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1504 > Exam 4 > Flashcards

Exam 4 Flashcards

1
Q

Congenital disorders of bone

A
  • osteogenesis imperfecta
  • achrondroplasia/Thanatophoric dwarfism
  • osteopetrosis
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2
Q

acquired diseases of bone

A
  • osteoporosis
  • paget disease (osteitis deformans)
  • rickets/osteomalacia
  • hyperparathyroidism
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3
Q

Osteomyelitis

A
  • pyogenic osteomyelitis
  • tuberculous osteomyelitis
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4
Q

Bone Tumors

A
  • bone forming tumors
  • cartilage forming tumors
  • fibrous and fibroosseuous tumors
  • misc. bone tumors
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5
Q

Bones General Information

A
  • 206 bones in body
  • organic—35
  • inorganic—65
  • inorganic= calcium hydroxapatite
  • 99% Ca and 85% P and 65% of Na and Mg in bones
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6
Q

Osteogenesis Imperfecta

A
  • brittle bone disease
  • genetic disorders—dom. negative mutation
  • defective synthesis of type 1 collagen
  • a1 and a2 chains of type 1 collagen affected
  • too little bone
  • 4 subtypes
  • type 2 variant= fatal in utero
  • type 1= normal lifespan= blue sclerae, hearing loss, small misshappen teeth
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7
Q

Achondroplasia

A
  • most common form of dwarfism—dispropotionate shortening of proximal extremities
  • bowing of legs
  • lordotic (Sway back) posture
  • autosom. dom
  • activating point mutation in fibroblast growth factor receptor 3—FGFR3
  • FGFR3 inhibits proliferation and function of growth plate chondrocytes
  • affects bones that grow by endochondral ossification
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8
Q

Thanatophoric Dwarfism

A
  • lethal variant of dwarfism
  • 1/20000 live births
  • thanatophoric = “death loving”
  • FGFR3 missense/point mutations
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9
Q

ostepetrosis

A
  • rare genetic disorder
  • *-reduced osteoclast-mediated bone resorption**
  • stone bone or marble bone
  • bone dense and stone like—fractures ike chalk

-extracellular space between osteoclast and bone is a like a 2ndary lysosome, acidified by proton pump

  • 1 variant= carbonic anhydrase II deficiency
  • fractures + cranial nerve + recurrent infections
  • hepatosplenomegaly
  • bone marrow transplant (bc of monocyte precursor for osteoclasts)
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10
Q

Osteoporosis

A
  • *-acquired: reducing bone mass**
  • localized: “disuse osteoporosis of limb”
  • generalized: entire skeleton
  • –primary (senile & post menopausal)
  • –secondary (metabolic disease, vitamin deficiencies, drug exposures)
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11
Q

Osteoporosis Morphology

A
  • *-reduction of osteblastic deposition**
  • corticles are thin
  • haversian canals are dilated
  • trabeculae reduced in thickness and lose interconnections
  • osteoclast activity is present but not dramatically increased
  • *-after bone loss= fractures
  • post menopausal- trabecular bone loss is severe= fractures and collapse of vertebral bodies
  • senile= cortical bone loss prominany, gractures weight bearing bones (neck)**
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12
Q

Osteoporosis Pathophysiology

A

-Peak bone mass= genetic, physical, nutrition

-w/ menopause= dec serum estrogen
inc IL1, IL6, TNF levels
inc RANK RANKL
inc osteoclast

-aging= dec replicative activity of osteoprogenitor cell
dec synthetic activity of osteoblasts
dec biologic activity of matrix growth factors
reduced physical activity

aging/menopause= osteoporosis

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13
Q

Osteoporosis Pathogenesis

A
  • age related
  • hormonal influence
  • physical activity
  • genetic factors—vit D receptor polymorphisms
  • calcium nutritional state—adolescent girls (less peak bone mass)
  • secondary causes—steroids, cigs, and alc
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14
Q

Osteoporosis Clinical

A
  • depends on bones involved
  • thoracic and lumbar vertebral fractures common
  • difficult to diagnose early
  • prevention & treatment should be before 30
  • –adequate dietary Ca intake
  • –Vit D supplementation
  • –Regular exercise regimen
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15
Q

