Pathology III exam 1

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  1. 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
  2. Describe the body of a person with achondroplasia
    Shortening of PROXIMAL extremities, bowing of legs, and LORDOSIS.
  3. What is the most common disease of the growth plate?
  4. What causes achondroplasia?
    A mutation in the FGF receptor 3, which suppresses cartilage growth
  5. What is the msot common form of lethal dwarfism?
    Thanatophoric dwarfism
  6. What does a person with thanatophoric dwarfism look like?
    shortening of the limbs, frontal bossing, macrocephaly, small chest cavity, bell shaped abdomen
  7. What is the usual cause ofdeath in a person with thantophoric dwarfism?
    respiratory depression (occurs near birth)
  8. OI is an abnl development of what?
    Type 1 collagen
  9. What is the most common inherited connective tissue disorder?
  10. What is marble bone dz?
  11. What is Albers-Schonberg Disease?
  12. What is osteopetrosis?
    An impaired formation of function of the osteoclasts that leads to decreased bone resorption
  13. What are signs of osteopetrosis?
    • diffuse symmetric skeletal sclerosis
    • many fx
    • bones lacking a medullary canal
    • Ends of long bones are bulbous and misshapen
  14. 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
  15. Bones with cup shaped deformity on xray and an accumulation of unmineralized bone
  16. Replacement of bone by loose connective tissue
  17. Bacterial causes of ostemyelitis
    staph, e coli, klebsiella, proteus
  18. cause of osteomyelitis in sickle cell pts
  19. cause of osteomyelitis in newborns
    H flu
  20. cause of osteomyelitis in IVDA
  21. Pott's Disease
    Presentation of extrapulmonary TB that most commonly occurs in the vertebral bodies.
  22. Subperiosteal abscesses
    Acute osteomyelitis, neutrophilic inflammation
  23. Brodies abscess
    abscess surrounded by sclerotic bone found in chronic osteomyelitis
  24. What age range of patients gets Pagets?
  25. Where in the world is Paget's more common?
    England, Australia, , N. Europe
  26. Where in the body does Paget's affect?
    L-S spine, pelvis, femur, skull
  27. What causes Paget's?
    Possible viral etiology
  28. Paget's Disease may lead to what?
  29. What is the osteolytic phase of Paget's?
    marrow replaced by CT with osteoclasts
  30. What is the mixed phase of Paget's?
    bone resportion and bone formation
  31. What is the osteosclerotic phase of Pagets?
    irregular bone deposition causing a mosaic pattern. Bone is abnormal.
  32. What age group is affected by osteomas?
  33. Where are osteomas most commonly located?
    flat bones of skull and face, may protrude into the sinuses
  34. What tumors are associated with Gardner's syndrome?
  35. How dangerous are osteomas?
    Are not malignant, primarily cause cosmetic defects
  36. What age group is affected by osteoid osteomas?
    10-30 yo
  37. Where are osteoid osteomas most commonly found?
    femur, tibia in metaphysis
  38. Which tumors are painful and relieved by aspirin and high levels of prostaglandins?
    Osteoid osteoma
  39. What is the biggest difference between an osteoid osteoma and an osteoblastoma?
    An osteoblastoma is painless, while an osteoid is painful
  40. What is the most common primary bone malignancy?
  41. What ages are affected by osteosarcomas?
    10-25, second peak after 40 yeras do to Pagets
  42. 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
  43. What is Codman's triangle?
    An elevation of periosteum seen in osteosarcomas
  44. What is the most common site of metastasis of osteosarcomas?
  45. What predisposes one to osteosarcomas?
    • Pagets (>50)
