetrosis
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How to treat osteopetrosis?
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bone marrow transplant
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Virus with possible implication of osteopetrosis
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retrovirus
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Cause of localized osteoporosis?
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disuse osteoperosis
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Matrix madness?
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Paget's disease: osteitis deformans
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Paget's disease?
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osteoclastic fury with mixed osteolytic, mized, and osteosclerotic phases
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thick trabeculae and soft cortex?
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histologic findings of osteosclerosis
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Virus implicated in osteitis deformans?
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paramyxovirus which is found only in osteoclasts
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leontiasis ossea
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lion malformation face
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cortical cutting cones and dissecting osteitis?
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hyperparathyroidism
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bones affected by hyperparathyroidism?
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cortical bone
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What is a brown tumor?
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reactive, fibrous tissue, hemorrhage, and hemosiderin
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generalized osteitis fibrosa systica
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generalized severe severe hyperparathyroidism.
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Osteoclastic bone resorption, osteomalacia, osteosclerosis, growth retardation, osteoporosis
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skeletal changes of chronic renal disease
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ischemic infarction of bone
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osteonecrosis
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where may bones be osteonecrosed?
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medullary cavity of metaphysis or diaphysis or subchondral of epiphysis
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what is creeping substitution?
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when a bone is infarcted and old bone is kept as a scaffold for new living bone
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What can cause articular collapse and secondary osteoarthritis?
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subchondral infarct/ osteonecrosis
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presentation of diaphyseal or metaphysial infarcts
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silent unless its large
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Most common cause of osteomyelitis?
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S aureus
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osteomyelitis associated with GU infection of iv users?
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E. coli, Pseudomonas, Klebsiella
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osteomyelitis associated with neonates?
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H influenzaue and group B strep
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osteomyelitis associated with sickle cell?
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salmonella
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What can cause septic arthritis, especially in children?
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subperiosteal absecess due to osteomyelitis
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Pott disease?
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extrapulmonary TB that affects the spine; if both disks adjacent to an intervertebral disk is affected, that avascular tissue dies and collapses, caseates...
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saber shin
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Deposition of reactive bone due to acquired syphilis
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Gardner syndrome?
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Fibromatosis, adenomatous polyposis, multiple osteomas, epidermal cysts
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subperosteal osteomas most common where?
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skull and facial bones
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a benign, small bump of bone that projects from the cortex
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osteoma, looks like reactive bone
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Where and who most often gets the following tumor:
less than 2 cm, produces PGE2? |
25 yr old Men on cortex of appendicular skeleton
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Round to oval masses of hemorrhagic critty tan tissue?
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either osteoid osteoma or osteoblastoma dependent on size, response to aspirin, and location
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most common primary malignant tumor of bone age distribution?
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bimodal: 20 yrs old and the eldery with Paget's or other bone conditions
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Bone tumor associated with mutation of Rb, p53, or overexpression of MDM2?
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Osteosarcoma
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Most common presentation of Osteosarcoma?
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metaphysis (right around where proliferation is!), primary, solitary, intramedullary, poorly-differentiated with a bony matrix
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What other cancer is associated with osteosarcoma?
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20% have pulmonary metastasis at time of diagnosis
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Codman's triangle?
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raised periosteum with reactive periosteal bone formation shadow seen on radiography
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Mushroom shaped cartilage with bony stalk that communicates with long bone?
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osteochondroma (exostosis)
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Why can't osteochondromas form on skull?
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it can only form in bones of enchondral origin
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multiple hereditary exostosis inheritance pattern?
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AD, genetically heterogenous and seen in children
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most common intraosseus cartilage tumor?
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chondroma
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What is Ollier's and Mafucci's?
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syndrome of multiple enchondromas
if patient also has hemangioma's, its marfucci's (Ollier's is Only chondroma) |
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gray-blue, translucent, nodular with periphery undergoing enchondral ossifcation?
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chondroma; center often calcifies and dies
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Tumor associated with hemangiomas, ovarian cancer, and brain gliomas?
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Marfucci syndrome of mutliple enchondromas
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O ring sign?
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well circumscribed oval lucency with thin rim of radiodense bone
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scant mineral matrix with chicken wire pattern of calcification?
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Chondroblastoma
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well-defined radiographic lucency with spotty calcification?
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chondroblastoma
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Rarest cartilage tumor target of choice?
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chondromyxoidfibroma in teens and metaphysis of long bones.
Mixed up teenagers, Myxoid, and Metaphysis! |
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Chondromyxoid fibroma morphology?
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well circumscribed, solid, glistening tan-gray:
poorly formed hyaline with a small foci of calcification and fibrous septae. you will see prominent stellate cells. The "X" in myxoid should remind you |
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first and second most common malignant tumor of bone?
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1) osteosarcoma
2) chondrosarcoma |
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Types of chondrosarcomas?
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clear cell type and mesenchymal
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Location and morphology of clear cell type chondrosarcoma?
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large sheets of chondrocytes with clear cytoplasm surrounded by osteoclast giant cells (osteosarcomas are by metaphysis while these are epiphyses)
reactive bone formation occurs between lesions |
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mesenchymal chondrosarcoma morphology and location?
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islands of hyaline surrounded by sheets of small round cells, by central skeleton (shoulder, pelvis, ribs)
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Chondrosarcomas and enchondromas are both sheets of hyaline cartilage. What should help differentiate?