Pagets Disease

A
  • osteitis deformans
  • 3 stages
  • osteolytic stage (bone reporption)
  • mixed osteoclastic-osteoblastic stage (bone deposition)
  • osteosclerotic stage (exhaustion)

-affects 2.5% population

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16
Q

Paget Disease Morphology

A
  • monostotic/polyostotic
  • lytic stage= numerous osteoclasts
  • mixed phase= bone surfaces lined by numerous osteoblasts
  • newly formed bone shows pathognomic–mosaic pattern like a jigsaw puzzle
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17
Q

Paget Disease—pathogeneis

A
  • inflammatory disorder
  • parmyxovirus infection
  • IL1 , IL6, MCSF
  • can activate osteoclasts
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18
Q

Paget Disease—Clinical Course

A
  • depends on extent and site of disease
  • paget diseae= monostotic in 15% cases—tibia, ilium, femur, skull, and humerus
  • polyostotic in 85%—axial skeleton or proximal femur
  • involves ribs, fibulae, small bones
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19
Q

Rickets and Osteomalacia

A
  • bc of Vit D deficiency
  • phosphate depletion
  • impairment of mineralization—soft bones

-osteoporosis = bone is mineralized but less bone there

  • rickets= in kids w/ interference w/ bone deposition
  • osteomalacia= in adults w/ remodeled bone indermineralized
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20
Q

Hyperparathyroidism

A

-parathormone has central role in maintaining serum Ca

  • osteoclast activation
  • inc resorption of Ca by renal tubules
  • inc urinary excretion of phosphate
  • inc synthesis of active Vit D, 1, 25, by kidneys
  • primary and seconday (chronic renal disease—renal osteodystrophy)
  • inc in osteoclast activity w/ bone resoprtion
  • brown tumor of hyperparathyroidism—osteitis fibrosa cystica= cystic change in bone
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21
Q

Fractures

A
  • most common
  • classify: complete/incomplete, close/compound, comminuted/displaced

-occurs at site of previous diseae (cyst, malig tumor, brown tumor bc of exess PTH)

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22
Q

Fracture Healing

A
  • blood clot= soft callus that holds ends together
  • bone progenitors deposit new woven bone= bony callus
  • remodeling
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23
Q

Fracture Healing—Disruption

A
  • displaced and comminuated fractures
  • inadequate immobilization
  • infection
  • inadequate Ca or phosphorus, Vit Deficiencies
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24
Q

Osteonecrosis

A
  • avascular necrosis
  • ischemic necrosis followed by infarction
  • vascular compression or disruption (fracture)
  • steroid admin
  • thromboembolic disease (calsson disease-N bubbles)
  • primary vessel disease (vasculitis)
  • sickle cell crisis
  • at the end of long bones
  • 50000 joint replacement to annually in USA for osteonecrosis
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25
Q

Osteomyelitis

A
  • inflammation of bone
  • infection in bone
  • pyogenic
  • tuberculous
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26
Q

Pyogenic Osteomyelitis

A
  • bc of bacteria
  • reach bone via hematogenous, extension from skin/soft tissue, or traumatic implantaion after comoound fractures
  • staph aureus= most common
  • e.coli and group b strep= in neonates, salmonella in sickle cell disease

-mixed bacterial infections (anaerobes)- after bone trauma

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27
Q

Pyogenic Osteomyelitis Morphology

A

-entrapped bone dies and forms sequestrum

-periosteal abcesses can form (esp in kids)

-periosteal rupture can lead to draining sinus

-involucrum= reactive woven or lamellar bone

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28
Q

Pyogenic Osteomyelitis Clinical Features

A

-acute systemic illness—malaise, fever, leukocytosis, throbing
diagnosis= radiologic findings

-treatment= antibiotic and surgical drainage

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29
Q

Tuberculous Osteomyelitis

A
  • mycobacterial infection—w/in developing countries
  • 1-3% of pulmonary Tb have bone infections
  • hematogenous spread
  • long bones and vertebrae are favored spots
  • tuberculosis of vertebral bodies—pott diseae-clinically serious
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30
Q