    • Radiation, chemo
    • Trauma does not cause but brings it to attention
  46. What age ranges are affected by chondroblastomas?
  47. Where in the bone do chondroblastomas arise?
    Epiphyseal: distal femur, proximal humerus
  48. Who are affected by chondromyxoid fibromas?
    young adults
  49. Where in the bone do you find chondromyxoid fibromas?
    long bones, small bones of feet
  50. Who is affected by chondrosarcomas?
    30-60 yo
  51. Where are chondrosarcomas found?
    in the bM of elbows, ribs, shoulder
  52. Who is affected by Giant cell tumors?
    FEMALES more than male, 20-30 yo
  53. Where are most giant cell tumors located?
    Metaphyseal-epiphyseal junction in the ends of bones (lower femur, upper tibia, lower radius)
  54. Who is affected by Ewing's sarcoma?
  55. Where are Ewing's sarcomas found?
    long bones, pelvis, ribs, vertebrae
  56. What is the mutation involved in Ewing's Sarcomas?
  57. Where do Ewings sarcomas arise?
    The medullary cavity but may involve the entire long bone
  58. 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.
  59. Who is affected by chordomas?
  60. Where are chordomas found?
    • Vertebral bodies, discs, sacrum
    • Splenooccipital area in children
  61. What is the most common primary tumor of bone, skull, spine, ribs?
    plasma cell myeloma
  62. What is Maffucci's Syndrome?
    chondromas and soft tissue hemangiomas
  63. what is olliere's disease?
    unilateral chondromas and ovarian tumors
  64. Which bony tumor has a radiolucent nius?
    osteoid osteomas
  65. How does Lyme affect the rest of the body?
    • Early: Joint, muscle pain, meningitis, cardiac arrhythmias
    • Late: damage to large joints and encephalitis
  66. What type of crystals are formed with gout?
    urate crystals
  67. What is the genetic cause of both Duchennes and Beckers MDD?
    X linked disorder involving structural protein dystrophin
  68. 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)
  69. 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
  70. What is the most common soft tissue tumor?
  71. What is the most common soft tissue sarcoma in adults?
    malignant fibrous histiocytoma
  72. What is the most common type of postirradiation sarcoma?
    malignant fibrous histiocytoma
  73. what is the most common soft tissue sarcoma in the pediatric population?
  74. What is the most common form of rhabdomyosarcoma?
  75. What is a dermatofibrosarcoma protuberans?
    A cross between a benign fibrohistiocytic tumor, and a malignant fibrous histiocytoma
  76. Whare are DFPs located?
    dermis and subcutanous tissue
  77. What lesion is pathonogmonic for DFP?
    fibroblastic cells in a storiform pattern
  78. 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
  79. What do you see microscopically with a fibrosarcoma?
    spindle shaped cells that are very cellular with few mitotic figures present
  80. What do you see microscopically with a fibrous histiocytoma?
    interlacing spindle cells
  81. 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)
  82. What is nodular fascitis?
    A painful, reactive fibroblastic proliferation found in young adults that is composed of plump, immature fibroblastic cells
  83. 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
  84. 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.
  85. 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
  86. how serious is an adamantinoma?
