The flashcards below were created by user
bigfootedbertha
on FreezingBlue Flashcards.
-
What is achondroplasia?
an impaired maturation of cartilage in the developing growth plate that affects all bones formed from cartilage and is a major cause of dwarfism
-
Describe the body of a person with achondroplasia
Shortening of PROXIMAL extremities, bowing of legs, and LORDOSIS.
-
What is the most common disease of the growth plate?
Achondroplasia
-
What causes achondroplasia?
A mutation in the FGF receptor 3, which suppresses cartilage growth
-
What is the msot common form of lethal dwarfism?
Thanatophoric dwarfism
-
What does a person with thanatophoric dwarfism look like?
shortening of the limbs, frontal bossing, macrocephaly, small chest cavity, bell shaped abdomen
-
What is the usual cause ofdeath in a person with thantophoric dwarfism?
respiratory depression (occurs near birth)
-
OI is an abnl development of what?
Type 1 collagen
-
What is the most common inherited connective tissue disorder?
OI
-
What is marble bone dz?
Osteopetrosis
-
What is Albers-Schonberg Disease?
Osteopetrosis
-
What is osteopetrosis?
An impaired formation of function of the osteoclasts that leads to decreased bone resorption
-
What are signs of osteopetrosis?
- diffuse symmetric skeletal sclerosis
- many fx
- bones lacking a medullary canal
- Ends of long bones are bulbous and misshapen
-
When do the different forms of osteopetrosis develop?
- Severe infantile malignant: evident in utero or soon after birth
- Mild autosomal dominant benign form: adolescence or adulthood
-
Bones with cup shaped deformity on xray and an accumulation of unmineralized bone
Osteomalacia-rickets
-
Replacement of bone by loose connective tissue
Hyperparathyroidism
-
Bacterial causes of ostemyelitis
staph, e coli, klebsiella, proteus
-
cause of osteomyelitis in sickle cell pts
salmonella
-
cause of osteomyelitis in newborns
H flu
-
cause of osteomyelitis in IVDA
pseudomonas
-
Pott's Disease
Presentation of extrapulmonary TB that most commonly occurs in the vertebral bodies.
-
Subperiosteal abscesses
Acute osteomyelitis, neutrophilic inflammation
-
Brodies abscess
abscess surrounded by sclerotic bone found in chronic osteomyelitis
-
What age range of patients gets Pagets?
55+
-
Where in the world is Paget's more common?
England, Australia, , N. Europe
-
Where in the body does Paget's affect?
L-S spine, pelvis, femur, skull
-
What causes Paget's?
Possible viral etiology
-
Paget's Disease may lead to what?
Osteosarcomas
-
What is the osteolytic phase of Paget's?
marrow replaced by CT with osteoclasts
-
What is the mixed phase of Paget's?
bone resportion and bone formation
-
What is the osteosclerotic phase of Pagets?
irregular bone deposition causing a mosaic pattern. Bone is abnormal.
-
What age group is affected by osteomas?
40-50
-
Where are osteomas most commonly located?
flat bones of skull and face, may protrude into the sinuses
-
What tumors are associated with Gardner's syndrome?
Osteomas
-
How dangerous are osteomas?
Are not malignant, primarily cause cosmetic defects
-
What age group is affected by osteoid osteomas?
10-30 yo
-
Where are osteoid osteomas most commonly found?
femur, tibia in metaphysis
-
Which tumors are painful and relieved by aspirin and high levels of prostaglandins?
Osteoid osteoma
-
What is the biggest difference between an osteoid osteoma and an osteoblastoma?
An osteoblastoma is painless, while an osteoid is painful
-
What is the most common primary bone malignancy?
Osteosarcomas
-
What ages are affected by osteosarcomas?
10-25, second peak after 40 yeras do to Pagets
-
Where are osteosarcomas located?Metaphysis, loewr femur and upper tibia (around knee joint), shoulder, and hip girdle can be affected, as well as the mandible
-
What is Codman's triangle?
An elevation of periosteum seen in osteosarcomas
-
What is the most common site of metastasis of osteosarcomas?
lung
-
What predisposes one to osteosarcomas?
- Pagets (>50)
- Radiation, chemo
- Trauma does not cause but brings it to attention
-
What age ranges are affected by chondroblastomas?
10-20
-
Where in the bone do chondroblastomas arise?
Epiphyseal: distal femur, proximal humerus
-
Who are affected by chondromyxoid fibromas?
young adults
-
Where in the bone do you find chondromyxoid fibromas?
long bones, small bones of feet
-
Who is affected by chondrosarcomas?
30-60 yo
-
Where are chondrosarcomas found?
in the bM of elbows, ribs, shoulder
-
Who is affected by Giant cell tumors?
FEMALES more than male, 20-30 yo
-
Where are most giant cell tumors located?