Bone Tumors

A

-most common tumors in bone are metastatic tumors

-primary bone tumors

  • morphologically diverse—benign to malig
  • benign more common
  • matrix producing and fibrous tumors=common
  • benign= osteochondroma & fibrous osseus defect
  • malig= osteosarcoma> chondrosarc>ewing sarcoma
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31
Q

Osteoma

A
  • benign—similar to bone histologically
  • in head/nec, paranasal sinus
  • middle age, solitary hard, bone, exophytic tumor
  • multiple lesions in gardner syndrome (hereditary)
  • mechanical problems, not locally aggressive or malig
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32
Q

Osteoid Osteoma & Osteoblastoma

A
  • benign= similar histo features
  • appear in teenage & 20s, male:female 2:1 for OO

-distinguished by size and clinical presentation

-OO: <2 cm diameter under periosteum, cortex of proximal femur/tibia or posterior spinal elements
localized pain
relieved by aspirin

-osteoblastoma: most arise= vertebral colum, >2cm
pain, difficult to localize
not relieved by aspirin

malig transformation rare unless treated by irradiation

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33
Q

Osteosarcoma

A
  • bone producing malig mesenchymal tumor
  • *-after myeloma and lymphoma, osteosarcoma = common primary malig tumor of bone**
  • 20% primary bone cancers
  • all ages, 75% patients less than 20
  • second peak in elderly= paget diseae, bone infarcts, previous irradiation
  • any bone can be affected

-most arise in metaphyseal regions of long bones———-60% knees, 15% hip, 10% shoulder, 8% jaw

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34
Q

Osteosarcoma Morphology

A

-gross= gritty-appearing, gray-white tumors
destroy surrounding cortex, producing soft tissue masses
spread in medullary canal and replace in marrow
penetrate epiphyseal plate/enter joint space

-microscopy= tumor cells vary in size/shape
hyperchromatic nuclei, bizzare giant cells
production of mineralized/unmineralized (osteoid) bone by malig cells is essential for diagnosis of osteosarcoma

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35
Q

Osteosarcoma patho

A

mutations

  • Rb gene mutation in majoriy
  • TP53 mutations
  • mutations in genes that regulate the cell cycle

—develop at sites of greatest boneg rowth

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36
Q

Osteosarcoma clinical

A
  • painful enlarging masses/patho features
  • triangular shadow on xray—codman triangle
  • heamtogenous spread, metastases to lung
  • chemotherapy & limb salvage therapy—70% survival
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37
Q

Osteochondroma

A
  • benign, cartilage capped tumors attached by bony stalk to skeleton
  • solitary—late adolescence & adulthood
  • multiple hereditary osteochondromas= early kid= autosom dom.
  • inactivation of EXT1 and EXT2 genes= encode glycosyl transferases—required for polymerization of heparin sulfate
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38
Q

Osteochondroma—part 2

A
  • only in bones of endochondral origin
  • near growth palte of long bones—knee, stops growing once growth is completed
    • pelvis, scapula, ribs
    • rarely short tubular bones of hands/feet
  • slow growing masses- pressure effects, stalk fractures
  • solitary rarely malignant
  • multiple hereditary carries risk of malig
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39
Q

Chondroma

A

-benign neoplasm of hyaline cartilage
w/in medulla= enchondroma, on surface of juxtacortical chondroma

-20-50 yrs

-solitary, metaphyseal region of tubular bones (hands and feet)

  • ollier disease= multiple chondromas, unilateral
  • mafucci syndorme= multiple chondroma & hemangiomas
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40
Q

Chondrosarcoma

A
  • malig= intramedullary & juxtacortical
  • >40 yrs, male:female= 2:1

-convential chondrosarcoma= most common variant, arises in medullary cavity
malig hyaline and myxoid cartilage

-pelvis, shoulders, ribs, not in distal extremities

  • painful, progressively enlarging masses
  • 5 yr survival rate—indolent
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41
Q

Fibrous Cortical Defect & Nonossifying Fibroma

A

-developmental abnomarlities, not true neoplasms

-benign fibroblasts & activated macrophages
=multinucleate forms

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42
Q

Fibrous Dysplasia

A
  • benign, all comp of normal bone present but dont mature
  • 3 patterns
  • monostotic= 70% cases= ribs, femur, jawbones, skull, humerus
  • polyostotic= craniofacial involvement in 50%
  • polyostotic disease w/ cafe au lait skin pigmentation and endocrine abnomalities= precocious puberty = McCune Albright Syndrome
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43
Q