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enchondromas occur in distal extremities and undergo enchondral ossifcation in the periphery while chondrosarcomas are axial and have weird cells surrounding the tumor
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Why are chondrosarcomas of higher grade more radiolucent?
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they don't have time to form reactive bone like low grades with a thick cortex
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Tx for chondrosarcomas?
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low grade: excision
high grade: chemo and excision because of aggressiveness |
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Fibrous cortical defect underlying problem?
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development defect, very common in children
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Large fibrous cortical defect?
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nonossifying fibroma not detected until adolescence
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storiform pattern?
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Nonossifying fibroma
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Localized developmental arrest that results in a benign tumor-like lesion?
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fibrous dysplasia
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which form of fibrous dysplasia often has craniofacial involvement?
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polyostotic fibrous dysplasia
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McCune Albright syndrome?
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somatic mutation of guanine nucleotide binding protein. Causes:
polyostotic fibrous dysplasia with cafe au lait spots and endocrinopathies |
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Histologic manifestation of fibrous dysplasia?
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lots of fibroblast proliferation with curvilinear trabeculae of random woven bone (looks like a boomerang)
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Why is there no osteoblastic rimming in fibrous dysplasia?
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because this is a developmental arrest so there is no bone building!
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Fibrosarcoma?
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collagen producing sarcoma that just spills out of bone!
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Tx for chondrosarcomas?
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low grade: excision
high grade: chemo and excision because of aggressiveness |
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Tx for chondrosarcomas?
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low grade: excision
high grade: chemo and excision because of aggressiveness |
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Fibrous cortical defect underlying problem?
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development defect, very common in children
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Fibrous cortical defect underlying problem?
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development defect, very common in children
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Large fibrous cortical defect?
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nonossifying fibroma not detected until adolescence
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Large fibrous cortical defect?
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nonossifying fibroma not detected until adolescence
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storiform pattern?
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Nonossifying fibroma
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storiform pattern?
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Nonossifying fibroma
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Localized developmental arrest that results in a benign tumor-like lesion?
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fibrous dysplasia
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Localized developmental arrest that results in a benign tumor-like lesion?
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fibrous dysplasia
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which form of fibrous dysplasia often has craniofacial involvement?
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polyostotic fibrous dysplasia
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which form of fibrous dysplasia often has craniofacial involvement?
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polyostotic fibrous dysplasia
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McCune Albright syndrome?
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somatic mutation of guanine nucleotide binding protein. Causes:
polyostotic fibrous dysplasia with cafe au lait spots and endocrinopathies |
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McCune Albright syndrome?
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somatic mutation of guanine nucleotide binding protein. Causes:
polyostotic fibrous dysplasia with cafe au lait spots and endocrinopathies |
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Histologic manifestation of fibrous dysplasia?
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lots of fibroblast proliferation with curvilinear trabeculae of random woven bone (looks like a boomerang)
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Histologic manifestation of fibrous dysplasia?
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lots of fibroblast proliferation with curvilinear trabeculae of random woven bone (looks like a boomerang)
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Why is there no osteoblastic rimming in fibrous dysplasia?
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because this is a developmental arrest so there is no bone building!
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Why is there no osteoblastic rimming in fibrous dysplasia?
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because this is a developmental arrest so there is no bone building!
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Fibrosarcoma?
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collagen producing sarcoma that just spills out of bone!
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Fibrosarcoma?
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collagen producing sarcoma that just spills out of bone!
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Tx for chondrosarcomas?
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low grade: excision
high grade: chemo and excision because of aggressiveness |
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Fibrous cortical defect underlying problem?
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development defect, very common in children
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Large fibrous cortical defect?
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nonossifying fibroma not detected until adolescence
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storiform pattern?
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Nonossifying fibroma
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Localized developmental arrest that results in a benign tumor-like lesion?
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fibrous dysplasia
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which form of fibrous dysplasia often has craniofacial involvement?
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polyostotic fibrous dysplasia
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McCune Albright syndrome?
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somatic mutation of guanine nucleotide binding protein. Causes:
polyostotic fibrous dysplasia with cafe au lait spots and endocrinopathies |
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Histologic manifestation of fibrous dysplasia?
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lots of fibroblast proliferation with curvilinear trabeculae of random woven bone (looks like a boomerang)
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Why is there no osteoblastic rimming in fibrous dysplasia?
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because this is a developmental arrest so there is no bone building!
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Fibrosarcoma?
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collagen producing sarcoma that just spills out of bone!
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malignant fibroblasts in herringbone pattern
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fibrosarcoma
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fibroblast proliferation with bizarre multinucleated tumor giant cells in metaphysis of long bones?
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malignant fibrous histiocytoma
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Homer-Wright rosettes?
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central fibrially spaces surrounded by tumor cells (tumor cells make the petals)
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Translocation of Ewings lead to?
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dominant oncogene that increases tumor cell proliferation
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most common bone sarcomas in children?
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1) osteosarcoma
2) ewing sarcoma |
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Small round blue cells in diaphysis of long bone and flat bone of pelvis in child?
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Ewing Sarcoma
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Radiography of Ewing sarcoma?
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onion skining layers of reactive bone
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Giant cell tumor?
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a purely lytic osteoclastoma that is benign but may metasize to lungs!
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Why is giant cell tumor in metaphysis only in adolescents?
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they are confined by the growth plate!
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Blastic metatastic disease in axial skeleton?
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prostate
kidney, lung, GI, melanoma metastasis to bones are lytic because of cytokines |