    it's a low grade malignancy with local recurrences but rarely mets
  87. Which tumors are vascular but can be seen in bone?
    hemangiomas, and they are common in the skull, vertebrae, and jaw
  88. Where is prostate cancer common and uncommon?
    Common in Scandinavian countries, uncommon in Japan and certain Asian countries
  89. Where does most prostate cancer arise?
    in the outer or peripheral glands
  90. What type of tumors are prostate cancer mostly?
    adenocarcinomas until proven otherwise
  91. How soon does prostate cancer spread?
  92. What do glands look like in prostate ca?
    • They lie back to back with little to no intervening stroma
    • conspicuous nucleoli
    • absent basal layer
  93. 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
  94. Prostate mets are usually.....
  95. What can elevate PSA?
    • hyperplasia, prostatitis, or carcinoma
    • sex, ejaculation, DRE
  96. What should your PSA be if you are under 50?
  97. T/F: PSA value is enhanced when combined with other procedures and are good at evaluating treatments
  98. Which testicular tumors tend to met earlier?
    non seminomatous germ cell
  99. T/f: most testicular tumors are benign
  100. What is the most common primary testicular tumor in children under age 3?
    Yolk sac tumors
  101. What is the most common testicular tumor?
  102. What do testicular seminomas look like?
    Large soft great white tumors
  103. Pts with seminomas have what kind of lab values?
    elevated HCG
  104. Which testicular cancers are benign?
    teratomas (mostly)
  105. Compare and contrast acute and chronic prostatitis and inflammation
    Acute: neutrophil, chronic=lymphocytic
  106. Which type of prostatitis is an important cause of recurrent UTI?
    chronic prostatitis
  107. What effect does achondroplasia have on intelligence/longetivity/reproduction?
  108. What is the basic structural defect of thanatophoric dwarfism?
    Decreased proliferation of chondrocytes in the growth plate
  109. What age does OI affect?
    Affects in utero (severe) or mildly affects children who lead nl lives into adulthood
  110. What are the teo forms of osteopetrosis?
    Autosomal dominant and autosomall fecessive
  111. How does osteopetrosis affect the nervous system?
    neural foramina are small and compress nerves
  112. How does osteopetrosis manifest itself in the bone?
    Deposited bone is not remodeled, and is woven into the architecture
  113. What does autosomal recessive osteopetrosis manifest itself as?
    severe infantile malignant osteopetrosis
  114. What are clinical features of autosomal recessive/severe infantile osteopetrosis?
    anemia, hydrocephaly, fx
  115. 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
  116. How does estrogen withdrawal affect bone?
    increases bone loss and decreases bone synthesis
  117. What is the genetic component of osteoporosis?
  118. Accumulation of blood/hemosiderin in the bone that resembles a tumor but isn't
    Brown tumor
  119. Pott's disease is the TB version of what?
  120. What are Paget's sx?
    they are usually asx
  121. What lab is elevated in Pagets?
    serum alk phos
  122. 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)
  123. How does Paget's affect the head?
    it enlarges it, leading to HA, visual disturbances, and deafness
  124. What kind of fx are featured in Pagets?
    transverse fx of the long bones
  125. Which two lesions have niduses?
    Osteoid Osteoma and osteoblastomas
  126. Which lesion has satellite nodules?
  127. How do osteosarcomas met?
    via the blood
  128. Which lesions feature exostoses?
  129. How do osteochondromas grow?
    opposite direction of the joint
  130. Which lesion features a cap of cartilage with bone underneath?
  131. What two lesions are associated with Gardners?
    Osteoid osteomas and multiple osteochondromas
  132. What does a chondroblastoma show on xray?
    chicken wire pattern (immature cartilage)
  133. What do xray findings of a chondrosarcoma show?
    osteolytic lesions with areas of calcification and two or more nuclei per cell
  134. What lesion is associated with atherosclerosis?
    Giant cell tumors
  135. Where do Ewings met to?
    lungs, bones, CNS, LN
  136. How dangerous is a chordoma?
    malignant, destroy bone
  137. What do chordomas look like microscopically?
    phsalifersous cells (large tumor cells with bubbly cytoplasm);
  138. cells that grow in cords and lobules separated by a mucoid matrix
  139. What lesion is associated with McCune Albright?
    fibrous dysplasia
  140. Where do bone mets m/c come from?
    breast, lung, prostate
  141. Where are bone mets usually found?
    axial skelton 70%, extremities 30%
  142. What is gouty nephropathy?
    Uric acid crystals may obstruct renal tubules
  143. What are signs of neurogenic muscular atrophy?
    myofibers appear angular and atrophic, and increased activit of some enzymes
  144. Which dystrophy features calf muscle enlargement?
  145. When do Duchenne's pts die?
    20s usually due to respiratory problems
  146. Where do fibrosarcomas met to?
    hematogenously to the lungs
  147. Interlacing spindle cells
    fibrous histiocytoma
  148. What do rhbadomyosarcomas look like?
    grape like masses
  149. What are rhabdomyosarcomas aka?
    sarcoma botyroides
  150. Where are leiomyomas found?
  151. Where are leiomyosarcomas found?
    malignant, seen in uterus and GIT
  152. What kind of cells do synovial sarcomas arise from?
    mesenchymal cells around joint cavities, NOT synovial cells
  153. What mutation is associated with synovial sarcomas?
    t(x, 18)
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Pathology III exam 1
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