Metastatic Tumors

A
  • pathways= direct extension, lymphatic/hematogenous, intraspinal seeding
  • any cancer can spread to bone
  • greater predilication= 75% Ca, prostate, breast, kidney, lung
  • in kids= neuroblastoma, wilms tumor, osteosarcoma, ewing sarcoma, & rhabdomyosarcoma
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44
Q

Arthritis

A
  • osteoarthritis
  • rheumatoid arthritis
  • juvenile rheumatoid arthritis
  • seronegative spondyloarthopathies
  • gout
  • pseudogout
  • infectious arthritis
45
Q

joint tumors/ tumor like lesions

A
  • ganglion & synovial cysts
  • tenosynovial giant cell tumor
46
Q

Osteoarthritis

A
  • degenerative joint disease= most common joint disorder
  • inevitable part of aging, physical disability >65 yr
  • degeneration of articular cartilage—2ndary changes in underlying bone
  • counter-intuitively, not inflammatory
  • Primary= oligoarticular: hands, knees, hips, & spine
  • Unusual= 5% in youth= predisposing condition, involves 1 or more predisposed joints
  • women= knees & hands affected
  • men= hips
47
Q

Osteoarthritis Morphology

A
  • early= alterlations in comp and structure of matrix
  • fibrillation & cracking of matrix= superficial layers of cartilage degraded

-chondromalacia= soft granular appearing articular cartilage

  • full thickness cartilage loss
  • bony eburnation= exposure/ smoothening of bone
  • osteoblastic activity
  • small fractures= dislodge bone pieces into joint spaces= joint mice
  • fracture gaps= synovial fluid to enter= fibrous walled cysts
  • mushroom shaped osteophytes—severe=pannus
48
Q

Osteoarthritis Pathogenesis

A
  • mechanical stresses and aging
  • genetic factors= polymorph and mutations in genes encoding matrix & signaling molecules
  • early= cartialge contains more H20 less proteoglycn
  • then= type 2 collagen network diminished, chondrocyte apoptosis

-overall= cartilage tensile strength & resilience compromised

49
Q

Osteoarthritis Clinical

A
  • insidious, affects patients in 50s-60s
  • *-deep aching pain exacerbated by use

-**
morning stiffness, creptius (grating/popping)

-limitation of range of movememnt

  • pinching of nerve roots
  • heberden nodes= osteophytes in fingers
50
Q

Rheumatoid Arthritis

A
  • systemic, chronic inflammatory autoimmune disease
  • –common, women:men= 3:1, peak 4th decade of life
  • affects tissues but mainly joints
  • non-suppurative synovitis, destroys articular cartilage and underlying bone
  • disabling arthritis
  • skin, heart, BV, muscles, lungs—may resemble lupus or scleroderma
51
Q

Rheumatoid Arthritis Patho

A
  • genetic factors= 50% risk = HLA DRB1 locus
  • environment—agents? inflammation & smoking
  • immune system= CD4 (TH1 Th17), b cells, plasma, macrophages
  • cytokine mediated inflammation- CD4 T cells
  • Antibodies= AntiCCP (cyclic) and Anti IgG of self (rheumatoid)
52
Q

Rheumatoid Arthritis Morphology

A

-Symmetric arthritis= affects small joints
PIP & MCP joints
spine. upper cervical
rarely hip joint

-Chronic papillary synovitis
synovial hyperplasia & prolif
dense cell infiltrates—CD4, plasma, macrophages
inc vascularity
neutrophils & organizing fibrin on synovial
inc osteoclast in underlying bone

-Pannus
prolif synovial lining
inflam cell, granulation tissue, fibrous CT

53
Q

Rheumatoid Arthritis Simple Morph

A
  • periarticular soft tissue edema
  • articular cartilage below pannus eroded
  • subarticular bone may be attached
  • pannus fills joint space
  • fibrosis & ossification (anyklosis)
  • rheumatoid subcutaneous nodules (25% patients)—mechanical pressure
54
Q

