Bones, Joints, soft tissue tumors

  1. Developmental congenital disorders of bone and cartilage (3)
    • Aplasia (commonly fingers or ribs)
    • Supernumerary digits or ribs
    • abnormal fusion of sutures
    • development is known as DYSOSTOSIS: localized problem in migration of mesenchymal cells and the formation of bone
  2. type of congenital disorder of bone and cartilage knowns as dysplasia
    • mutations that interfere with bone formation/growth/maintenance
    • "abnormal growth", not pre-malignant
  3. Osteogenesis Imperfecta: common name, pathogenesis, symptoms
    • "brittle bone disease"
    • inherited conditions characterized by abnormal develop of type I collagen
    • results in bone fragility, abnormal dentition, hearing loss, blue sclera
    • *may mimic child abuse
  4. Achondroplasia pathogenesis and features
    • impaired proliferation and maturation of cartilage
    • 80% from spontaneous mutation
    • affects all bones that develop by endochondral ossification
    • shortening of arms and legs, bowing of legs, lordotic posture
  5. Osteopetrosis is known as...
    "marble bone disease" or "Albers-Schonberg d."
  6. Osteopetrosis: pathogenesis and features
    • characterized by deposition of over mineralized, brittle bone
    • caused by defective osteoclasts
    • increased incidence of fractures and cranial nerve palsies
    • crowding of bone marrow
  7. acquired diseases of bone (4)
    • osteoporosis
    • Pagets diseases 
    • rickets and osteomalacia
    • hyperparathyroidism
  8. Osteoporosisis characterized by ____ and is caused when _____.
    • reduced bone mass
    • bone resorption exceeds bone formation
  9. primary forms of osteoporosis (2)
    • age related (Senile) osteoporosis
    • post menopausal
  10. Risk factors for osteoporosis
    • genetic factors
    • diet
    • physical activity
    • peak bone mass (reached in 3rd decade)
  11. ostoeoporosis can be treated with
  12. Paget's disease is characterized by
    • accumulation of abnormal dense bone
    • bone is disordered and weak
    • causes headaches, bone enlargement, pain, neurological defecits, bowing of legs
  13. what type of fracture is indicative of Paget disease
    "chalk stick" fractures
  14. What would be different about serum in a person with Paget's disease?
    elevated serum alkaline phosphatase
  15. Treatment of paget disease
    bisphosphonates and/or calcitonin
  16. paget disease histology
    woven bone with mosaic pattern
  17. Rickets and osteomalacia are characterized by
    vitamin D deficiency causing accumulation of unmineralized matrix
  18. which groups get Rickets vs osteomalacia
    • rickets: kids
    • osteomalacia: adults
  19. In hyperparathyroidism, excess PTH in the body leads to...
    • body senses a greater need for serum calcium, which causes unabated osteoclast activity
    • elevated serum calcium
  20. treatment for hyperparathyroidism
  21. which fractures fail to heal?
    • comminuted
    • infected
    • inadequate immobilization, leading to pseudoarthosis
  22. how do fractures heal?
    heal with a bony callus, which remodels with used back to normal form
  23. Osteomyelitis is inflammation of bone and marrow caused by infection.  This infection can come from...
    • hematogenous spread (most common)
    • direct extension of infection
    • traumatic implantation
  24. most common pathogen that causes osteomyelitis?
    S. aureus
  25. can the causative organism of osteomyelitis always be isolated?
    no, only in about half of cases
  26. bone may separate in 2 way in osteomyelitis
    • sequestrum (non-vital bone)
    • involucrum (vital bone)
  27. treatment of osteomyelitis?
    debridement with vigorous and prolonged antibiotic therapy
  28. what is an osteoma?