Metaphyseal-epiphyseal junction in the ends of bones (lower femur, upper tibia, lower radius)
-
Who is affected by Ewing's sarcoma?
5-20yo
-
Where are Ewing's sarcomas found?
long bones, pelvis, ribs, vertebrae
-
What is the mutation involved in Ewing's Sarcomas?
t(11;22)
-
Where do Ewings sarcomas arise?
The medullary cavity but may involve the entire long bone
-
What does Ewing's Sarcoma look like microscopically?
small blue cell tumors with very dense cellular portion of tissue. cells look monomorphic, with some wrinkling in them.
-
Who is affected by chordomas?
40-50yo
-
Where are chordomas found?
- Vertebral bodies, discs, sacrum
- Splenooccipital area in children
-
What is the most common primary tumor of bone, skull, spine, ribs?
plasma cell myeloma
-
What is Maffucci's Syndrome?
chondromas and soft tissue hemangiomas
-
what is olliere's disease?
unilateral chondromas and ovarian tumors
-
Which bony tumor has a radiolucent nius?
osteoid osteomas
-
How does Lyme affect the rest of the body?
- Early: Joint, muscle pain, meningitis, cardiac arrhythmias
- Late: damage to large joints and encephalitis
-
What type of crystals are formed with gout?
urate crystals
-
What is the genetic cause of both Duchennes and Beckers MDD?
X linked disorder involving structural protein dystrophin
-
What are the differences between Beckers and Duchennes?
Beckers' clinical manifestations are less severe, and sx progress more slowly. Dystrophin is present but in abnl form. (in Duchenne's it was missing)
-
What is the underlying disorder with myasthenia gravis?
it is an autoimmune disorder where there are Abs to acetylcholine receptors; often displaying thymic hyperplasia or thymomas
-
What is the most common soft tissue tumor?
lipoma
-
What is the most common soft tissue sarcoma in adults?
malignant fibrous histiocytoma
-
What is the most common type of postirradiation sarcoma?
malignant fibrous histiocytoma
-
what is the most common soft tissue sarcoma in the pediatric population?
rhabdomyosarcoma
-
What is the most common form of rhabdomyosarcoma?
embryonal
-
What is a dermatofibrosarcoma protuberans?
A cross between a benign fibrohistiocytic tumor, and a malignant fibrous histiocytoma
-
Whare are DFPs located?
dermis and subcutanous tissue
-
What lesion is pathonogmonic for DFP?
fibroblastic cells in a storiform pattern
-
What is a fibrosarcoma?
A malignant tumor located in the DEEP tissues of the thigh, knee, and trunk, that may grow slowly over yeras and met to the lungs
-
What do you see microscopically with a fibrosarcoma?
spindle shaped cells that are very cellular with few mitotic figures present
-
What do you see microscopically with a fibrous histiocytoma?
interlacing spindle cells
-
What do you see microscopically with a synovial sarcoma?
It has a biphasic pattern with an epithelial component and spindle cell component (glandular structures surrounded by spindle shaped cells)
-
What is nodular fascitis?
A painful, reactive fibroblastic proliferation found in young adults that is composed of plump, immature fibroblastic cells
-
What is a lipoma?
A soft tissue tumor in the subcutaneous tissue of adults that is slowly growing and composed of a soft yellow mass of mature adipose tissue
-
What is a liposarcoma?
A malignant tumor of ofadipocytes that peaks in the 5th to 6th decades, and is found in DEEP soft tissue or verisceral sites of the LE and abdomen.
-
What is an adamantinoma?
A rare bone tumor found in the tibia, femur, ulna, and fibula characterized by a poorly defined lytic lesion with sclerosis outline lucent areas and spindle cells surrounding basaloid cells
-
how serious is an adamantinoma?
it's a low grade malignancy with local recurrences but rarely mets
-
Which tumors are vascular but can be seen in bone?
hemangiomas, and they are common in the skull, vertebrae, and jaw
-
Where is prostate cancer common and uncommon?
Common in Scandinavian countries, uncommon in Japan and certain Asian countries
-
Where does most prostate cancer arise?
in the outer or peripheral glands
-
What type of tumors are prostate cancer mostly?
adenocarcinomas until proven otherwise
-
How soon does prostate cancer spread?
early
-
What do glands look like in prostate ca?
- They lie back to back with little to no intervening stroma
- conspicuous nucleoli
- absent basal layer
-
How is the Gleason score calculated?
- Based on architecture, glandular differentiation and nuclear features
- Take the best architectual feature and worst architectual feature and add them (after grading them).
- Gleasons of 6 are relatively common
-
Prostate mets are usually.....
osteoBLASTic
-
What can elevate PSA?