Rheumatoid Arthritis Clinical

A
  • weakness, malaise, low grade fever
  • arthritis develops insidously

-aching/stiffness of joints, mainly morning (morning stiffness

  • joints enlarge, motion limited= compelte ankylosis
  • vascular involvement—raynaud phenomenon
55
Q

Rheumatoid Arthritis Dx

A
  • radiologic finding
  • sterile, turbid synovial fluid w/ decreased viscosity, poor mucin clot formation & inclusion-bearing neutrophils
  • anti CCp and rheumatoid factor
  • disease can stabilize or regress…mostly it is progressive
  • joint destruction in 10-15 yrs
56
Q

Juvenile Rheutamoid Arthritis

A
  • disorders, multifactorial
  • large joints are affected—symptoms before 16 yrs old
  • joint warmth, swelling, pain
  • Th1, Th17 involved
  • TNF, IL1, and Il6 cause damage
57
Q

Seronegative Spondyloarthropathies

A
  • in ligamentous attachments to bone, not synovium
  • sacroiliac joints, w/ w/o other joint
  • no rheumatoid factor
  • HLA B27
58
Q

Gout

A
  • affects 1% of pop, prediliction for males
  • excessive amts of URIC ACID in tissues and body fluids
  • uric acid comes from purine metabolism
  • monosodium urate crystals precipitate and induce acute inflam. reaction
  • recurrent episodes of acute arthritis, w/ large crystalline aggregates TOPHI
  • primary 90% cases, secondary (hyperuricemia)
59
Q

Gout Morphology

A

-Acute arthritis—long slender monosodium rate crystals in neutrophils
synoviam edematous, congested, inflamm cells
crystal resolubilizes, episode remits

-Chronic Tophaceous Arthritis–evolves from acute attacks
synovium hyperplastic, thickened, fibrotic, pannus
severe= cartilage & bone erosion= ankylosis

-tophi= pathognomonic for gout

60
Q

Gout Clinical

A
  • men > women
  • symptoms after 30 yrs
  • risk factors= obesity, excess alc, purine rich, diabetes, metabolic syndrome, renal failure

-four stages= asymptomatic hyperurecimia
acute gouty arthritis
intercritcal gout
chronic tophaceous gout

61
Q

Psuedogout

A
  • chondrocalcinosis
  • Ca pyrophosphate crystal deposition in cartilage
  • meisci, IV disc, articular surface
  • overproduction/decreased breakdown of pyrophosphate
  • mutations in rare familial form
  • therapy supportive
62
Q

Suppurative Arthritis

A
  • bactermia
  • hemophilus influenzae—children under 2

-staph aureus in older kids and adults

  • salmonella= sickle cell
  • sudden onset of pain, swelling ,restricted range of motion
  • fever, leukocytosis, elevated ESR
  • joint aspirate (purulent fluid), culture= diagnostic
63
Q

Lyme Arthritis

A
  • borrelia burgdorferi= spirochete
  • transmitted by deer tick= arthropod borne, leading arthro born disease

-in lyme CT

stage 1- borrelia multiple at site of inocultation under skin

stage 2- early disseminated stage= skin lesions, lymphadenopathy, migratory joint/muscle pain, cardiac arrhythmias, mening—IgM and IgG

Stage 3- late disseminate stage= chronic arthritis (large joints) & encephalities

64
Q
A
65
Q

Joint Tumors

A
  • primary joint neoplasms= uncommon
  • benign
  • relect the cells and tissue types native to joints===synovial membrane, vessels, fibrous tissues, cartilage
66
Q

Tumor Like Lesions

A

-ganglion & synovial cyst

  • ganglion is a small cyst near joint capsule or tendon
  • cyst w/o true lining, degeneration of CT
  • asymptomatic, pea sized swelling
  • bible therapy—whack w/ large tomr to rupture cyst
  • herniation of synovium through joint capsule or massive enlargememnt of bursa can produce synovial cyst (bakers cyst in popliteal)
67
Q

Tenosynovial Giant Cell Tumor

A
  • closely related benign neoplasms of synovium
  • reactive proliferations
  • consistently associated w/ acquired 1;2 translocation: fusion of collagen 6A3 gene to coding sequence of MCSF
68
Q