    slow growing, exophytic growth of woven and lamellar bone
  29. Symptoms of Gardner syndrome?
    • multiple osteomas
    • colorectal adenomatous polyps
    • supernumerary and impacted teeth
    • epidermal cysts
  30. People with Gardner syndrome have an increased risk for what conditions
    colorectal and thyroid carcinomas
  31. How is osteoid osteoma and osteblastoma related?
    some believe they are variants of the same process
  32. What is the difference in experienced pain for osteoid osteoma and osteoblastoma?
    • Osteoid osteoma: nocturnal pain relieved by aspirin
    • Osteoblastoma: ill-defined pain NOT relieved by aspiring
  33. Radiology differences for Osteoid osteoma and osteoblastoma
    • osteoid osteoma: well-circumscribed radiolucency with central radiopaque nidus and surrounding reactive sclerosis
    • osteoblastoma: ill -or well-defined radiolucency with patchy calcifications or radiopaque
  34. relative sizes and location of osteoid osteoma and osteoblastoma tumors
    • osteoid osteoma: < 2 cm, appendicular skeleton
    • Osteoblastoma: > 2 cm, central skeleton
  35. Osteosarcoma is more commonly a ____ tumor than a _____ tumor
    • metastatic
    • primary
  36. What is the most common primary tumor of bone?
  37. Osteosarcoma is caused by
    production of osteoid by malignant cells most commonly in the 2nd decade
  38. osteosarcoma is commonly found in what part of the body?
    distal femur and proximal tibia (knee)
  39. Osteosarcoma radiology
    radiolucent, mixed or radiopaque; "sunburst" appearance, Codman's triangle
  40. What is the most common benign tumor of bone?
  41. Chondroma (endochondroma)
    • mature hyaline cartilage
    • small bones of hands and feet
    • any age
    • multiple lesions in Ollier's disease, Maffucci syndrome
  42. chondrosarcoma
    • malignant cartilaginous tumor
    • painful, progressively enlarging mass
    • well-differentiated to highly pleomorphic
    • rate of growth and behavior are correlated with histologic grade
  43. fibrous dysplasia pathogenesis
    normal bone replaced by fibrous tissue and malformed bone
  44. fibrous dysplasia radiology
    lesions are circumscribed and radiolucent or ill-defined and "ground glass"
  45. Features of McCune-Albright Syndrome
    • polyostotic fibrous dysplasia
    • cafe-au-lait pigmentations
    • precocious puberty in females
    • other endocrine abnormalities
  46. Ewing sarcoma
    • Lesions from PNET family of tumors
    • t(11,22) or t(21,22)
    • adolescents
    • painful lesion +/- fever
    • "onion skin" osteitis
  47. Giant cell tumor of bone comprises ___% of all benign bone tumors
  48. Giant cell tumor is characterized by
    • solitary, radiolucent lesion
    • commonly in epiphysis of long bones
    • may be mistake for arthritis
  49. The most common disorder of the joints?
  50. osteoarthritis pathogenesis
    • dengerative joint disease
    • dengeneration of the articular surface
    • eburnation (polishing)
    • bony proliferations at the joint margins (osteophytes)
  51. osteoarthritis features
    • can affect any joint
    • heberden's nodes: osteophytes at the distal interphalangeal joints
    • "joint mice" (loose bodies)
    • deep aching pain, exacerbated by use
  52. In osteoarthritis, vs rheumatoid, there is
    • NO fusion of bones
    • NO panes formation
    • minimal inflammation
  53. Rheumatoid arthritis is an _____ disease and is caused by ________
    • autoimmune disease
    • systemic, chronic inflammation
  54. rheumatoid arthritis affects _______ joints and results in _______ which progresses to _______
    • usually small
    • non-suppurative proliferative synovitis
    • joint destruction
  55. treatment for rheumatoid arthritis?
    anti-inflammatory and anti-tumor necrosis factor agents
  56. ____% of those untreated for the initial infection of Lyme disease get Lyme _____.
    • 60-80%
    • arthritis
  57. What disease is characterized by recurrent episodes of acute arthritis due to accumulation of uric acid in joints?
  58. in Gout, __________ crystals are deposited in the joints
    monosodium urate
  59. What is the most common soft tissue tumor in adults?
    lipoma (a type of adipose tumor)
  60. types of reactive proliferations that cause fibrous tumors
    • nodule fascitis
    • myositis ossificans (metaplastic bone, often after trauma)
  61. What condition is caused by mutated repair mechanisms causing fibrous tissues to be ossified
    fibrodysplasia ossificans progressiva
  62. fibrosarcoma
    • malignant neoplasm of fibroblasts
    • usually in adults
    • usually in deeper tissues
    • often metastasizes to the lunes
  63. what is a benign tumor of the skeletal muscles, sometimes associated with TB?
  64. what is a malignant tumor of skeletal muscle?
Card Set
Bones, Joints, soft tissue tumors
bones ppt diseases