- hyperplasia, prostatitis, or carcinoma
- sex, ejaculation, DRE
-
What should your PSA be if you are under 50?
2.5
-
T/F: PSA value is enhanced when combined with other procedures and are good at evaluating treatments
True
-
Which testicular tumors tend to met earlier?
non seminomatous germ cell
-
T/f: most testicular tumors are benign
false
-
What is the most common primary testicular tumor in children under age 3?
Yolk sac tumors
-
What is the most common testicular tumor?
Seminoma
-
What do testicular seminomas look like?
Large soft great white tumors
-
Pts with seminomas have what kind of lab values?
elevated HCG
-
Which testicular cancers are benign?
teratomas (mostly)
-
Compare and contrast acute and chronic prostatitis and inflammation
Acute: neutrophil, chronic=lymphocytic
-
Which type of prostatitis is an important cause of recurrent UTI?
chronic prostatitis
-
What effect does achondroplasia have on intelligence/longetivity/reproduction?
None
-
What is the basic structural defect of thanatophoric dwarfism?
Decreased proliferation of chondrocytes in the growth plate
-
What age does OI affect?
Affects in utero (severe) or mildly affects children who lead nl lives into adulthood
-
What are the teo forms of osteopetrosis?
Autosomal dominant and autosomall fecessive
-
How does osteopetrosis affect the nervous system?
neural foramina are small and compress nerves
-
How does osteopetrosis manifest itself in the bone?
Deposited bone is not remodeled, and is woven into the architecture
-
What does autosomal recessive osteopetrosis manifest itself as?
severe infantile malignant osteopetrosis
-
What are clinical features of autosomal recessive/severe infantile osteopetrosis?
anemia, hydrocephaly, fx
-
What does autosomal dominant osteopetrosis manifest itself as?
will pop up in adolescence or adulthood due to repeated fx, some anemia and CN deficits if the foramina are narrowed
-
How does estrogen withdrawal affect bone?
increases bone loss and decreases bone synthesis
-
What is the genetic component of osteoporosis?
VDR
-
Accumulation of blood/hemosiderin in the bone that resembles a tumor but isn't
Brown tumor
-
Pott's disease is the TB version of what?
Osteomyelitis
-
What are Paget's sx?
they are usually asx
-
What lab is elevated in Pagets?
serum alk phos
-
How does Paget's affect the CV system?
leads to hypervascular bone lesions, characterized by warm skin and increased CO (which can lead to CHF)
-
How does Paget's affect the head?
it enlarges it, leading to HA, visual disturbances, and deafness
-
What kind of fx are featured in Pagets?
transverse fx of the long bones
-
Which two lesions have niduses?
Osteoid Osteoma and osteoblastomas
-
Which lesion has satellite nodules?
Osteosarcomas
-
How do osteosarcomas met?
via the blood
-
Which lesions feature exostoses?
osteochondromas
-
How do osteochondromas grow?
opposite direction of the joint
-
Which lesion features a cap of cartilage with bone underneath?
osteochondromas
-
What two lesions are associated with Gardners?
Osteoid osteomas and multiple osteochondromas
-
What does a chondroblastoma show on xray?
chicken wire pattern (immature cartilage)
-
What do xray findings of a chondrosarcoma show?
osteolytic lesions with areas of calcification and two or more nuclei per cell
-
What lesion is associated with atherosclerosis?
Giant cell tumors
-
Where do Ewings met to?
lungs, bones, CNS, LN
-
How dangerous is a chordoma?
malignant, destroy bone
-
What do chordomas look like microscopically?
phsalifersous cells (large tumor cells with bubbly cytoplasm);
-
cells that grow in cords and lobules separated by a mucoid matrix
chordomas
-
What lesion is associated with McCune Albright?
fibrous dysplasia
-
Where do bone mets m/c come from?
breast, lung, prostate
-
Where are bone mets usually found?
axial skelton 70%, extremities 30%
-
What is gouty nephropathy?
Uric acid crystals may obstruct renal tubules
-
What are signs of neurogenic muscular atrophy?
myofibers appear angular and atrophic, and increased activit of some enzymes
-
Which dystrophy features calf muscle enlargement?
Beckers
-
When do Duchenne's pts die?
20s usually due to respiratory problems
-
Where do fibrosarcomas met to?
hematogenously to the lungs
-
Interlacing spindle cells
fibrous histiocytoma
-
What do rhbadomyosarcomas look like?
grape like masses
-
What are rhabdomyosarcomas aka?
sarcoma botyroides
-
Where are leiomyomas found?
uterus
-
Where are leiomyosarcomas found?
malignant, seen in uterus and GIT
-
What kind of cells do synovial sarcomas arise from?
mesenchymal cells around joint cavities, NOT synovial cells
-
What mutation is associated with synovial sarcomas?
t(x, 18)
|
|