1-Tumors of Adipose Joint
2-Fibrous Tumors and Tumor Like Lesions

A

1-lipoma
liposarcoma

2-reactive proliferations
fibromatoses
fibrosarcoma

69
Q

1-fibrohistiocytic tumors
2-skeletal muscle tumors
3-smooth muscle tumors

A

1-benign fibrous histiocytoma (dermatofibroma)
pleomorphic fibroblastic sarcoma/pelomorphic undifferntiated sarcoma

2-rhabdomyosarcoma

3-leiomyoma
leiomyosarcoma

70
Q

Soft tissue

A

is NOT:
epithelium
bone
cartilage
CNS
hematopoeitic
lymphoid

71
Q

soft tissue tumors

A
  • from pluripotent mesenchymal stem cells
  • soft tissue sarcomas= annual diagnosis
  • <1 % cancers but 2% of cancer deaths
  • arise w/o antecedent cause
  • rare, radiation, burn, toxins
  • small minority w/ genetic syndromes
  • mainly in lower extremities, especially thigh
  • 15% in children, incidence increases w/ age
72
Q

Soft Tissue Tumors Prognosis

A
  • diagnostic classification= histology & immunohistochemistry, EM, cytogenetics, & molecular genetics
  • grading scale= scale 1 to II

-staging=
>20 cm, 80% cases have metastases
<5 cm, 30% cases show metastases

73
Q

Lipoma

A
  • benign tumor of fat
  • most common soft tissue tumor in adults
  • solitary lesions, if many= hereditary syndrome
  • mobile, slowly enlarging, painless masses
  • angiopomas = manifest local pain
  • complete excision completely curative
74
Q

Liposarcoma

A
  • malig neoplasms w/ adipocyte differentiation
  • 5th & 6th decade of life
  • deep soft tissue or in retroperitoneum
  • well differentiated tumor- good prognosis
  • myxoid liposarcoma= adult fat cells & primitive cells
  • cells indivative of fatty differentiation= lipoblasts
75
Q

Fibrous Tumors: Reactive Proliferations:
Nodular Fasciitis

A
  • self limited fibroblastic proliferation
  • volar forearm, chest, back
  • solitary, rapid growth, occasionally painful
  • recures rarely after excision
76
Q

Fibrous Tumors: Reactive Proliferations:
myositis Ossificans

A
  • metaplastic bone
  • proximal muscles of extremities in athletic adolescents & young adults
  • affected area swollen & painful
  • painless, hard, mass
  • DD= extraskeletal osteosarcoma
77
Q

Fibromatoses

A
  • fibroblastic prolif
  • grow infiltrative fashion, recur after surgical removal
  • some lesions locally aggressive, dont metastasize
  • tumor cells= fibroblasts & myofibroblasts
  • cosmetic problem & pain—recur after removal

superficial= superficial fascia= genetically diff from deep

deep= desmoid= ab wall & muscles of trunk/extremities. isolated or multiple (garnder syndrome)

78
Q

Fibrosarcoma

A
  • malig neoplasms composed of fibroblasts
  • typically in deep tissues of thigh, knee, and retroperitoneal
  • grows slowly
  • recurs locally after excision (>50% cases)
  • metastasize (>25% cases)= lungs
  • soft, unencapsulated, infiltrating masses
  • hemorrhage & necrosis
  • all differentiation
79
Q

fibrohistiocytic tumors: benign fibrous histiocytoma

A
  • cured by simple excision-dermatofibroma—relatively common, benign
  • in adults, small <1 cm, circumscribed
  • blank, interlacing spindle mixed w/ foamyy lipid rich histiocyte cell
  • borders infiltrative, but not extensive local invasion
  • cured by simple excision
80
Q

fibrohistiocytic tumors: pleomorphic: fibroblastic/undifferntiated sarcoma

A
  • waste basket category for poor differentitated sarcomas
  • cellular pleomorphism, bizarre multinucleate cells * storiform architecture
  • proximal extremities or retroperitoneum
  • aggressive, unless excised metastatic
81
Q

skeletal muscle tumor: rhabdomyosarcoma

A
  • most common
  • soft tissue sarcoma of kids < 20
  • head, neck, GI tract

3 types: embryonel= sarcoma botryides near vag, alveolar,
and pleomorphic

82
Q

Rhabdomyosarcoma Dx and Clinical

A
  • round/ elongated (tadpole or strap cells)
  • diagnosis by skeletal muscle differentiation

-immunohistochemical demonstrationg= skeletal muscle specific transcription factors, and intermediate filament desmin

  • aggressive neoplasms
  • combo of surgery, chemotherapy, and radiation
  • malig curape in 2/3 children
  • pleomorphic varient in adults= prognosis less favorable
83
Q

Smooth Muscle Tumors: Leiomyoma

A
  • benign smooth muscle tumors
  • in uterus
84
Q

Smooth Muscle tumors: Leiomyosarcoma

A
  • 10-20% soft tissue sarcomas
  • adults, mainly females
  • skin, deep soft tissue of extremities, retroperitoneum
  • superficial tumors excised—good prognosis
  • retroperitoneal difficult to resect/spread
85
Q

Synovial sarcoma

A
  • thought too recapitulate synovium but neoplastic cells not like synovium
  • 10% cases have intraarticular
  • 20s to 40s
  • classic biphasic synovial sarcoma
  • monophasic sarcoma occurs

-treated aggressively w/ limb saving surgery and chemotherapy—5 yr survival rates: 25-62

86
Q

Disorders of Peripheral Nerves

A
  • patterns of peripheral nerve injury
  • disorders associated w/ periphereal nerve injury
87
Q

Disorders of Neuromuscular Junction

A

myasthenia gravis
lambert eaton syndrome
misc. neuromuscular junction disorders

88
Q

disorders of skeletal muscle

A

patterns of skeletal muscle injury
inherited disorders of skeletal muscle
acquired disorders of skeletal muscle

89
Q

Peripheral nerve sheath tumors

A
  • schwannomas and neurofibroimatosis type 2
  • neurofibromas
  • malig peripheral nerve sheath tumors
  • neurofibromatosis type 1
  • traumatic neuroma
90
Q

Pattterns of Peripheral Nerves

A
  • axonal: secondary demyelination (wallerian degeneration)
  • demyelinating
  • polyneuropathies—stocking and glove distribution
  • polyneuritis multiplex—radial nerve palsy w/ loss of sensation in foot
  • simple mononeuropathy—carpal tunnel syndrome
91
Q

Peripheral Nerve Injury: Guillain Barre Syndrome

A
  • life threatening, progressive demyelinating disorder
  • ascending motor weakness, resp muscle failure, death
  • inflammatory
  • infection (c. jejuni, EBV, CMV, HIV) or vaccine trigger
  • nerve roots & proximal nerve segments
  • humoral and cellular immune responses
  • plasmapheresis
92
Q

Peripheral Nerve Injury: Diabetic Peripheral Neuropathy

A

-most common causes of peipheral neuropathy

-long standing disease

-subtypes: autonomic neuropathy
lumbosacral radiculopathy, &
peripheral sensorimotor polyneuropathy= common

  • pathogenesis is complex (AGE, hyperglycemia, ROS)
  • strict glycemic control is the best form of therapy
93
Q

Myasthenia Gravis

A
  • autoimmune disorder
  • 3/100000, more females over males

-disease transferred by serum

-anti-acetyl choline receptor Ab

  • 60% of cases have thymic B hyperplasia and the other 20% have thymoma
  • thymectomy= improve clinical symptons in patients w/ thymic involvement
94
Q

Myasthenia Gravis part 2

A
  • reportitive EP stiumlation- muscle weakness worsens
  • cholinesterase inhibiting drugs= improves muscle strength
  • cholinesterase inhibitory drugs, immunosuppresion plasmapheresis
  • thmectomy in patients w/ thymic involvement
95
Q

Lambert Eaton Syndrome

A
  • autoimmune disorder
  • disease can be transferred by serum
  • Ab inhibit function of presynaptic Ca channels
  • usually in association w/ small cell lung carcinoma- a paraneoplastic syndrome
  • inhibiting Ach-esterase doesnt help
  • immunosuppression & plasmapheresis
  • prognosis worse than myasthenia gravis bc of association w/ small cell lung ca
96
Q

disorders of peripheral skeletal muscle

A
  • can be inherited or acquired
  • type 1- slow twitch “aerobic or type 2- fast twitch “anaerobic”

-patterns of muscle injury is via atrophy—primary or secondary(neurogenic)

97
Q

Muscle Fiber Atrophy

A
  • neurogenic- fiber type grouping & atrophy
  • prolonged disuse- focal/generalized… type 2>type1
  • glucocorticoids- proximal muscles and type 2
98
Q

Inherited Skeletal Muscle Disorders

A
  • duchennes muscular dystrophy
  • becker muscular dystrophy
  • myotonic dystrophy
  • limb girdle muscular dystrophy
  • fascioscapulohumeral
  • distal
  • ocular
  • oculopharyngeal
99
Q

Duchenne Muscular Dystrophy

A

-DMD and beckers= most common of dystrophinopathies

-dystrophin is absent in DMD—mutated and partial function in beckers

  • dystrophin on X chromosome—427 kD
  • dystrophin glycoprotein complex important for skeletal and also cardiac muscle function
100
Q

DMD stats

A

-1/3500 birthds

  • symptoms at age 5
  • wheel chair bound by adolescence
  • dead by adulthood

beckers= less severe symptoms

101
Q

Myotonic Dystrophy

A
  • sustained involuntary contraction of muscle groups
  • CTG= trinucleotide repeats on Chr 19
  • affects mRNA for dystrophila-myotonia protein kinase
  • “anticipation”
102
Q

Limb-Girdle Muscular Dystrophy

A
  • heterogenous genetic basis
  • autosomal
  • mutations in caveolin & sarcoglycan proteins
103
Q

Channelopathies: Ion Channel Myopathy

A
  • myotonia
  • relapsing hypotonia due to abnormal serum K

-hyperkalemic periodic paralysis—-mutations in skeletal muscle Na channel protein SC4NA

-malignant hyperthermia—mutations in ryanodine receptor, a Ca channel release protein
triggered by halogenated anesthetics

104
Q

Metabolic myopathies

A
  • inborn errors of metabolism
  • glycogen synthesis and storage disorders
  • abnormalities in lipid handling

—mitochondrial myopathies= parking lot inclusions

105
Q

Inflammatory Myopathies: Primary

A

-polymyositis= autoimmune=inc MHC 1 on myofibers, CD8 T cell attack

-dermatomyositis- autoimmune type 1 IFN and autoAb
most common inflammatory myopathy in kid
in adults = paraneoplastic

-inclusion body myositis= most common inflam. in adults over 60
hyperphosphorylated tau, amyloid

other immune liek SLE can also be involved

106
Q

peripheral nerve sheath tumors: schwannomas & NF2

A
  • encapusulated, easily separated from underlying nerves
  • most commonly affects 8th cranial nerve
  • tinnitus & hearing loss
  • sporadic, 10% occurs as neurofibromatosis type 2 NF2
  • NF2 patients have bilateral schwannomas
  • multiple schwannomas, meningiomas, ependymomas
107
Q

Neurofibromas

A
  • most common on skin and peipheral nerves
  • 3 types
  • localized cutaneous neurofibromas
  • plexiform neurofibromas—NF1
  • diffuse neurofibromas—-NF1

-plexiform difficult to remove surgically (contrast to schwannomas)

108
Q

Malignant Peripheral Nerve Sheath Tumors

A
  • 50% of malig peripheral nerve sheath tumors arise in NF1
  • 3-10% of NF 1 patients develop malig
  • large poor defined soft tissues masses
  • highly cellular, w/ features of overt malig
109
Q

Neurofibromatosis Type 1

A
  • autosomal dom
  • mutation in tumor suppressor “neurofibromin” —Chr17q
  • 3 main types
  • malig peripheral nerve sheath tumors
  • optic gliomas
  • other glial tumors

—also learning disabilities, seizures, skeletal abnorm, vascular abnorm, pigmented nodules of iris, cafe au lait spots on skin

1504 (5 decks)

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